path kidney and the collecting system - formatted

Question 1

1. 14.APRIL02 CT guided biopsy of an atypical non-functional kidney, shows “ replacement of the normal architecture by numerous foamy macrophages intermingled with plasma cell, lymphocytes, polymorphonuclear leukocytes and occasional giant cells. The latter are not of the Langerhan’s type,” This suggests:
2. Renal Tuberculosis
3. Renal cell carcinoma
4. Xanthogranulomatous pyelonephritis
5. Oncocytoma
6. Post obstructive atrophy

Answer 1

3. Xanthogranulomatous pyelonephritis

• Xanthogranulomatous pyelonephritis is an unusual and relatively rare form of chronic pyelonephritis characterized by accumulation of foamy macrophages intermingled with plasma cells, lymphocytes, polymorphonuclear leukocytes, and occasional giant cells.
• Often associated with Proteus infections and obstruction, the lesions sometimes produce large, yellowish orange nodules that may be confused with renal cell carcinoma

Question 2

2. 13.APRIL02 A 50-year-old diabetic woman has papillary necrosis on IVP. Which of the following pathological features would most favour analgesic nephropathy over diabetic nephropathy?
3. Duration of diabetes of less than 15 years
4. The absence of calcification
5. Less than 5 papillae affected
6. Lesions of differing ages
7. Associated accelerated atherosclerosis

Answer 2

4. Lesions of differing ages

• Kidneys either normal or slightly  size with irregular outline due to cortical atrophy overlying necrotic papillae
• Papillae in various stages of necrosis with calcification, fragmentation, sloughing (cf diabetic papillary necrosis where all papillae are at same stage of necrosis)

Question 3

3. 14.02.65 The features of analgesic nephropathy do not include ?
4. Renal artery stenosis
5. Renal calculi
6. Increased urinary Tamm-Horsfall protein
7. Nephritis
8. TCC

Answer 3

1. Renal artery stenosis
2. 14.02.65 The features of analgesic nephropathy do not include ? Rob 530
3. Renal artery stenosis
4. Renal calculi (Nephrocalcinosis & stones 20 acquired renal tubular acidosis)
5. Increased urinary Tamm-Horsfall protein (Ischaemic ATN)
6. Nephritis (Papillary necrosis occurs first  20 tubulointerstitial nephritis)
7. TCC

• Tamm-Horsfall mucoprotein is the basic matrix of all renal casts
o it originates from tubular epithelial cells and is always present in the urine.
o When the casts contain only mucoproteins, they are called hyaline casts and may not have any pathologic significance.
o Hyaline casts may be seen in the urine after exercise or heat exposure, but may also be observed in pyelonephritis or chronic renal disease
• Tamm-Horsfall protein = urinary glycoprotein
o Seen in multiple myeloma
o Ischaemic ATN

• Papillae in various stages of necrosis with calcification, fragmentation, sloughing (cf diabetic papillary necrosis where all papillae are at same stage of necrosis)

Question 4

4. 14.03.60 40 year old patient has staghorn calculus not present previous year, LEAST LIKELY cause is ?
5. Hyperuricaemia
6. Crohns Disease
7. Hypercalcuria
8. Hyperoxaluria
9. Recurrent urinary tract infections

Answer 4

1. Hyperuricaemia

• staghorn calculi
o The majority of staghorn calculi are infection stones.
o They may start as matrix calculi composed of a glycoprotein cast within the collecting system, upon which triple phosphate infection stones are deposited
o Most staghorn stones are composed of struvite
o Other stone materials less often encountered that can also assume a staghorn configuration include cystine, calcium oxalate monohydrate, and uric acid
• hyperuricemia
o uric acid stones are present in 22% of patients with gout and 42% of those with secondary hyperuricemia
o causes urine to be acidic(c.f. Infection by urea-splitting organisms – Proteus and Staph convert urea to ammonia  alkaline urine with precipitation of magnesium ammonium phosphate salts )
• crohns frequently causes oxalate stones, which may cause staghorn type calculi

Question 5

5. 14.03.61 Which is NOT a type of renal cell cancer ?
6. Clear
7. Papillary
8. Collecting duct
9. Chromophobe
10. Small cell

Answer 5

5. Small cell

Question 6

6. 14.02.66 Features of Clear cell renal cell Ca include ? Rob p539
7. commonest at lower pole
8. tan/white tumour macroscopically

Answer 6

2. tan/white tumour macroscopically (yellow-gray-white in colour)

In general RCC is more common in the renal poles, but can occur anywhere, most commonly the poles of which the upper pole is most common.
Clear cell - most common - characterized by abundant clear cytoplasm. Most commonly sporadic, but is the type seen in VHL (sporadic also has mutations in VHL tumor suppressor gene).
Robbins pg953 RY

Question 7

7. 14.03.62 Which is TRUE? Rob p541
8. Intestinal cystitis/ Hunner Ulcer is associated with SLE
9. Malokplakia is metaplasia secondary to chronic infection
10. 5% of bladder tumours are adenocarcinoma
11. 5 year survival for grade I tumours is 75%
12. Sessile bladder tumour – 1/3 of low grade, 80% of high grade TCC

Answer 7

1. Intestinal cystitis/ Hunner Ulcer is associated with SLE
   * LW: agree it can be, unknown etiology, but considerd auto immune origin as associated with SLE.
2. 14.03.62 Which is TRUE? Rob p541
3. Intestinal cystitis/ Hunner Ulcer is associated with SLE: TRUE
4. Malakoplakia is metaplasia secondary to chronic infection: FALSE, not metaplasia, but related to chronic infection.
5. 5% of bladder tumours are adenocarcinoma: FALSE; rare as in less than 5% (rare )
6. 5 year survival for grade I tumours is 75%: FALSE (99%)
7. Sessile bladder tumour – 1/3 of low grade, 80% of high grade TCC: FALSE (low grade tumours are almost always papillary)

• Malakoplakia is caused by defects in phagocytic or degradative functions of histiocytes in response to gram negative coliforms; laminated mineralized concretions known as Michaelis-Gutmann bodies are typically present forming an inflammatory plaque but no metaplasia) (leukoplakia = keratinizing squamous metaplasia / dysplasia) Cystitis Glandularis and Cystitis Cystica = intestinal metaplasia).

Question 8

8. 14.02.46 Features of Bladder tumours do NOT include:
9. 70% Solitary
10. low grade lesions tend to be flat
11. Transitional cell in 95%

Answer 8

2. low grade lesions tend to be flat (low grade tumours are almost always papillary)

**LJS - disagree with 70% solitary - Rob says often multiple (field defect)
Precursor lesions can be papillary (ranging from papilloma to high grade papillary urothelial carcinoma) or CIS (flat). Terminology around precursor lesions appears to have changed btw editions of Rob - ?old question

8. 14.02.46 Features of Bladder tumours do NOT include:
9. 70% solitary (70% confined to bladder at diagnosis lateral or posterior bladder walls)
10. low grade lesions tend to be flat (low grade tumours are almost always papillary)
11. Transitional cell in 95% (90% of TCC’s)

Question 9

9. 15.02.69 Sclerosing retroperitonitis NOT due to:
10. Crohns
11. Lymphoma
12. Migraine medication
13. Sclerosing conditions elsewhere

Answer 9

*LW:
Incomplete recall with correct answer not included.

Robbins quotes following causes:
Drugs - ergot derivatives (MIGRAINE Med), B adrenergic blockers.
Adjacent inflamm conditions: vasculitis, diverticulitis, Crohn disease.
malignant disease - lymphoma, urinary tract carcinomas.
70% idiopathic.

Not sure about Crohn’s (not in retroperitoneum) unless ascending or descending bowel/duodenum involved. - not listed in radiopedia nor statdx

Causes of retroperitoneal fibrosis

• haemorrhage
• infection
• inflammation (pancreatitis, duodenitis, pyelonephritis)- IgG4
• trauma
• tumour (primary, met or lymphoma)
• toxin (antihypertensives, )
• radiation

Question 10

List morphological appearance of bladder cancer

Answer 10

• The gross patterns of urothelial cell tumors vary from purely papillary to nodular or flat to mixed papillary and nodular.
• The tumors may also be invasive or noninvasive.
• The papillary lesions appear as red elevated excrescences varying in size from less than 1 cm in diameter to large masses up to 5 cm in diameter.
• Multicentric origins may produce separate tumors.
• As noted, the histologic changes encompass a spectrum from benign papilloma to highly aggressive anaplastic cancers.
• Overall, about half of bladder cancers are high-grade lesions.
• Most arise from the lateral or posterior walls at the bladder base.
• Papillomas and low-grade lesions are almost always papillary.
• Higher grades may be flat or papillary.
• Many high-grade III lesions may be fungating, necrotic, sometimes ulcerative tumors that have unmistakably invaded deeply .
• With the higher grade neoplasms, in areas of the bladder devoid of tumor, there may frequently be areas of mucosal hyperplasia, dysplasia, or carcinoma in situ.
• In most analyses, less than 10% of low-grade cancers invade, but as many as 80% of high-grade transitional cell carcinomas are invasive.
• Aggressive tumors may extend only into the bladder wall, but the more advanced stages invade the adjacent prostate, seminal vesicles, ureters, and retroperitoneum, and some produce fistulous communications to the vagina or rectum.
• About 40% of these deeply invasive tumors metastasize to regional lymph nodes.
• Hematogenous dissemination, principally to the liver, lungs, and bone marrow, generally occurs late, and only with highly anaplastic tumors

 

Question 11

11. Sep03.37 Focal renal infarct, least likely?
12. Atherosclerosis
13. Mitral valve regurgitation
14. PAN
15. HUS
16. Endocarditis

Answer 11

*LW:
Preferred answer is Mitral Regurgiation.
4. HUS (Acute cortical necrosis not focal renal infarct)
*LW: Mitral valve regurgitation by itself would not cause renal infarction, while HUS will cause microthrombi within renal vasculature and acute cortical necrosis, thus is more likely than isolated MR. Hence preferred answer is MR.

11. Sep03.37 Focal renal infarct, least likely: ** check this **
12. Atherosclerosis (thrombosis in advanced atherosclerosis)
13. Mitral valve regurgitation
14. PAN (acute vasculitis of polyarteritis nodosa)
15. HUS (Acute cortical necrosis not focal renal infarct)
16. Endocarditis (vegetative endocarditis)

• Most due to embolism
• Major source of emboli = mural thrombi from L atrium and vent. from AMI
• Less common sources
o vegetative endocarditis
o thrombosis in aortic aneurysms and aortic atherosclerosis
• Rare causes
o thrombosis in advanced atherosclerosis
o acute vasculitis of polyarteritis nodosa

• On gross examination, the kidneys are swollen and pale; many fleabite hemorrhages are on the surface.
• In HUS and TTP, the platelet and fibrin microthrombi within the renal microvasculature are accompanied by thrombocytopenia and a microangiopathic hemolytic anemia.
• Vasculitis is usually absent.

Question 12

12. Sep03.56 CRF secondary to Goodpastures on haemodialysis presents with collapse with haematuria. What is the most likely cause ?
13. Renal cysts
14. Stones
15. Obstruction/ hydronephrosis

Answer 12

*LW: Robins states dialysis associated renal cystic disease can bleed causing haematuria. Hence renal cysts is my preferred option. Above instances can cause renal failure and haematuria, but acute presentation with hypotension favours a cyst rupture.

• The renal manifestations of all forms include hematuria with red cell casts in the urine, moderate proteinuria occasionally reaching the nephrotic range, and variable hypertension and edema.
• In Goodpasture syndrome, the course may be dominated by recurrent hemoptysis or even life-threatening pulmonary hemorrhage.
• Serum analyses for anti-GBM, antinuclear antibodies, and ANCA are helpful in the diagnosis of specific subtypes.
• Although milder forms of glomerular injury may subside, the renal involvement is usually progressive during a matter of weeks, culminating in severe oliguria.
• Recovery of renal function may follow early intensive plasmapheresis (plasma exchange) combined with steroids and cytotoxic agents in Goodpasture syndrome.
• This therapy appears to reverse both pulmonary hemorrhage and renal failure. Other forms of RPGN also respond well to steroids and cytotoxic agents. Despite therapy, patients may eventually require chronic dialysis or transplantation.
• Most cases begin clinically with respiratory symptoms, principally hemoptysis, and radiographic evidence of focal pulmonary consolidations.
• Soon, manifestations of glomerulonephritis appear, leading to rapidly progressive renal failure.
• The common cause of death is uremia (but not sudden collapse???)
• Most cases occur in the teens or twenties, and there is a preponderance among men.
• The kidneys reveal the characteristic findings of focal proliferative glomerulonephritis in the early cases, or crescentic glomerulonephritis in patients with rapidly progressive glomerulonephritis.
• Linear deposits of immunoglobulins and complement are also seen by immunofluorescence studies along the glomerular basement membranes, similar to those in the alveolar septa

Question 13

13. Sep03.60 Magnesium ammonium phosphate stones
14. UTI – urea transformed into ammonia
15. Nidus

Answer 13

1. UTI – urea transformed into ammonia

• Magnesium ammonium phosphate stones are formed largely after infections by urea-splitting bacteria (e.g., Proteus and some staphylococci), which convert urea to ammonia.
o The resultant alkaline urine causes the precipitation of magnesium ammonium phosphate salts.
o These form some of the largest stones, as the amounts of urea excreted normally are huge.
o Indeed, so-called staghorn calculi are almost always associated with infection.

• Magnesium ammonium phosphate hexahydrate (struvite) calculi are associated with an alkaline urine and usually are accompanied by, and are the result of, a urea-splitting bacillary urinary tract infection, usually with Proteus mirabilis.
• Pure struvite stones are rare and of relatively low radiopacity but often are laminated with more dense calcium salts, usually calcium phosphate, less often calcium carbonate, resulting in a magnesium-ammonium-calcium phosphate or triple-phosphate stone.
• These laminations suggest that recurrent infections and frequent changes in urinary pH have occurred in the chemical milieu in which the calculus formed.
• Triple-phosphate stones usually form a cast of the renal pelvis and, by direct extension, one or more infundibula and calyces.
• Characteristically, the peripheral components of an infection-related staghorn calculus are directly connected extensions of the renal pelvis component
• Struvite and apatite, occurring either separately or admixed as a triple-phosphate stone, account for 5% to 30% of upper tract calculi.
• In combination, they are the most common constituents of a staghorn calculus.

Question 14

14. Sep03.66 Fatty lesion next to effaced kidney. Liposarcoma? Which of the following is most relevant?
15. Tuberous sclerosis
16. Previous radiotherapy

Answer 14

2. Previous radiotherapy

• AML - tuberous sclerosis
• Liposarcomas are one of the most common sarcomas of adulthood and appear in the forties to sixties
o they are uncommon in children.
• The cause of most soft tissue tumors is unknown.
• There are documented associations, however, between radiation therapy and rare instances in which chemical burns, heat burns, or trauma were associated with subsequent development of a sarcoma
• They usually arise in the deep soft tissues of the proximal extremities and retroperitoneum and are notorious for developing into large tumors.
• Histologically, liposarcomas can be divided into the following variants.
o well-differentiated
o myxoid
o round cell
o pleomorphic
• The cells in well-differentiated liposarcomas are readily recognized as lipocytes.
• In the other variants, most of the tumor cells are not obviously adipogenic, but some cells indicative of fatty differentiation are almost always present.
• They are known as lipoblasts, which mimic fetal fat cells
• They contain round cytoplasmic vacuoles of lipid that scallop the nucleus.
• The myxoid and round cell variant of liposarcoma has a t(12;16) chromosomal abnormality in most cases.
• well-differentiated variant is relatively indolent,
• the myxoid type is intermediate in its malignant behavior
• round cell and pleomorphic variants usually are aggressive and frequently metastasize.
• All types of liposarcoma recur locally and often repeatedly unless adequately excised.
• 2/3rd’s of retroperitoneal liposarcomas are low-grade, well differentiated neoplasms.
• Most well-differentiated tumors contain mature adipose tissue and only occasional atypical cells.
• These neoplasms are often referred to as well-differentiated, lipogenic, or lipoma-like liposarcomas.
• Sclerosing liposarcomas are less commonly encountered well-differentiated tumors.
• Sclerosing liposarcomas contain an extensive fibrous tissue stroma.
• In one recent series, the sclerosing components of several well-differentiated liposarcomas (that also contained lipogenic areas) were of similar attenuation or signal intensity to muscle on computed tomography (CT) and magnetic resonance (MR) imaging examinations and did not contain any recognizable fat.

• Renal manifestations of TS
o Renal failure in severe cases (5%)
o Hypertension
o Angiomyolipomas – multiple, large
 5% have fat elements that can be detected only by microscopy
 AML associated with tuberous sclerosis in 20%
 AML in 80% of patients with tuberous sclerosis (80% TS have AML, 20% AML have TS)
o Cysts
o Renal cell carcinoma (3%), bilateral in 40%

Question 15

15. Sep03.82 5 year survival for RCC with extension into the renal vein
16. 90%
17. 70%
18. 30%
19. 10%

Answer 15

• LW: Renal vein extension is Stage 3 disease, with radiopedia stating stage 3 disease = 30% 5YS.
• *Need to check in Robbins**

3. 20%•

• 5 year survival in patients with renal vein invasion or extension into the perinephric fat is 15-20% may be bad recall
• Tumour stage + histologic grade are the most important prognosticators
• Papillary carcinomas have better prognosis
• Overall 45% 5YS
• 70% 5YS without metastases
• 15-20% 5YS if perinephric fat or renal vein invasion

Question 16

16. Sep03.83 Wilms tumour, age at presentation

1. 2-5 years

Answer 16

• Wilms tumour is usually diagnosed between the ages of 2 and 5 years.

Question 17

17. Sep03.86 Incidence of polycystic kidneys
18. 1:1000
19. 1:10000
20. 1:10
21. none of the above

Answer 17

1. 1:1000• APCKD : 1 in 400-1000

Question 18

1. PATH2004 Which of the following are all recognised bladder carcinoma morphological patterns:
2. Papillary, ulcerative, diffuse, cystic, infiltrative
3. Papillary, tubular, sessile, excavative
4. Papillary, tubular, cystic, flat
5. Flat, excavative, polypoid
6. Flat, papillary-invasive, papilloma-papillary

Answer 18

5. Flat, papillary-invasive, papilloma-papillary

Question 19

2. PATH2004 Focal renal infarct, least likely:
3. Aortic atherosclerosis
4. Mitral valve regurgitation
5. Post AMI
6. PAN
7. Adult Haemolytic Uremic Syndrome
8. Candida endocarditis

Answer 19

2. MR
   * LW: Mitral valve regurgitation by itself would not cause renal infarction, while HUS will cause microthrombi within renal vasculature, thus is more likely than isolated MR. Hence preferred answer is MR.

Question 20

6. PATH2004 Regarding renal cell carcinoma. Which of the following is the LEAST CORRECT:
7. Strong association with Von-Hippel-Lindau syndrome
8. Clear cell carcinoma is the most common (70-80%)
9. Increased risk of renal cell carcinoma in chronic renal failure
10. Most frequent presentation is flank pain or discomfort
11. Hereditary tumours are more frequently bilateral and multifocal

Answer 20

4. Most frequent presentation is flank pain or discomfort

• The three classic diagnostic features of costovertebral pain, palpable mass, and hematuria unfortunately appear in only 10% of cases. The most reliable of the three is hematuria, but it is usually intermittent and may be microscopic; thus, the tumor may remain silent until it attains a large size.

Question 21

7. PATH2004 Which of the following is not derived from renal tubules?
8. Papillary cell carcinoma
9. clear cell carcinoma
10. oncocytoma
11. renal tubular adenoma
12. angiomyolipoma

Answer 21

5. angiomyolipoma

Question 22

8. PATH2004 Regarding prostatic carcinoma. Which of the following is the least correct:
9. 70-80% of males between the ages of 70-80 have foci
10. Histologically sheets or cords of cells in dense fibrous sroma
11. 70% arise in the peripheral zone
12. Local invasion into the seminal vesicles, bladder and ureter
13. Carcinoma of the prostate is less common in Asia compared with Australia and New Zealand

Answer 22

2. Histologically sheets or cords of cells in dense fibrous sroma - FALSE
   • Histologically, most lesions are adenocarcinomas that produce well-defined, readily demonstrable gland patterns.

Question 23

9. PATH2004 Following NOT associated with increased risk of testicular malignancy
10. Cryptorchidism
11. Testicular feminisation
12. Testicular microlithiasis
13. Klinefelters syndrome
14. TB of the testis

Answer 23

*LW:
Favored answer: Microlithiasis - NOT associated with increased risk of malignancy.

pathoutlines states granulomatous infection of the testes can result in seminoma.

**LJS - disagree
big Rob 9th ed p 975: “Klinefelters ass/w x50 incr risk of mediastinal GCT but these patients do not develop testicular tumours”. I think this is most incorrect
-Whilst testicular microlithiasis is controversial and benign on its own, there is an ass/w testicular GCT. Stat dx “Coexistent germ cell tumor in 18-75% of symptomatic males referred for ultrasound”
-Mumps and HIV are risk factors for testicular GCT, but can’t find anything about TB (Robbins, pathoutlines, statdx, RP, google papers). Seminomas can have associated granulomatous inflammation found at bx, but this is described in pathoutlines as a ddx for granulomatous orchitis, not risk factor for seminoma. TB can mimic testicular tumour. I suspect this was the incorrect answer originally
https://www.pathologyoutlines.com/topic/testisgranulomatousorchitis.html
http://www.pathologyoutlines.com/topic/testisseminomas.html

3. Testicular microlithiasis This is now considered to be not true as well. SG

Question 24

10. PATH2004 scrotal US – correct
11. varicocele usu right sided
12. hydroceles not associated with ca
13. cause of hydrocele may be unknown
14. cryptorchidism may be associated with inguinal hernia

Answer 24

*LW:
Variocele usually left sided,
hydroceles not associated with cancer,
cause of hydrocele may be unknown, and cryptorchidism may be associated with inguinal hernia. Maybe incorrect recall, should read which is incorrect?

3. cause of hydrocele may be unknown this is true as well (SG)
4. cryptorchidism may be associated with inguinal hernia

Question 25

11. PATH2004 aFP increased in

1. yolk sac tumours

Answer 25

YES

• AFP synthesized by the fetal gut, liver cells, and yolk sac
• One year after birth, the serum levels of AFP fall to less than 16 ng/ml,
• HCG is normally synthesized and secreted by the placental syncytiotrophoblast.
• The beta subunit of HCG has unique sequences not shared with other human glycoprotein hormones, and therefore the detection of HCG in the serum is based on a radioimmunoassay using antibodies to its beta chain.
• Yolk sac tumors produce AFP exclusively, and choriocarcinomas elaborate only HCG.
• Either or both of these markers are elevated in more than 80% of patients with NSGCT at the time of diagnosis.

Question 26

1. In epididymitis, which is most likely to have orchitis without epididymitis :
2. Syphilis
3. TB
4. Pseudomonas
5. E. Coli
6. Gonococcus

Answer 26

*AJL has changed the question around.
Answer is now syphilis.

1. In epididymitis, which is most likely to have orchitis without epididymitis :
2. Syphilis (testis first if acquired)
3. TB (Begins in epididymis and spreads to testis)
4. Pseudomanas and this one
5. E. Coli and this one
6. Gonococcus and this one

Robbins p973
• It is classically taught that, of the three major specific inflammatory states, gonorrhea and tuberculosis almost invariably arise in the epididymis, whereas syphilis affects first the testis.
• In sexually active men younger than age 35 years, the sexually transmitted pathogens Chlamydia trachomatis and Neisseria gonorrhoeae are the most frequent culprits.
• In men older than age 35, the common urinary tract pathogens, such as Escherichia coli and Pseudomonas, are responsible for most infections.

Mumps is stated infection which primarily affects testes; Primary orchitis is rare and caused by mumps (usually bilateral).

Question 27

2. 40 yo has staghorn calculus not present previous year, least likely cause is:
3. Hyperuricemia
4. Crohns disease
5. Hyper calciuria
6. Hyperoxaluria
7. Recurrent urinary tract infections

Answer 27

1. Hyperuricemia !!!!

Question 28

5. Regarding testicular tumours
6. Choriocarcinoma are large haemorrhagic masses
7. Spermatocystic seminoma occurs in 15-40 year olds
8. Seminoma is most common homogeneous testicular mass
9. Mixed tumours are rare in adults (<5%)
10. Teratoma is the second commonest adult tumour

Answer 28

3. Seminoma is most common homogenous testicular mass
4. Regarding testicular tumours
5. Choriocarcinoma are large haemorrhagic masses (Usually small lesions - < 5cm)
6. Spermatologic seminoma occurs in 15-40 year olds (> 65 years)
7. Seminoma is most common homogenous testicular mass
8. Mixed tumours are rare in adults (<5%) (60%)
9. teratoma second commonest adult tumour (Adults: Pure forms rare (2 – 3%), mixed more common (45%) ; Children: Pure forms common (2nd to yolk sac tumours))

Question 29

6. Nodular hyperplasia of prostate which is not true
7. 20% in men 40 years old
8. Occurs in periurethral and transitional zones
9. Demonstrates regions of infarction and metaplasia
10. Only 50% are symptomatic
11. Has 2-3 x increased risk of carcinoma

Answer 29

5. has 2-3X time have increase risk of carcinoma
6. Nodular hyperplasia of prostate which is not true
7. micro seen in 20% in men <40 years old (20% of 40yo men)
8. occurs in periurethral and transitional zones (Almost exclusively in periurethral and transitional zones of gland extending from bladder neck to verumontanum)
9. demonstate regions of infarction and metaplasia (Frequently, foci of squamous metaplasia and small areas of infarction)
10. only 50% are symptatic
11. has 2-3X time have increase risk of carcinoma

Question 30

7. 18.03.82 DEFINITION of Bowen disease ? define it

Answer 30

5. A persistent progressive non-elevated red scaly or crusted plaque which is due to an intradermal carcinoma and is potentially malignant

Question 31

1. HUS - ? most likely
2. Following viral infection
3. Kidneys may appear normal acutely

Answer 31

1. Following viral infection

Hemolytic uremic syndrome (HUS) is a multisystem thrombotic microangiopathic disease characterized by the triad of renal failure, hemolytic anemia and thrombocytopenia. It is the most common cause of renal failure in infancy and childhood requiring dialysis. There are two forms of this syndrome:typical or D+ HUS: corresponds to >90% of all HUS, occurs in childhood and is caused by Shiga toxin-producing Escherichia coliatypical or D- HUS: can occur after infections, use of certain drugs, following other pathologies (e.g. malignancy), or, rarely, due to defective cobalamin metabolism 3This article is based on the features related to the typical HUS. HUS affects ~ 2:100,000 people worldwide 3. Clinical presentationIt is most commonly seen in young children.Usually, there is an interval of 2-12 days between the ingestion of contaminated food and the first day of diarrhea and abdominal pain. After 1-3 days the diarrhea becomes bloody (~90%). Fever is usually absent. PathologyHUS usually occurs (>90%) following a gastrointestinal infection with Shiga toxin-producing E. coli (STEC). Some cases are associated with medical conditions such as collagen vascular conditions like SLE, underlying malignancy, or medications such as cyclosporine, OCP, or 5 FU. Injury to the endothelium of the capillaries results in mechanical destruction of the RBCs. Kidneys can be the only organ involved; however involvement of the other organ like liver, pancreas, heart, intestine, muscles also possible

**LJS - typically follows bacterial infection (E.coli > shigella). Kidneys echogenic and enlarged due to renal impairment.

Question 32

2. Patient with previous Goodpastures, on dialysis. Gross hematuria and hypotensive. ?cause
3. Prostate vol 25cc
4. Renal cysts
5. Renal calculi
6. Amyloid

Answer 32

*LW: Favour renal cysts with rupture secondary to dialysis induced renal cystic disease, causing acute haematuira and hypotension.

Previous answer:
4. Amyloid

Goodpasture syndrome, also referred as antiglomerular basement membrane (anti-GBM) antibody disease, is an autoimmune disease characterised by damage to the alveolar and renal glomerular basement membranes by a cytotoxic antibody. It is a type of pulmonary-renal syndrome.Goodpasture syndrome is defined by:- pulmonary haemorrhage- glomerulonephritis- circulating antiglomerular basement membrane antibodiesTreatment and prognosisThe overall prognosis is poor, although the use of immunosuppressive drugs and plasmapheresis has improved survival.Differential diagnosisOften radiograph and CT findings are indistinguishable from pulmonary oedema. Other causes of pulmonary haemorrhage should also be considered.

Question 33

3. Hypertensive patient most likely findings
4. 2cm adrenal Ca
5. 10cm adenoma
6. Bilateral benign tumours
7. Bilateral hyperplasia
8. Adrenal atrophy secondary to sustained HTN

Answer 33

3. Bilateral benign tumours (Adenomas are common, phaeos r bilat in 10%)

Question 34

4. Vascular 5cm renal mass found on investigation for RAS which was normal. ?Most likely
5. Incidental RCC
6. RCC as cause for hypertension
7. Oncocytoma
8. Need to check for urinary VMA before biopsy

Answer 34

1. Incidental RCC - RCC 80-90% of all primary renal tumours

**LJS - could also be renin secreting RCC. RCC has common paraneoplastic syndromes incl renin secretion

Question 35

16. 20year old with 6cm testicular tumour and raised bHCG. ?Most likely
17. Choriocarcinoma
18. Spermatocytic seminoma
19. Yolk Sac tumour
20. Mixed teratoma
21. Seminoma

Answer 35

• LW: Seminoma are common, and can produce hCG, and be large masses, hence my favoured answer
• *AJL: Robbins says Chorio is ‘rarely above 5cm’ therefore Seminoma is the best answer (agree with LW above) (tricky tricky examiners!)

1. Choriocarcinoma - are usually small, so less likely.
2. Spermatocytic seminoma - flase, elderly
3. Yolk Sac tumour - pure form rare in adults
4. Mixed teratoma -
5. Seminoma - Robbins states approx 15% of seminomas have syncytiotrophoblastas and hence can have elevated bHCG.

Previous answer:
I prefer choriocarcinoma given- appropriate age- bHCG increase but they prefer
5. Seminoma (50% of testicular tumourss, correct age group, 5-10% have elevated B HCG);

Question 36

17. Elderley patient with retroperitoneal soft tissue mass – Which is most likely
18. Rhabdomyosarcoma
19. Should consider fibrosarcoma
20. More likely to be malignant if retroperitoneal
21. Very unlikely to be synovial sarcoma as it is extraarticular
22. If has clear distinct margins it is unlikely to be a malignant tumour

Answer 36

• LW:
  RadPrimer states 80% of retroperitoneal masses / tumours are malignant. Hence my preferred answer is 3.
  Fibrosarcoma are usually in limbs.

**LJS - agree. Retroperitoneal fibrosarcoma is rare
Can get fibrosarcoma in various systems as malignant tumour of fibroblast origin (bone, breast, soft tissue, ovary etc). Still exists in 2015 WHO soft tissue tumour classification. Liposarcoma is most common retroperitoneal primary

2. Should consider fibrosarcoma – it is a common cause of a retroperitoneal mass

I am not sure if i agree with this
An inflammatory fibrosarcoma is a rare tumour with a varied biological behaviour, from benign to locally aggressive and occasionally malignant. It generally involves mesentery and retroperitoneal structures, but it rarely can involve the hollow viscera and commonly affects children and young adults.

Question 37

Newborn with multiple small renal cysts. Mother, father and sister to be imaged. Which is most likely? (? Unilateral, ? bilateral)

a. Mother, father and sister have normal imaging
b. M & F normal, sister may be abnormal
c. Sibling may have it
4. Both parents may have it

Answer 37

b. M & F normal, sister may be abnormal(Depending on age of sister)

Question 38

21. With regards to germ cell tumours, which is false?

Choriocarcinoma >5cm mass

Answer 38

choriocarcinoma is small

Question 39

Undescended testes are associated with

Answer 39

• reduced fertility
• increased risk of testicular cancer
• psychological problems when the boy is grown
• more susceptible to testicular torsion
• infarction
• inguinal hernias.

To reduce these risks, undescended testes are usually brought into the scrotum in infancy by a surgical procedure called an orchiopexy.

Question 40

22. BPH false:
23. 20% incidence male aged 40
24. rises in central zone
25. corpora amylacea – Ca within
26. Foci of squamous metaplasia and infarction

Answer 40

*AJL - I interpreted Ca as being calcium not cancer.
Radiopaedia says the corpora is ‘One of the key mechanisms for development of prostate calcifications’ and there is calcification in 10% of BPH. The 2 Robbins I checked (2018 basic and 2020 big) say BPH ‘virtually always occurs in the inner transition zone’, no mention is made of the central zone.
Therefore I agree with the original answer:
2. arises in central zone - FALSE arises in transition zone.

*LW: 3: corpora amylacea – Ca (cancer) within: FALSE
Robbins states: most hyperplastic lesions arise in the inner transitional and central zones (which is collectively referred to as the central gland - careful with wording!).
Areas of infarction common as is foci of squamous metaplasia.
Glandular lumen often contain inspissated proteinaceous secretory material termed Corpora Amylacea. No mention that this has cancer within it, however can calcify…. thus option 3 is my preferred answer (i.e it is false if referring to cancer), with option 2 being true (if corproa referring to cancer).
if option 3 corpora referring to calcification, then option two would then become my preferred option as incorrect.

Previous answer
2. rises in central zone F – arises in transitional zone

22. BPH false:
23. 20% incidence male aged 40 T – directly from Robbins
24. rises in central zone F – arises in transitional zone
25. corpora amylacea – Ca within T - are luminal secretions that calcify; Common in benign, rare in cancer
26. Foci of squamous metaplasia and infarction T – directly from Robbins (see below)

BPH: 20% of 40 y.o. / 70% by 60 / 90% by 70
CZ – 25% of normal prostate, surrounds ejaculatory ducts, accounts for 25% of cancers
TZ – 5% prostate volume, surrounds proximal urethra, responsible for BPH. Rare for tumours to develop here.

2 other histological changes: i) foci of squamous metaplasia; ii) small areas of infarction

Prostate cancer: No direct correlation between histo and clinical; only 50% of those with microscopic evidence have clinical symptoms. Arises exclusively in the inner aspect.

Question 41

22. Prostatic carcinoma – which is false:
23. 70% males b/w 70-80
24. Common in Asians
25. 80% have foci of PIN
26. Gleesons score correlates with well and poorly differentiated areas

Answer 41

2. Common in Asians F – extremely rare in Asians
   * AJL - Robbins says it is rare in people living in asian countries. People acquire the ‘western’ risk when they move to a western country eg USA.
3. Prostatic carcinoma:
4. 70% males b/w 70-80 T
5. Common in Asians F – extremely rare in Asians
6. 80% have foci of PIN T – directly from Robbins
7. Gleesons score correlates with well and poorly differentiated areas T

Question 42

5. Prostate cancer:
6. Well defined adenocarcinoma most common
7. <2% lymphatic involvement

Answer 42

1. Well defined adenocarcinoma most common
2. Prostate cancer:
3. Well defined adenocarcinoma most common
4. <2% lymphatic involvement - lymph node spread is common and often precedes bone mets

Question 43

6. BPH which is true:
7. 30-40% at 70 years
8. Severity correlates with symptoms
9. Macroscopic specimen nodular
10. If necrosis, malignant degeneration

Answer 43

3. Macroscopic specimen nodular T – nodules are fairly readily identifiable
4. BPH which is true:
5. 30-40% at 70 years F - >90% at age 70
6. Severity correlates with symptoms F
7. Macroscopic specimen nodular T – nodules are fairly readily identifiable
8. If necrosis, malignant degeneration F – foci of infarction

Question 44

7. TCC risks – least likely:
8. Schistosomiasis
9. Analgesic nephropathy
10. COP with lymphoma
11. Bowel Cancer – treated
12. Upper tract TCC

Answer 44

I like schitosomiasis -> SCC

7. TCC risks – least likely:
8. Schistosomiasis: correct answer, as Increased risk SCC , not TCC SG/*LW
9. Analgesic nephropathy T – analgesia usage
10. COP with lymphoma T - cyclophosphamide
11. Bowel Cancer – treated
12. Upper tract TCC T

Question 45

8. Kidney, 1yo with 3cm mass removed, pathology says nephrogenic rests, most correct:
9. Not a real entity
10. Increased risk of Wilm’s
11. Possible entity but not in this age group
12. Only micro diagnosis

Answer 45

2. Increased risk of Wilm’s (T)

Nephrogenic rests can also be classified into
i. perilobar (associated with Wilms)
ii. intralobar (associated with Drash syndrome and WAGR)
on the basis of location and the syndromes with which they are associated

Question 46

9. Oncocytoma, 5cms, 60 year old female – says benign, most correct:
10. 10% renal masses are this
11. Should be considered a low grade malignancy
12. Rare <1%

Answer 46

*LW:
Robbins: states accounts for 5-15% of resected tumours. Considered benign. So would favour option 1.

• repeated question. 5-15% renal tumours (acording to big robbins), but due to the difficulties distinguishing oncocytoma from low grade RCC (mainly on biopsy but in a some cases also on resection - radiopaedia + a path article) ‘should be considered low grade malignancy’ could also be the correct answer. (RY)
  2. Should be considered a low grade malignancy T – death from malignancy following sx 3%

9. Oncocytoma, 5cms, 60 year old female – says benign, most correct:
10. 10% renal masses are this - 5% of surgically resected tumours
11. Should be considered a low grade malignancy T – death from malignancy following sx 3%
12. Rare <1% - 2%

5% are multiple
Grade I - B9, grade II and III can metastasise.
Occur in salivary glands, kidneys, parathyroids, thyroid glands.

Question 47

10. Severe adult polycystic kidneys – least likely:
11. Bilateral elevated hemidiaphragms
12. Contour abnormality of the aorta
13. Basal cysts
14. Fluid overload
15. Hyperparathyroidism

Answer 47

3. Basal cysts
4. Severe adult polycystic kidneys – least likely:
5. Bilateral elevated hemidiaphragms (big kidneys)
6. Contour abnormality of the aorta (aortic abn – root dilataton, coarctation, AR, aneurysm)
7. Basal cysts
8. Fluid overload (renal failure)
9. Hyperparathyroidism (from renal failure)

Question 48

11. 20 week fetus, maternal grandparent has some sort of polycystic kidney disease, multiple cysts, variable size and decreased liquor:
12. ARPKD
13. ADPKD
14. MCKD

Answer 48

*LW: I favour MCKD:
If was ADPKD, although above info states can rarely occur in fetes, then there would be provided history of mother involvement of cystic disease given AD inheritance, although they don’t mention mothers age, one would assume maternal cysts / increased maternal renal size would have been seen on obstetric US.
ARPKD kidneys are more echogenic rather than cystic in fetus.
MCKD are variable sized cysts in a fetus that cause oligohydramnios. If question states single kidney is cystic, this would confirm MCKD, although MCKD can have contralateral anomaly / MCKD, the other options are always bilateral.

**LJS - agree. Though on the basis of MCKD referring to medullary cystic kidney disease (not multicystic dysplastic kidney - MCDK) - or is this a typo??
Most forms are AR inheritance, so mother could be unaffected, and there is an infantile form, has multiple small cysts (typically < 1.5cm), mostly at corticomedullary junction. Though not sure about the variable size thing. Terminology around subtypes seems to change in each Robbins edition
If MCDK were an option this might be better - though ?no oligo unless bilateral/contralateral other renal abn

Previous answer:
2. ADPKD (Can occasionally get very early onset in fetus, see: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=8074152&dopt=Abstract as well as many other articles in pubmed.)

11. 20 week fetus, maternal grandparent has some sort of polycystic kidney disease, multiple cysts, variable size and decreased liquor:
12. ARPKD (would not survive until old age)
13. ADPKD (Can occasionally get very early onset in fetus, see: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=8074152&dopt=Abstract as well as many other articles in pubmed.)
14. MCKD (Could be this one if grandfather is young see below.)

To be honest I’m not sure, could be B or C, but I favour B as I can’t find anything to say that the adult onset form would be detected in the fetus and the grandfather would have to be quite young, see below.
Medullary cystic disease
a cause of progressive renal impairment leading to uraemia which is characterized pathologically by a variable number of small cysts in the renal medulla, with tubular atrophy and interstitial fibrosis in the cortex.

Four recognized subtypes.
1) Sporadic or nonfamilial medullary cystic disease - 20% of cases.
2) Familial juvenile nephronophthis is an autosomal recessive condition - 50% of cases.
3) Renal - retinal dysplasia refers to a subtype associated with retinitis pigmentosa.
4) Adult medullary cystic disease accounts - 15% of cases - autosomal dominant disorder.
Young adults, uraemia and death within 2 years

Medullary cystic disease accounts for about 25% of cases of chronic renal failure in children and adolescents.
Slowly progresses to end-stage renal failure over 10 - 15 years.
At ultrasound, CT, MRI or intravenous urography (IVU), the kidneys are small to normal in size, with a relatively smooth outline.

IVU shows a thin renal cortex with thin linear collections radiating from the pyramids. However, IVU is rarely performed because of co-existent renal impairment. A variable number of small medullary cysts measuring up to 2 cm are seen on CT, US or MRI.

If the typical cysts are not identified the condition may go undiagnosed, because biopsy material may not include a sample of a medullary cyst, and because the histologic changes in the cortex are not specific.

Question 49

12. Testicle in 60 yo male,1 month mildly tender, vascular on USS, most likely:
13. Lymphoma
14. Choriocarcinoma
15. Classical seminoma
16. Spermatocytic seminoma
17. Mixed ?Embryonal

Answer 49

1. Lymphoma (most common form of testicular cancer in men >60)

Question 50

13. Small intratesticular mass, worst prognosis:
14. 20 yo with choriocarcinoma
15. Yolk sac
16. Seminoma
17. Teratoma in 5yo

Answer 50

1. 20 yo with choriocarcinoma - 80% 5 years

Question 51

14. Cushing’s syndrome: most common cause aside from exogenous treatment:
15. Pituitary adenoma – micro
16. Macroadenoma
17. Extopic ACTH
18. Adrenal hyperplasia
19. ?Adrenal adenoma

Answer 51

1. Pituitary adenoma – micro - 50+%
   * LW: RObbins states 70-80% of endogenous cases of hypercortisolism due to primary hypersecretion of ACTH.
2. Cushing’s syndrome: most common cause aside from exogenous treatment:
3. Pituitary adenoma – micro - 50+%
   Technically called Cushing Disease.
4. Macroadenoma
5. Ectopic ACTH - 80% of Cushing’s ACTH dependent i) exogenous, ii) paraneoplastic
6. Adrenal hyperplasia - 10-15%
7. ?Adrenal adenoma - > 25%

Question 52

15. Renal failure, secondary to amyloid, scan of kidney:
16. Large
17. Small
18. Normal
19. Renal artery stenosis
20. Multiple masses

Answer 52

*LW:
Robbins states characteristically kidney size tends to be either normal or increased. Advanced cases may be shrunken and contracted due to vascular narrowing.
So this confusingly allows options 1, 2, 3 to be correct.
Prof states normal or large, if associated with myeloma maybe more so, definitely not small.
So probable option large followed by normal would be best options.

RY* I would favour small, although depends on timing/severity.

• “Imaging findings tend to be non-specific and are not always present, but smaller and thinned cortex kidneys are seen in about 50% of patients with systemic amyloidosis. A note is made that, in acute stages, the kidneys may be enlarged.” - radiopaedia
• Aids to differential diagnosis also states “enlarged + echogenic in acute, with atrophy in chronic disease”.
• “Grossly, the kidneys may be of normal size and color, or, in advanced cases, they may be shrunken” Robbins pg 261
• but as a general rule “when amyloid accumulates in larger amounts, the organ is frequently enlarged and the tissue appears gray with a waxy, firm consistency” (pg260)

Previous answer:
Large

Question 53

1. Hypertension with increased VMAs
2. Neuroblastoma less than 50% occur in adrenals
   b) Flexner – Wintersteiner rosettes are classic in neuroblastoma
   c) Can also be found in Blueberry Muffin Syndrome, Pepper Syndrome, Chocolate Oreo Cookie Syndrome
   d) Prognosis is better in infants older than 12 months in advanced neuroblastoma (stage III and IV).
   e) In neuroblastoma, better prognosis is associated with deletions in chromosome 1, amplification of n-myc oncogene and elevated expression of Trk A.

Answer 53

1. Neuroblastoma less than 50% occur in adrenals: TRUE

Hypertension with increased VMAs (CC)

1. Neuroblastoma less than 50% occur in adrenals:
   36% unilateral adrenal, 10% bilateral adrenal,
   extra – adrenal sympathetic chain 18%,
   Pelvis (organ of Zuckerkandl) 5%, Hen’s teeth rare – intra-renal.
   Extra-abdominal:
   Thorax & posterior mediastinum 14 %
   Neck 5%
   Skull/esthesioneuroblastoma of olfactory bulb & cerebellum/cerebrum 2%
   Other sites 10% [Dahnert 6th ed P 941]
2. Flexner – Wintersteiner rosettes are classic in neuroblastoma
   ANSWER) False – Homer Wright pseudorosettes are found in neuroblastoma [Robbins Path P 460]

c) Can also be found in Blueberry Muffin Syndrome, Pepper Syndrome, Chocolate Oreo Cookie Syndrome
ANSWER) False – Blueberry muffin, Pepper syndrome and Hutchinson Syndrome can produce VMA [Dahnert 6th ed. P 941]

d) Prognosis is better in infants older than 12 months in advanced neuroblastoma (stage III and IV).
ANSWER) False, prognosis is higher in infants less than 12 months [Robbins 5th ed]

e) In neuroblastoma, better prognosis is associated with deletions in chromosome 1, amplification of n-myc oncogene and elevated expression of Trk A.

ANSWER) In neuroblastoma, WORSE prognosis is associated with deletions in chromosome 1, amplification of n-myc oncogene and elevated expression of Trk A [Robbins 5th ed.]
Added options b-e

Question 54

2. 18mo with homogenous testicular mass likely to be
3. Yolk sac tumour
4. Seminoma
5. Embryonal
6. Congenital infection
7. Lymphoma

Answer 54

1. Yolk sac tumour - True most common tumour in kids <3yrs. Also known as infantile embryonal carcinoma and endodermal sinus tumour.
2. 18mo with homogenous testicular mass is likely to be (CC)
3. Yolk sac tumour - True most common tumour in kids <3yrs. Also known as infantile embryonal carcinoma and endodermal sinus tumour.
4. Seminoma - False
   i. (2 types – typical (85%) and anaplastic (5 – 10 %) first peak in 30s
   ii. Spermocytic type seminoma >65yo, rarely produces metastases.
5. Embryonal - Embryonal carcinoma occurs mostly in the 20 – 20 age group
6. Congenital infection - infection not commonly congenital, and unusual without epididymitis. Adult infection can be bacterial or viral. Viral forms can sometimes have focal or diffuse orchitic enlargement – mumps (84% preceded by parotitis, coxsackie) [Dahnert 6th ed. P 942]
7. Lymphoma - testicular lymphoma accounts for 5% of testicular neoplasms and make up the most common form of cancer in men aged over 60.
   Added option e, and ‘is likely to be’ to question

Question 55

3. 10yo dies PCKD, cause
4. ICH
5. Hepatitis
6. CNS tumour
7. Pulmonary oedema
8. Amyloidosis

Answer 55

2. Hepatitis (Not quite true, but bad recall? – maybe should be hepatic fibrosis?)
3. 10yo dies PCKD, cause (CC)
4. ICH
5. Hepatitis (Not quite true, but bad recall? – maybe should be hepatic fibrosis?)
6. CNS tumour
7. Pulmonary oedema (secondary to liver failure??)
8. Amyloidosis

(CC) – I don’t really disagree with the final choice of the previous answer. Cause of death according to Robbins 6th ed. “ infants rapidly succumb to renal failure”, which can lead to pulmonary oedema from non cardiogenic etiology. However, Robbins also says in older children who survive infancy develop periportal fibrosis and proliferate well differentiated biliary ductules termed “ congenital hepatic fibrosis”. This eventually leads to portal hypertension, right heart failure and hence pulmonary oedema [Dahnert 6th ed p 946] in the juvenile spectrum of the disorder. So in this form of question D would be more correct. However, I would stick to Robbins since this is the source of most questions. If B said hepatic fibrosis then this would be correct. Alternatively if the patient was an infant rather than a 10 year old then D would be more correct than B as most infants succumb to renal failure, which leads to non cardiogenic failure.

Question 56

4. Amyloidosis which is NOT correct
5. 2-5% cause renal disease
6. Is associated with TB
7. 40% associated with haemodialysis
8. have types call lardaceous spleen and sago spleen
9. RA 5%

Answer 56

1. 2-5% cause renal disease - Amyloidosis of the kidney is the most common and cause of major morbidity [Robbins 5thed p 236]. Dahnert goes on to say primary amyloid is found in 35% and 85% in secondary renal amyloid, the latter would mostly be dialysis associated in first world populations
2. Amyloidosis which is NOT correct (CC)
3. 2-5% cause renal disease - Amyloidosis of the kidney is the most common and cause of major morbidity [Robbins 5thed p 236]. Dahnert goes on to say primary amyloid is found in 35% and 85% in secondary renal amyloid, the latter would mostly be dialysis associated in first world populations
4. Is associated with TB - Is associated with TB. Chronic inflammatory infective and non infective states is associated with AA type deposition from protracted cell breakdown including chronic osteomyelitis, rheumatoid arthritis, scleroderma, dermatomyositis [Robbins 6th ed]
5. 40% associated with haemodialysis – Robbins states 70% associated with haemodialysis [BR 6th ed ] however option A would be the most incorrect of the options.]
6. have types call lardaceous spleen and sago spleen - (T) – Robbins 6th ed
7. RA 5% - (in 3% of pts with RA – Robbins 5th ed p 234)

Renal disease is a frequent manifestation of the systemic amyloidoses and often is the major source of morbidity for individuals with these disorders. Without treatment, amyloidosis-associated kidney disease usually progresses to end-stage renal disease (ESRD). J Am Soc Nephrol 17: 3458–3471, 2006

Question 57

5. Regarding a man from middle east with schistosomiasis – which is least correct?
6. Periportal fibrosis
7. Ureteric fibrosis (uteric calcification)
8. Cirrhosis
9. Blindness from Mazzotti / Mazzotti-like reaction is rare
10. Chagoma entry site may be seen

Answer 57

5. Chagoma entry site may be seen – F – this applies to Chagas disease, not schistosomiasis. The statement is true regarding Chagas - Typanosoma cruzi of Chaga’s disease causes a transient erythematous nodule known as the “chagoma” [Robbins 5th ed. P370]
6. Regarding a man from middle east with schistosomiasis – which is least correct? (CC)
7. Periportal fibrosis - T -in 10% of heavily infected individuals also called pipe stem or Symmer fibrosis. {Robbins 6th ed]
8. Ureteric fibrosis – T - S. Haematobium can result in stenoses of the ureters and increased incidence of SCC bladder.
9. Cirrhosis - from granulomas, widespread fibrosis portal enlargement leading to portal hypertension. [Robbins 6th ed.] [ Dahnert - capsular tortoise shell calcifications]
10. Blindness from Mazotti / Mazzotti-like reaction is rare – T – this primarily relates to onchocerciasis (filiarisis) where Anti- filarial treatment in Onchocerciasis can accentuate punctate keratitis from microfilaria resulting in blindness. There is a case report of Mazzotti / Mazzotti-like reaction for schistosomiasis – very rare.
11. Chagoma entry site may be seen – F – this applies to Chagas disease, not schistosomiasis. The statement is true regarding Chagas - Typanosoma cruzi of Chaga’s disease causes a transient erythematous nodule known as the “chagoma” [Robbins 5th ed. P370]

Question 58

6. Old diabetic guy with addison’s
7. Hypothalamic / pituitary cause may be possible
8. Cutaneous hyperpigmentation is lacking
9. Autoimmune aetiology accounts for majority of cases
10. Reduction in serum glucocorticoids, with normal mineralocorticoid levels
11. TB is the most common cause

Answer 58

3. Autoimmune aetiology accounts for majority of cases – T – accounts for 60-70% of cases. May occur as a sporadic or familial disorder. May coexist with other autoimmune disease (eg Hashimoto, pernicious anemia, T1DM, idiopathic hypoparathyroidism)
4. Old diabetic guy with addison’s, which is true? (TW)
5. Hypothalamic / pituitary cause may be possible – F – Addisons is primary adrenocortical insufficiency with progressive destruction of adrenal cortex. Hypothal / pituitary cause is secondary.
6. Cutaneous hyperpigmentation is lacking – F – hyperpigmentation can be seen in Addisons. With decreased levels of glucocorticoids / mineralocorticoids there is increased ACTH precursor hormone levels which stimulate melanocytes. Melanotropic hormone levels are low in secondary adrenocortical insufficiency (ie suppressed ACTH).
7. Autoimmune aetiology accounts for majority of cases – T – accounts for 60-70% of cases. May occur as a sporadic or familial disorder. May coexist with other autoimmune disease (eg Hashimoto, pernicious anemia, T1DM, idiopathic hypoparathyroidism)
8. Reduction in serum glucocorticoids, with normal mineralocorticoid levels - F - >90% of adrenal cortex destroyed – levels of circulating glucocorticoids and mineralocorticoids are significantly decreased.
9. TB is the most common cause - F – previously this was the most common cause accounting for 90% cases – now less common with antituberculous agents – but is on the rise again.

Addisons
90% due to autoimmune attack on adrenal gland.
Other causes: lymphomas, amyloidosis, sarcoidosis, hemochromatosis (could also cause DM), fungal, adrenal hemorrhages and TB (which used to account for 90%)

Question 59

7. acromegaly 3cm adrenal lesion likely a
8. metastases
9. adenocarcinoma
10. incidental adenoma
11. Part of MEN II
12. Ganglioneuroblastoma

Answer 59

3. incidental adenoma – T - Adenomas in 1% of normal population [Dahnert 6th ed.]

Question 60

8. Which of the following does not cause renal impairment
9. NF2
10. vHL
11. Systemic sclerosis
12. TS
13. Sturge Weber

Answer 60

1. NF2 – T - multiple inherited schwannomas, meningiomas, ependymomas [Robbins 6th ed. ]
2. Which of the following does not cause renal impairment? (CC)
3. NF2 – T - multiple inherited schwannomas, meningiomas, ependymomas [Robbins 6th ed. ]
4. vHL – F – associated with multiple cysts in kidneys [Robbins 6th ed.]
5. Systemic sclerosis – F - development of malignant hypertension can result in fatal renal failure
6. TS – F - major features [Dahnert] 6th ed.]:
7. Cortical/subcortical tubers
8. Subependymal giant cell astrocytoma
9. Cardiac rhabdomyosarcoma
10. Facial angiofibroma
11. Renal hamartoma
12. Renal AML and multiple cysts, renal leiomyoma
13. Shagreen patches
14. Ash leaf spots
15. Lymphangioleiomyomatosis
16. Sturge Weber –F - not as true as option A [Robbins 5th ed p1163. However I note that hypertension can secondarily cause some renal impairment via the phaechromocytoma association. But this I think would be considered the lesser of the more correct option A. Dahnert 6th ed. also notes that SWS can results in angiomatous malformation of the kidneys although not as recognised as the encephalotrigeminal manifestation
    Added options b,c,d,e

Question 61

3. Incidence of AD polycystic kidney disease:
4. 1:100
5. 1:1000
6. 1:3000
7. 1:10000
8. 1:100000

Answer 61

2. 1:1000

Question 62

4. Focal renal infarct, what is least likely:
5. Aortic atherosclerosis
6. Mitral valve regurgitation
7. Post AMI
8. PAN
9. Candida endocarditis
10. Adult haemolytic ureamic syndrome

Answer 62

Answer is controversal either 2 or 6

*LW: Isolated MR would not cause renal infarcts. HUS cause microthrombi and thus would more likely cause focal renal infarcts, hence preferred option is 2: MR.

4. Focal renal infarct, what is least likely: (GC)
5. Aortic atherosclerosis T – thromboembolism
6. Mitral valve regurgitation
   I think F – MR is not a cause of embolism in itself, but could cause embolic renal infarction if the regurg is due to infective endocarditis.
7. Post myocardial infarction T – LV mural thrombus
8. Polyarteritis nodosa
   T – kidney involved in 70-90%. Focal panmural necrotising vasculitis – mucoid degeneration & fibrinoid necrosis begins in media – intimal proliferation & thrombosis.
9. Candida endocarditis T – embolism
10. Adult haemolytic uraemic syndrome
    Similar to childhood (classic) HUS but seen in association with:
    • Infection – verocytotoxic E.coli (O157:H7), shigella, viral
    • Postpartum

Classic triad:
1. Thrombocytopaenia
2. Microangiopathic haemolytic anaemia
3. Acute renal failure
Path: capillary & endothelial injury – mechanical damage to RBCs – formation of hyaline thrombi (predominantly glomeruli, but may extend into arterioles & larger arteries if severe) – cortical necrosis (fleabitten).
Several MCQ teams have answered that HUS is False as it causes cortical necrosis and not a focal renal infarct. Dahnert says focal infarction DOES occur.

Causes of renal infarction:
Most due to embolism
Major source of emboli = mural thrombi from LA & LV from AMI
Less common sources
• vegetative endocarditis
• thrombosis in aortic aneurysms and aortic atherosclerosis
Rare causes
• thrombosis in advanced atherosclerosis
• acute vasculitis of polyarteritis nodosa
Ref. Robbins, Adelaide path notes, Dahnert pg 929

• 2° HUS – scleroderma, SLE, malignant HTN, drugs etc.

Question 63

Neuroblastoma staging

Answer 63

International Neuroblastoma Staging System

Stage 1: Confined to adrenal gland
Stage 2: Extension beyond adrenal but does not cross midline
Stage 3: Extension across midline
Stage 4: Distant metastases
Stage 4s (special): Unique grouping of metastases, with excellent prognosisSkin, liver, and < 10% of bone marrow (not bone)

Question 64

5. ARPCKD – – Which is false?
6. not associated with berry aneurysms
7. Associated with bile duct cysts
8. Associated with liver fibrosis,
9. Associated with the PKHD1 gene, encoding fibrocystin
10. Histiology demonstrated dilatation of the collecting tubules

Answer 64

2. Associated with bile duct cysts F – ADPCKD RP says it is
   * LW: agree this is least correct, Robbins states proliferation of portal bile ducts, No mention of bile duct cysts.
3. ARPCKD – Which is false? (JS)
4. not associated with berry aneurysms T - ADPCKD
5. Associated with bile duct cysts F – ADPCKD RP says it is
6. Associated with liver fibrosis, T – Congenital hepatic fibrosis – periportal fibrosis and proliferataion of well-differentiated biliary ductules (Robbins)
7. Associated with the PKHD1 gene, encoding fibrocystin T
8. Histiology demonstrated dilatation of the collecting tubules T
   Added options d,e.

Question 65

7. Oncocytoma, 5cms, 60 year old female – says benign, most correct:
8. 10% renal masses are this
9. Should be considered a low grade malignancy
10. Rare <1%
11. Tends to be poorly encapsulated
12. Arises from the renal capsule

Answer 65

1. 10% renal masses are this T
2. Oncocytoma, 5cms, 60 year old female – says benign, most correct: (JS)
3. 10% renal masses are this T
   5-15% of surgically resected renal neoplasms are oncocytomas (Robbins)
4. Should be considered a low grade malignancy F
   Considered benign
5. Rare <1% F
   See answer a
6. Tends to be poorly encapsulated F
   Well encapsulated
7. Arises from the renal capsule F
   Epithelial tumour arising from intercalated cells of collecting ducts

*repeated question. Was answered as 2. should be considered a low grade malignancy. They stated death from malignancy following sx = 3% and oncocytoma only 5% of surgically resected tumours. Big Robbins has very little on renal oncocytoma, but does state 5-15% of renal tumours (p953). It is important to know they cannot be confidently distinguished pre-operatively from renal cell carcinomas on biopsy, hence they are surgically resected (Radiopaedia). A pathology article also stated sometimes difficult to classify as oncocytoma versus low grade RCC on resection (but better than biopsy). Hence 2. could also be the correct answer. (RY)

Question 66

10. Features of oncocytomas, which is false?
    a Yellow grey in colour.
    b Have a central scar.
    c Often detected incidentally on CT.
    d Contain eosinophilic cells, in cytoplasam.
    e Lack of calcification.

Answer 66

a Yellow grey in colour. (Tan or mahogany brown)

10. Features of oncocytomas, which is false? (AB)
    a Yellow grey in colour. FALSE(Tan or mahogany brown)

b Have a central scar. TRUE

c Often detected incidentally on CT. TRUE (account to 1 – 15% of surgically resected renal neoplasms)

d Contain eosinophilic cells, in cytoplasam. TRUE (composed of large eosinophillic cells with small round benign appearing nuclei having large nucleoli)

e Lack of calcification. TRUE Most oncocytomas do not calcify (a minority do)

Answer:
Renal oncocytomas are brown, benign, contain eosinophilic cells, and have a central stellate scar. They are often asymptomatic and are detected incidentally. Dystrophic calcification can occur, but is uncommon.
Reference: Radiologic-Pathologic Correlations From Head To Toe, 1st ed.
Added to Qu- ‘which is false?’

Question 67

10.	In relation to Renal cell carcinoma, which is false?
a Clear cell is commonest.
b Papillary type is hypervascular.
c Associated with smoking.
d Can present with fever.
e Can secret excess erythropoietin

Answer 67

b Papillary type is hypervascular.

Answer: B.

Clear cell accounts for 70-80% of RCC.
Smoking is the major risk factor, doubling incidence.
RCC can present with constitutional symptoms like fever, usually when the tumour is large.
It can secrete erythropoietin, causing polycythaemia.

Virtually all papillary type are hypovascular, compared to more than 75% of clear cell being hypervascular.
Reference: Robbins, 7th ed.; Radiologic-Pathologic Correlations From Head To Toe, 1st ed.

Question 68

13.	Phaeochromocytoma not a common location?
a Adrenal cortex. 
b Adrenal medulla. 
c Mediastinum.
d Bladder.
e Organ of Zuckerkandal.

Answer 68

a Adrenal cortex.

13. Phaeochromocytoma not a common location? (AB)
    a Adrenal cortex.
    b Adrenal medulla. This is cost common location 90%
    c Mediastinum.
    d Bladder.
    e Organ of Zuckerkandal. Most common extraadrenal location
    Answer: A. 90% of phaeochromocytomas occur in the adrenal medulla. Outside of this they are called paragangliomas, and occur in the sympahetic chain. The organ of Zuckerkandl is the most common extra-adrenal site. 1% occur in the thorax (mainly posterior mediastinal). They can enter urogenital organs like the bladder.
    Reference: MRI of Adrenal and Extraadrenal Pheochromocytoma, AJR, 2005.

Question 69

13.	CT request for patient with MEN 1 which is not a feature? 
a increased VMA. 
b Increased parathormone.
c GI mass.
d Pituitary mass.

Answer 69

a increased VMA

Answer: A. 
MEN-1 encompasses: 
parathyroid hyperplasia/adenomas; 
pituitary adenomas (usually prolactinomas); 
pancreatic endocrine tumours (gastrinoma, insulinoma); 
duodenal gastrinomas; 
carcinoid; 
thyroid and arenocortical adenomas. 

Phaeochromocytomas are a feature of MEN-2A and MEN-2B.
Reference: Robbins, 7th ed.

Question 70

13. Regarding angiomyolipoma, which is the least strongly associated?
14. Seizures
15. Angiofibromas of the face
16. Dystrophic Calcification
17. Retinal hamartoma

Answer 70

3. Dystrophic Calcification - Ca++ in AML’s is very rare

Answer: AMLs are associated with tuberous sclerosis (cortical tubers, subependymal harmatomas, retinal glial hamartomas, renal cysts, cutaneous angiofibromas). Mental retardation and seizures are features of TS. Retinal hamartomas may occasionally cause vitreous haemorrhage.
Reference: Robbins, 7th ed.; Radiologic-Pathologic Correlations From Head To Toe, 1st ed.; various ophthalmology sites re: bleeding retinal hamartomas.

Question 71

13. with regards to prostate cancer which is false
14. 70% of tumours are peripheral
15. Skull, clavicle, shoulder, T-spine, mets are less likely than pelivis or lumbar spine.
16. Spreads to para aortic nodes before regional nodes
17. Lymphatic spread before bone

Answer 71

3. Spreads to para aortic nodes before regional nodes

Answer: C. 70% peripheral involvement of prostate. Lymphatic spread initially to regional nodes. Later, haematogenous spread to bones - most commonly lumbar spine, pelvis, ribs, and femoral heads.
Reference: Imaging in Oncology from The University of Texas M. D. Anderson Cancer Center: Diagnosis, Staging, and Surveillance of Prostate Cancer, AJR, 2007.

Question 72

1. Renal cell Ca, which is true?
2. clear cell is commonest subtype
3. Papillary is very hypervascular on angio
4. Not associated with VHL
5. Papillary RCC share similar ontogenic features and histologic features with oncocytomas.
6. Renal medullary carcinoma tends to have a good prognosis

Answer 72

1. clear cell is commonest subtype T – Most common histologic subtype accounting for 70% of all RCCs. Previously known as conventional RCC.
2. Renal cell Ca, which is false? (TW)
3. clear cell is commonest subtype T – Most common histologic subtype accounting for 70% of all RCCs. Previously known as conventional RCC.
4. Papillary is very hypervascular on angio F - hypovascular
5. Not associated with VHL F – 96% of clear cell RCC are associated with 3p deletions including somatic inactivating mutations of the vHL gene.
6. Papillary RCC share similar ontogenic features and histologic features with oncocytomas. F – Chromophobe RCC share similar features with oncocytomas. Spoke wheel pattern of contrast enhancement described in both. Oncocytoma arise from type B intercalated ducts of collecting system, Chromophobe RCC postulated to differentiate toward type B intercalated cells of the collecting duct.
7. Renal medullary carcinoma tends to have a good prognosis - F – poor prognosis
   Radiographics 2006; 26: 1795.
   Added options d,e

Question 73

2. Regarding polyarteritis nodosa, which is the least correct:
3. The kidneys are most commonly involved at autopsy
4. Aneursyms are seen at angiography in 50%
5. 70% are seropositive for Hepatitis B surface antigen
6. Churg Strauss syndrome frequently involves pulmonary and splenic vessels
7. Serum antineutrophil antibody titres correlate with disease activity

Answer 73

3. 70% are seropositive for Hepatitis B surface antigen - F - PAN is associated with HBV and HIV antigenemia, however only up to 30% positive for HBsAg.
4. Regarding polyarteritis nodosa, which is the least correct: (TW)
5. The kidneys are most commonly involved at autopsy - T Kidneys involved 70-90%
6. Aneursyms are seen at angiography in 50% T – (take your pick of %) multiple (>10) aneuyrsms of small + medium sized arteries (50-60%). Andurysms are found in 12-94% of patients with PAN.
7. 70% are seropositive for Hepatitis B surface antigen - F - PAN is associated with HBV and HIV antigenemia, however only up to 30% positive for HBsAg.
8. Churg Strauss syndrome frequently involves pulmonary and splenic vessels – T CS syndrome vascular lesions may be histologically identical to those of classic PAN – however there is strong assoc with allergic rhinitis, asthma and eosinophilia. Vessels in lung, heart, spleen, peripheral nerves and skin frequently involved.
9. Serum antineutrophil antibody titres correlate with disease activity – T positive perinuclear ANCA titers.

(*AJL - ANCA is anti-neutophil cytoplasmic antibodies which is different to ANA (antineutrophil antibodies). PAN activity is related to p-ANCA (not ANA) therefore I think this may be the least correct.)

All Dahnert 6th, except d - Robbins

Question 74

1. Clear cells are not seen in…
2. Xanthogranulomatous pyelonephritis
3. Adrenal adenoma
4. Adrenal carcinoma
5. Wilms tumour .

Answer 74

1. Xanthogranulomatous pyelonephritis F – foamy histiocytes
2. Clear cells are not seen in… (TW)
3. Xanthogranulomatous pyelonephritis F – foamy histiocytes
4. Adrenal adenoma T – resembles zona fasciculate cells more than glomerulosa cells due to numerous lipid laden clear cells.
5. Adrenal carcinoma T – rare variant – clear cell adrenocortical carcinoma.
6. Wilms tumour – T hydropic cell variant (clear cell variant) of Wilms’ reported.

• XGPN – replacement of renal parenchyma with foamy histiocytes, occasional multinucleated giant cells and inflam. cells – appearances can mimic clear-cell RCC.
• several rare variants of adrenal cortical carcinoma – oncycytic adrenal cortical carcinoma, myxoid adrenal cortical carcinoma, clear cell adrenal cortical carcinoma
• Wilms’ tumor: triphasic with undifferentiated blastema. Report of clear cell variants (Arch Pathol Lab Med 1995).

Question 75

2. A retroperitoneal mass is discovered in a patient with a history of tuberous sclerosis. Which of the following is the most likely?
3. Myelolipoma
4. Hibernoma
5. Angiomyolipoma
6. Lipoma

Answer 75

3. Angiomyolipoma T – 80% TS have AML. Renal AML – low density retroperitoneal mass (Dahnert 6th)

• All the above options can occur in the retroperitoneum, but given Hx of TS – AML is most likely. When AML’s occur with TS they are typically multiple and bilateral.
• Extra-adrenal myelolipomas ~10% (with overall total ML prevelance of 0.08-0.2%).
• Hibernoma is a rare, benign, soft tissue tumor composed of brown fat.
• Lipomas can occur in retroperitoneum, but exceedingly rare.
(Radiographics 2009; 29). Added options a,b,d

Question 76

3. Which of the following could explain ARDS, hypertension, arterial thickening, hypothyroidism, pericarditis and renal cysts?
4. Maffucci’s syndrome
5. Amyloidosis
6. MEN Type 1
7. MEN Type 2
8. Dialysis and CRF

Answer 76

5. Dialysis and CRF – see below.
6. Which of the following could explain ARDS, hypertension, arterial thickening, hypothyroidism, pericarditis and renal cysts? (TW)
7. Maffucci’s syndrome Maffucci – nonhereditary Mesodermal dysplasia – enchondromas + multiple soft tissue cavernous hemangiomas and less commonly lymphangiomas
8. Amyloidosis
9. MEN Type 1 - MEN type 1 – PPP – Parathyroid hyperplasia, Pancreatic islet cell tumor, Pituitary (anterior). (for completion – MEN type 3 (type IIB) – MPM – medullary Ca thyroid, Phaeochromocytoma, Mucosal neuroma).
10. MEN Type 2 MEN type 2 (AKA type 2A) – PMP – Parathyroid hyperplasia, medullary carcinoma of thyroid, Pheochromocytoma.
11. Dialysis and CRF – see below.
    .
    - Amyloidosis – extracellular deposition of insoluble abnormal fibrils that injure tissue. Multiple associations. Can cause pericarditis. Renal involvement – amyloid deposited in renal interstitium and glomeruli – initially kidneys enlarged, then small in chronic stage.
    - Dialysis and CRF – develop acquired cystic disease (up to 80% patients on dialysis for 7y). CRF patients get HTN (sympathetic, Na+, RAS), vascular disease / accelerated atherosclerosis, Ca+/PO4 homeostasis. Prevalence of hypothyroidism is increased in CRF. Primary hyperthyroidism is extremely rare in pts with CRF (Oxford text clin nephrology). Pericarditis and asymptomatic pericardial effusion common (uremic and dialysis associated pericarditis).
    Dahnert, path notes, Ox text clin nephrology, Ox text medicine.
    Changed qu from hyperthyroidism to hypothyroidism. Even with hyperthyroidism in original question – still think ‘e’ is best option.

Question 77

5. Bilateral Ovarian Tumours
   a. Endometroid
   b. Mucinous cysadenocarcinoma
   c. Thecoma
   d. Dermoid cyst
   e. Brenner

Answer 77

*LW: per robbins
With regards to which ovarian neoplasm effects both ovaries / bilateral most commonly:
- Endometroid is most common tumour to show bilateral involvement - 40% of endometroid tumors are bilateral.
-dermoid: 10-15% are bilateral

Both represent 10-15% of total ovarian tumors.
So I would favour, out of these options, that per Robbins, Endometroid, is most likely bilateral.

**LJS - agree

d. Dermoid cyst - T - bilateral in up to 20%. Most common bilateral, along with serous cystadenoma and cystadenofibroma (12-20%)

5. Bilateral Ovarian Tumours (TW)
   a. Endometroid - bilateral 15%
   b. Mucinous cystadenocarcinoma - bilateral 5-10%, mucinous cystadenoma 5%
   c. Thecoma - 1% bilateral
   d. Dermoid cyst - T - bilateral in up to 20%. Most common bilateral, along with serous cystadenoma and cystadenofibroma (12-20%)
   e. Brenner - 5-7% bilateral. Almost always benign ovarian tumor.

Question 78

8. Small round cell tumour in kids – least likely
   a. Kidneys
   b. Bone
   c. Eye
   d. Testis
   e. Adrenal

Answer 78

d. Testis - F - but some can get desmoplastic small round tumor in a paratesticular location

8. Small round cell tumour in kids – least likely (TW)
   a. Kidneys - T - Wilms
   b. Bone - T - Ewings
   c. Eye - T - sheets, trabeculae and nest of small blue cells with scant cytoplasm (Retinoblastoma)
   d. Testis - F - but some can get desmoplastic small round tumor in a paratesticular location
   e. Adrenal - T - neuroblastoma

Owing to their primitive histologic appearance, many childhood tumors have been collectively referred to as small round blue cell tumors. These are characterised by sheets of cells with small, round nuclei. The differential diagnosis of such tumors includes neuroblastoma, lymphoma, rhabdomyosarcoma, Ewing sarcoma, and occasionally Wilms’ tumor

Question 79

9. Bladder CA – LEAST LIKELY
   a. Stones increased risk SCC
   b. Schistosomiasis increase risk SCC
   c. Urachal remnants Adeno carcinoma (think it said urethral?)
   d. Analine dye – TCC
   e. Flat CA better prognosis than papillary CA

Answer 79

e. Flat CA better prognosis than papillary CA - F - flat is bad

9. Bladder CA – LEAST LIKELY (TW)
   a. Stones increased risk SCC - T - metaplasia, with increased risk of SCC
   b. Schistosomiasis increase risk SCC - T
   c. Urachal remnants Adeno carcinoma (think it said urethral?) - T - urachal remnants are at increased risk of adenocarcinoma.
   d. Analine dye – TCC - T - most aromatic amines and their precurors or derivatives. Polycyclic aromatic hydrocarbons. Analine dye, arylamines.
   e. Flat CA better prognosis than papillary CA - F - flat is bad

Question 80

10. Testes – Most common Germ Cell Tumour
    a. Choriocarcinoma
    b. Seminoma
    c. Yolk sac
    d. Embryonal
    e. Sertoli-Leydig

Answer 80

b. Seminoma - T - 50% of GCT’s - of these 85% are typical, 10% anaplastic, and 5% spermatocytic.

10. Testes – Most common Germ Cell Tumour (TW)
    a. Choriocarcinoma - F - 1-3%. Most aggressive type of GCT. Present in about 10% of mixed GCTs, but is rare in its pure form in the nut.
    b. Seminoma - T - 50% of GCT’s - of these 85% are typical, 10% anaplastic, and 5% spermatocytic.
    c. Yolk sac - F - AKA endodermal sinus tumor. Pure yolk sac tumor is the most common malignant testicular GCT in pre-pubertal children. Pure YS is rare in adult, but occurs in 40% of mixed GCTs.
    d. Embryonal - F - 20-25% of NSGCTs (ie of the other 50%).
    e. Sertoli-Leydig - F - interstitial cell tumor (sex cord and stromal tumor)

GCTs account for about 95% of testicular cancers. Others are sex cord-stromal tumors (sertoli, leydig), plus secondary malignancy. These are divided into SGCTs and NSGCTs (as initial therapy is selected according to histology). Compared to NSGCTs, pure seminomas are more likely to be localized at presentation. Seminomas rarely spread via the blood stream to other areas that are more commonly involved by NSGCT. Seminomas are exquisitely sensitive to XRT, which NSGCT are more radioresistant.
Classic Seminoma: b-hCG elevated. AFP normal.
Pure embryonal: b-hCG elevated. AFP normal
Yolk Sac / endodermal sinus: AFP elevated. Do no produce hCG. Most common pre-pubertal GCT tumor. Shiller-Duval bodies.
Choriocarcinoma: b-hCG elevated. AFP normal. Most aggressive type of GCT.

Question 81

13. Infection not associated with testes
    a. Gonococcal
    b. HPV
    c. Mumps
    d. TB
    e. Syphilis

Answer 81

b. HPV - T - HPV is associated with the penis causing nasty warts.

13. Infection not associated with testes (JS)
    a. Gonococcal - F - extension of infection from urethera to prostate, seminal vesicles and epididymis.
    b. HPV - T - HPV is associated with the penis causing nasty warts.
    c. Mumps - F - testicular involvement is uncommon in school-aged children but orchitis occursin 20-30% of post pubertal males
    d. TB - F - begins in the epididymis and spreads to the testes
    e. Syphilis - F - involves both testes and epididymis but testes first

Question 82

14. Choriocarcinoma testicles which is false?
    a. High malignant
    b. <1% of tumours
    c. No enlargement of testes
    d. Comprised of pure synctiotrophoblasts
    e. Elevated B-HCG

Answer 82

d. Comprised of pure synctiotrophoblasts - F - composed of both syncytiotrophoblast and cytotrophoblast

14. Choriocarcinoma testicles which is false? (JS)
    a. High malignant - T
    b. <1% of tumours - T - in its pure form, constitutes less than 1% of germ cell tumours
    c. No enlargement of testes - T - usually small lesions with no testicular enlargement, detected as a small palpable nodule
    d. Comprised of pure synctiotrophoblasts - F - composed of both syncytiotrophoblast and cytotrophoblast
    e. Elevated B-HCG - T -

Question 83

27. Associated with sarcoidosis….
    a. Hypertrophic cardiomyopathy
    b. Proliferative glomerulonephritis
    c. Erythema marginatum
    d. Diabetes mellitus
    e. Loeffler’s syndrome

Answer 83

b. Proliferative glomerulonephritis - T - see below.

27. Associated with sarcoidosis…. (GC)
    a. Hypertrophic cardiomyopathy - F - restrictive cardiomyopathy Other cardiac manifestations: arrhythmia, heart block, failure.
    b. Proliferative glomerulonephritis - T - see below.
    c. Erythema marginatum - F - erythema nodosum (multiple bilateral tender erythematous nodules mostly on anterior tibia). EM occurs in rheumatic fever (Jones criteria).
    d. Diabetes mellitus - F - diabetes insipidus from HPA involvement. Other CNS manifestations: basal leptomeningeal involvement (diffuse or nodular), periventricular and deep WM T2 hyperintensities, cranial nerve palsies (7th>8,2,5), spinal cord granuloma.
    e. Loeffler’s syndrome - F - Lofgren’s syndrome (acute sarcoidosis) = fever + malaise + bilateral hilar lymphadenopathy, erythema nodosum, large joint arthralgia, occ. uveitis/parotitis.

Renal manifestations of sarcoidosis are commonly related to nephrocalcinosis, which results from hypercalcemia.
On occasion, the granulomatous process can directly involve the kidney, producing interstitial nephritis and glomerulonephritis. The major histological findings of renal sarcoid that have been described are: FSGS, membranous GN, mesangial proiferative GN, IgA nephropathy, and cresenteric GN. Most patients have proteinuria, clinical nephritic syndrome, and/or HTN. [RG 2004; Atlas of sarcoidosis 2005; Dahnert]
Added options a, c, d, e.

Question 84

31. Which of the following is the most common presentation of an angiomyolipoma?
    a. Haematuria
    b. Nephrotic syndrome
    c. Retroperitoneal haemorrhage
    d. Hypertension
    e. Renal failure

Answer 84

a. Haematuria - T - most asymptomatic. Flank pain, haematuria.

AML = renal choristoma (benign tumor composed of tissues not normally occurring within the organ of origin). AKA renal hamartoma. Small lesions are asymptomatic (60%). AMLs >4cm are symptomatic in 82-94%, and bleed spontaneously in 50-60%. Wunderlich syndrome = hemorrhagic shock due to massive bleeding into AML or in to retroperitoneum.

Question 85

39. Which tumour is associated with Schiller-Duval bodies?
    a. Spermatocytic seminoma
    b. Embryonal
    c. Yolk sac tumour
    d. Teratoma
    e. Choricarcinoma

Answer 85

c. Yolk sac tumour - T - AKA endodermal sinus tumor. Shiller-Duval bodies. Pure yolk sac tumor is the most common malignant testicular GCT in pre-pubertal children. Pure YS is rare in adult, but occurs in 40% of mixed GCTs.

39. Which tumour is associated with Schiller-Duval bodies? (TW)
    a. Spermatocytic seminoma - F - subtype of seminoma, tends to occur in older men 50-60yo. Better prognosis.
    b. Embryonal - F - 20-25% of NSGCTs (ie of the other 50%).
    c. Yolk sac tumour - T - AKA endodermal sinus tumor. Shiller-Duval bodies. Pure yolk sac tumor is the most common malignant testicular GCT in pre-pubertal children. Pure YS is rare in adult, but occurs in 40% of mixed GCTs.
    d. Teratoma - F - 4-10% of GCT. 2nd most common testicular tumour in young boys 5yrs or less.
    e. Choricarcinoma - F - 1-3%. Most aggressive type of GCT. Present in about 10% of mixed GCTs, but is rare in its pure form in the nut
    Classic Seminoma: b-hCG elevated. AFP normal.
    Pure embryonal: b-hCG elevated. AFP normal
    Yolk Sac / endodermal sinus: AFP elevated. Do no produce hCG. Most common pre-pubertal GCT tumor.
    Choriocarcinoma: b-hCG elevated. AFP normal. Most aggressive type of GCT.

Question 86

53. Which is true regarding cervical cancer?
    a. Extension into the upper vagina is associated with a poor prognosis
    b. Adenocarcinoma has a worse prognosis than squamous cell carcinoma
    c. An early lesion looks like a fungating ulcer

Answer 86

b. Adenocarcinoma has a worse prognosis than squamous cell carcinoma - T - AdenoCa is derived from the mucus-secreting columnar epithelium of the cervical canal; comprises about 5-10% of all primary malignant tumours of the cervix. Compared with SCC it has a worse prognosis. [Adelaide notes]

53. Which is true regarding cervical cancer? (GC)
    a. Extension into the upper vagina is associated with a poor prognosis - F- still surgically manageable (stage IIA), 5YS 82%.
    b. Adenocarcinoma has a worse prognosis than squamous cell carcinoma - T - AdenoCa is derived from the mucus-secreting columnar epithelium of the cervical canal; comprises about 5-10% of all primary malignant tumours of the cervix. Compared with SCC it has a worse prognosis. [Adelaide notes]
    c. An early lesion looks like a fungating ulcer - F - invasive Ca develops in the transformation zone, manifests in 3 patterns: fungating (or exophytic), ulcerating (see below), and infiltrating cancer. Macroscopically, early lesions appear as granular irregular areas of the cervical epithelium. Late lesions appear as fungating ulcerated areas which destroy the cervix [Robbins; Adelaide notes]

FIGO Staging:
0 Ca in-situ - (100% 5YS)
I confined to cervix - (90%)
II extends beyond uterus but not to pelvic wall or lower ⅓ of vagina - (82%)
–IIA into upper vagina (surgical)
–IIB parametrial involvement (non-surgical) - (Dahnert gives 5YS 65% for IIB)
III to lower ⅓ vagina, or pelvic wall, or hydronephrosis - (35%)
IV beyond true pelvis (rectum/bladder, distant mets) - (10%)

Question 87

54. A mass visualised in the upper vagina is least likely to represent a….
    a. Endocervical polyp
    b. Endometrial polyp
    c. Nabothian cyst
    d. Rhabdomyosarcoma
    e. Endocervical cancer

Answer 87

e. Endocervical cancer - F - tends to extend into corpus and lateral parametrium, rather than exocervical lesions that invade the upper part of the vagina. Endophytic growth pattern is more common in older women, since the transformation zone “retracts” (squamocolumnar jxn restored to original site, as at birth). Cervical ectropion in younger women accounts for majority of these cancers being exophytic.

54. A mass visualised in the upper vagina is least likely to represent a…. (GC)
    a. Endocervical polyp - T - usually pedunculated; most commonly appear as a mass with or without cysts filling the endocervical canal or vaginal canal. Account for 60% or cervical polypoid lesions.
    b. Endometrial polyp - T - frequently associated with tamoxifen therapy. Pedunculated polyps may extend through cervix.
    c. Nabothian cyst - T - retention cyst formed as a result of the healing process of chronic cervicitis. Tunnel cluster is a special type of NC, characterised by complex multicystic dilatation of the endocervical glands (macroscopically appears similar to adenoma malignum, but doesn’t show any atypia).
    d. Rhabdomyosarcoma - T - children under the age of 5yrs; embryonal (most commonly the botryoid subtype).
    e. Endocervical cancer - F - tends to extend into corpus and lateral parametrium, rather than exocervical lesions that invade the upper part of the vagina. Endophytic growth pattern is more common in older women, since the transformation zone “retracts” (squamocolumnar jxn restored to original site, as at birth). Cervical ectropion in younger women accounts for majority of these cancers being exophytic.
    [Robbins; MR imaging of uterine cervix RG 2003]

Question 88

55. Which is true regarding Bowen’s disease of penis?
    a. It is associated with HPV infection
    b. Cowden’s disease
    c. Turcot’s

Answer 88

a. It is associated with HPV infection – T – most commonly type 16.

55. Which is true regarding Bowen’s disease of penis? (GC & TW)
    a. It is associated with HPV infection – T – most commonly type 16.
    b. Cowden’s disease - F - multiple hamartoma syndrome (increased breast cancer risk)
    c. Turcot’s - F - FAP variant: adenomatous colonic polyposis and tumors of CNS (mostly gliomas). [cf Gardners: variation of FAP, multiple osteomas, epidermal cysts, fibromatosis]
    • In external male genitalia, 3 lesions that display histologic features of Ca in situ have been described: Bowen disease, erythroplasia of Queyrat, and bowenoid papulosis. All these have strong association with infection by HPV (type 16 in 80%).
56. Bowen disease: occurs in the genital region of men and women, usually in >35yo. In men, it is prone to involve the skin of the shaft of the penis and scrotum. Over the span of years, Bowen disease may become invasive and transform into typical SCC in approx 10% of patients.
57. Erythroplasia of Queyrat: on the glans and prepuce.
58. Bowenoid papulosis: younger age than Bowen’s and multiple (cf. solitary), pigmented reddish-brown papular lesions.

Question 89

61. VHL is associated with all of the following except…
    a. Clear cell RCC
    b. Papillary RCC
    c. Retinal angiomas
    d. Pheochromocytomas
    e. 3p deletions

Answer 89

b. Papillary cell RCC - F - RCCs of predominant papillary, chromophobe, or oncocytic histology are not associated with vHL disease (UTD). pRCC is the 2nd most common subtype, and most common multifocal or bilateral renal tumor. Most pRCCs are sporadic.

61. VHL is associated with all of the following except… (TW)
    a. Clear cell RCC - T - all vHL-associated RCCs are clear cell tumors, although these clear cell tumors may contain minor papillary components (usually <25% of the lesion).
    b. Papillary cell RCC - F - RCCs of predominant papillary, chromophobe, or oncocytic histology are not associated with vHL disease (UTD). pRCC is the 2nd most common subtype, and most common multifocal or bilateral renal tumor. Most pRCCs are sporadic.
    c. Retinal angiomas - T - see below
    d. Pheochromocytomas - T - vHL phenotype: pheochromocytoma (frequently bilateral), paraganglioma (rarely), retinal angioma, cerebellar hemangioblastoma, epididymal cystadenoma, renal and pancreatic cysts, pancreatic neuroendocrine tumors, and renal cell carcinoma.
    e. 3p deletions - T - vHL gene Ch 3p25. Specific germline mutations or deletions of the vHL gene can influence the clinical manifestations of vHL disease

Question 90

62. Which is true regarding HSV?
    a. HSV1 encephalitis is most common in older adults
    b. It is grossly necrotising and often haemorrhagic
    c. HSV1 is the commonest cause of herpes meningitis

Answer 90

b. It is grossly necrotising and often haemorrhagic - T - hemorrhagic, necrotizing encephalitis of gray and white matter (primarily limbic system). Severe edema an dmassive tissue necrosis with hemorrhage typical.

62. Which is true regarding HSV? (TW)
    a. HSV1 encephalitis is most common in older adults - F - occurs in any age, but most common in children and young adults.
    b. It is grossly necrotising and often haemorrhagic - T - hemorrhagic, necrotizing encephalitis of gray and white matter (primarily limbic system). Severe edema an dmassive tissue necrosis with hemorrhage typical.
    c. HSV1 is the commonest cause of herpes meningitis - F - HSV-2 is responsible for most cases of herpetic viral meningitis (Path notes). HSV-1 causes 95% of all herpetic encephalitis.

Question 91

64. Which is the least common renal tumour in children?
    a. Clear cell sarcoma
    b. Clear cell carcinoma
    c. Wilms tumour
    d. Rhabdoid
    e. Mesoblastic nephroma

Answer 91

b. Clear cell carcinoma – F – in contrast to adults, renal cell carcinoma is rare in childhood. Classification of RCCs: CCC 75-85%; Chromophilic (papillary) 10-15%; Chromophobic 5-10%; Oncocytic uncommon; Collecting duct (Bellini’s duct) very rare

64. Which is the least common renal tumour in children? (TW)
    a. Clear cell sarcoma – T – second most common pediatric renal tumor. Less favorable prognosis than Wilm’s.
    b. Clear cell carcinoma – F – in contrast to adults, renal cell carcinoma is rare in childhood. Classification of RCCs: CCC 75-85%; Chromophilic (papillary) 10-15%; Chromophobic 5-10%; Oncocytic uncommon; Collecting duct (Bellini’s duct) very rare.
    c. Wilms tumour – T – most common renal malignancy in children
    d. Rhabdoid – T – Rhabdoid tumor of the kidney is a highly malignant renal tumor, occurs most frequently in children less than 2yo, and almost never in those older than 5yo.
    e. Mesoblastic nephroma – T – most common renal mass encountered in neonates. Typically encountered during first few months of life (mean age Dx 3/12). Fetal renal hamartoma.

Question 92

68. Phaeochromocytoma is not associated with…
    a. Elevated VMA
    b. Headache
    c. Retroperitoneal mass
    d. Nephritic syndrome

Answer 92

d. Nephritic syndrome - F - characterised by haematuria, proteinuria (small), hypertenon, uremia. Variable renal insufficiency.

68. Phaeochromocytoma is not associated with… (TW)
    a. Elevated VMA - T - vanillylmandelic acid is a catecholamine metabolite. Has poor diagnostic sensitivity and specificity compared with fractioned 24hr urinary fractionated metanephrines. Some groups advocate fractionated plasma free metanephrines as a first line test for pheochromocytoma.
    b. Headache - T - classic triad of symptoms of phaeochromocytoma consists of episodic headache, sweating, and tachycardia.
    c. Retroperitoneal mass - T - anatomy.
    d. Nephritic syndrome - F - characterised by haematuria, proteinuria (small), hypertenon, uremia. Variable renal insufficiency.

Question 93

105. Phaeochromocytoma not associated with
     a. MEN
     b. VHL
     c. Sarcoidosis
     d. NF
     e. Can’t remember e

Answer 93

c. Sarcoidosis - F

105. Phaeochromocytoma not associated with (TW)
     a. MEN - T - MEN2A: P-M-P (pheochromocytoma, medullary carcinoma thyroid, phyerparathyroidism due to primary parathyroid hyperplasia). MEN2B / 3: M-P-MN (medullary carcinoma thyroid, pheo, mucosal neuromas / intestinal ganglioneuromas, marfanoid). Chromosome 10
     b. VHL - T - two primary familial disorders associated with pheochromocytoma, both autosomal dominant inheritance: vHL and MEN2. vHL phenotype: pheochromocytoma (frequently bilateral), paraganglioma (rarely), retinal angioma, cerebellar hemangioblastoma, epididymal cystadenoma, renal and pancreatic cysts, pancreatic neuroendocrine tumors, and renal cell carcinoma. Chromosome 3p25
     c. Sarcoidosis - F
     d. NF - T - AD, chromosome 17, Approx 2% of pts with NF1 develop catecholamine-secreting tumors (usually a solitary benign adrenal pheochromocytoma).
     e. Can’t remember e