path recall August 2014 WA - formatted

Question 1

Pneumothorax least likely

1. Alpha 1 antitrypsin
2. Ehler Danlos
3. Marfan
4. Kleinfelters
5. Homocystinuria

Answer 1

4. Kleinfelters Pneumothorax least likely
5. Alpha 1 antitrypsin - emphysema
6. Ehler Danlos - emphysema
7. Marfan - emphysema
8. Kleinfelters
9. Homocystinuria - spontaneous pneumothorax

Question 2

Small cell lung carcinoma

1. Typical carcinoid is a precursor
2. Associated with raised PTH and calcium

Answer 2

2. Associated with raised PTH and calcium
   *LW
   Well PTHrP, although true, paraneoplasic PTHrP and HyperCa++ more common in SCC.

paraneoplastic syndrome- hypoglycaemia- hypercalcaemia- hyperparathyroidism- SIADH- limbic encephalitis

Question 3

Constrictive pericarditis is most likely caused by: (repeat) A. Radiotherapy

Answer 3

Idiopathic most common, surgery second, radiotherapy 3rd, infection, Sarcoid, renal failures rheumatic fever (rare), SLE (rare)

Question 4

Diastolic dysfunction most likely caused by: (repeat)

1. Hypertension
2. Constrictive pericarditis
3. Diabetes

Answer 4

1. Hypertension
2. Hypertension
3. Constrictive pericarditis less common
4. Diabetes

Question 5

What is implicated in coronary artery disease?

1. Lupus anticoagulant
2. Some gene…

Answer 5

What is implicated in coronary artery disease?

1. Lupus anticoagulant associated with MI, but not atherosclerosis
2. Some gene… not sure of the answerlupus anticoagulant - antibody against cell membrane phospholipid and glycoprotein- procoagulant state

Question 6

What isn’t caused by fibromuscular dysplasia?

1. Pulsatile tinnitus
2. Angina
3. TIA
4. Mesenteric ischaemia

Answer 6

All correct

Question 7

What is associated?

1. Smoking and RB-ILD
2. Granulomas and immune complexes in Wegners

Answer 7

1. Smoking and RB-ILD

Question 8

Cardiac neoplasms are associated with:

1. Valvular problems
2. Emboli
3. Pericardial effusion

Answer 8

1. Valvular problems
2. Emboli
   both true

Question 9

H. Pylori is associated with?

1. Gastric cancer
2. Mantle cell lymphoma marginal zone (MALToma)
3. Decrease vitamin B12
4. Hyperplastic polyp
5. Duodenal villous atrophy

Answer 9

1. Gastric cancer

**LJS - ass/w intestinal metaplasia which increases risk for adenocarcinoma
Also induces mucosa-associated lymphoid tissue, which has the potential to transform into MALToma (Robbins)

Question 10

Carcinoid acts most aggressive where:

1. Oesophagus
2. Appendix
3. Stomach
4. Colon
5. Terminal ileum

Answer 10

5. Terminal ileum

Small bowel most aggressive. Foregut and hindgut (+appendix incidental/least aggressive)

Question 11

Cholangiocarcinoma risk factors:

1. Hepatitis B Chronic
2. Male or female (?)
3. Hepatic fibrosis
4. Primary biliary cirrhosis

Answer 11

1. Hepatitis B Chronic

**LJS - and hepatic fibrosis - fibropolycystic liver disease spectrum, incl Caroli, biliary hamartomas, congenital hepatic fibrosis etc)

Risk for cholangiocarcinoma:
- primary sclerosing cholangitis (PSC)
- recurrent pyogenic cholangitis (hepatolithiasis)
- choledocholithiasis more than cholelithiasis 10,11
- Clonorchis sinensis
- Caroli disease / choledochal cystslifetime risk of 10-15% 2toxins
- thorotrast
- dioxin- polyvinyl chloride
- heavy alcohol use
viral infection(s)- HIV- hepatitis B- hepatitis C- EBV

Question 12

Polyarthralgia and skin fat necrosis caused by:

1. Gastrinoma
2. Somatostatinoma
3. Islet cell tumour
4. Ductal Adenocarcinoma
5. Glucagonoma

Answer 12

4. Ductal Adenocarcinoma – lipase secretion syndrome

Polyarthralgia and skin fat necrosis caused by:

1. Gastrinoma ZE
2. Somatostatinoma DM, diarrhoea, cholelithiasis
3. Islet cell tumour
4. Ductal Adenocarcinoma – lipase secretion syndrome
5. Glucagonoma – 4D’s: dermatitis, DM, DVT, depression

Question 13

Adrenal Corticocarcinoma:

1. 11-hydroxylase
2. 17-hydroxylase
3. Can resemble adrenal hyperplasia

Answer 13

3. Can resemble adrenal hyperplasia I guess?

**LJS - I’m not sure what this question is asking. But I don’t think CAH (normal adrenals, or bilateral increased thickness with normal shape) resembles adrenal cortical carcinoma (often large at dx, heterogenous, necrosis, haemorrhage, vascular invasion).

60% adrenal cortical carcinomas are functional. 11 beta-hydroxylase and 17 alpha hydroxylase are both enzymes in the adrenocortical hormone synthesis pathway

Question 14

Anal carcinoma staging with ipsilateral iliac or inguinal lymph nodes:

A. 1
B. 2
C. 3A
D. 3B

Answer 14

T?N1Has to be stage 3- but definitely not stage 3b (T4N0 - i.e no nodes)
3a - T12N1
3b - T4n0
3c - T34 N1

**LJS - of answers given, can only be 3A (3B is T4, N0, M0)

Stage 0 - carcinoma in situ, no nodes or mets
Stage 1 - Tumour < 2cm (T1), no LN or mets
Stage 2 - T2 or T3, no LN or mets
Stage 3A - T1 or T2, N1, no mets
Stage 3B - T4 (into adjacent organs), no LN or mets
Stage 3C - T3 or T4, N1, no mets
Stage 4 - Mets

Question 15

Splenectomy causes what bacterial susceptibility:

1. H. Influenza
2. Tuberculosis
3. Mycosis fungoides

Answer 15

1. H. Influenza

Question 16

Which affects both the liver and the skin:

1. Alpha-1 antitrypsin deficiency
2. Wilson’s disease

Answer 16

*LW:
Both technically true per UpToDate:
- Aplha 1 skin manifestations include: necrotising panniculitis, systemic vasculitis, psoriasis, and angio oedema.
- Wilsons skin manifestations include: blue lunulae (lunulae ceruleae), acanthosis nigricans, and pretibial hyperpigmentation. (Dermatologic manifestations may also occur from treatment with penicillamine)

Previous answer:
2. Wilson’s disease

Question 17

Which of these is not associated?

A. VIPoma with diarrhoea and raised K+

Answer 17

A. VIPoma with diarrhoea and raised K+ - hypokalaemia

insulinoma - low glucose
somatostinoma - DM, diarrhoea, gallstone
Gastrinoma - ellison zollinger
VIPoma - WHDA - watery diarrhoea, hypokalaemia, achorydia
glucagonoma - DM, DVT, depression, dermatitis,

Question 18

Causes of fulminant hepatitis:

A. Autoimmune
B. Hepatitis B
C. Carbon tetrachloride

Answer 18

**LJS - Robbins says paracetamol OD most common in USA, and other causes include:
-AI hepatitis
-Other drugs/toxins
-Acute hep A and B - vriral hepatitis accounts for 10% cases of acute hepatic failure
(Hep B and E most common cause in Asia)

So all are true, and according to Robbins Hep B NOT most common (though radiopedia says it’s most common)

*LW:
Just further info, not really helping….
UTD
- Viral and drug-induced hepatitis are the most common causes of acute liver failure in adults.
- Drug-induced liver injury is the most common cause of acute liver failure in Australia, Europe, the United Kingdom, and the United States, whereas in Asia and Africa, viral hepatitis predominates
- In the United States, The most common causes of acute liver failure were acetaminophen overdose (46 percent), indeterminate (14 percent), idiosyncratic drug reactions (12 percent), hepatitis B virus (7 percent), and hepatitis A virus (3 percent).

Previous answer:

Hepatitis B - most common
others are true
Also idiopathic, drugs, mushrooms, metabolic disease, HELLP, Bud-chiari, shock, RHF
Acute hepatitis = fulminant hepatitis- acute loss of hepatic function without chronic liver failure
Symptoms- encephalopathy- coagulopathy
Cause- vascular : BC, RHF- infection : hep B (most common)- idiopathic- Toxin: alcohol, paracetamol, muschroom- autoimmune: - metabolic : wilson, pregnancy steatohepatitis,

Question 19

What does analgesic nephropathy cause?

A. Renal papillary necrosis

Answer 19

A. Renal papillary necrosis

Question 20

Deficiency of which of these does not cause neurological issues?

1. B1 (thiamin)
2. B2 (riboflavin)
3. B3 deficiency
4. B12
5. Vitamin E

Answer 20

*AJL - I think B2 is the correct answer.
Called Ariboflavinosis which causes inflammation of mucosal membranes.
Hypovitaminosis E causes dysfunction of the CNS with ataxia and tremor.

**LJS - agree.

Previous answer…
5. Vitamin E

Deficiency of which of these does not cause neurological issues?

1. B1 (thiamin) Wernickes
2. B2 (riboflavin) although assoc with deficiencies in other B vitamins (acts as a cofactors)
3. B3 deficiency causes pellagra – CNS, skin, GIT = dementia, dermatitis, diarrhoea
4. B12 subactue combined degeneration
5. Vitamin E

Question 21

Which does not cause hydrocephalus?

1. Choroid plexus papilloma
2. Central neurocytoma
3. Ependymoma
4. Medulloblastoma
5. PXA (maybe ganglioglioblastoma)

Answer 21

5. PXA (maybe ganglioglioblastoma)

Question 22

Which is graded WHO I:

1. DNET
2. Ganglioglioma
3. Ependymoma
4. Central neurocytoma
5. Pilomyxoid astrocytoma

Answer 22

1. DNET WHO 1
2. Ganglioglioma - 80% WHO 1
3. Ependymoma II
4. Central neurocytoma II
5. Pilomyxoid astrocytoma II

Question 23

What is most likely to recur post surgery?

1. Dentigerous cyst
2. Ameloblastoma
3. Odontoma
4. Periapical cyst
5. Keratocystodontonic cyst

Answer 23

2. Ameloblastoma 50-90%

5. Keratocystodontonic cyst 50% recur

Question 24

Which is not a feature of NF2?

1. Ependymoma
2. Meningioma
3. Schwannoma
4. Optic glioma

Answer 24

4. Optic glioma

Question 25

What is caused by HPV in the nasal cavity? 1. NK/T 2. Papilloma 3. Nasopharyngeal carcinoma 4. Juvenile angiofibroma

Answer 25

3. Nasopharyngeal carcinoma **LJS - disagree. NPC ass/w EBV. Sinonasal papillomas (e.g. inverted papilloma) ass/w low risk HPV subtypes - big Rob 9th ed

Question 26

Which is diaphyseal in location? A. Osteoblastoma 2. Chondromyxoid fibroma 3. Osteochondroma 4. Chondroblastoma 5. Non-ossifying fibroma

Answer 26

A. Osteoblastoma – maybe more common in spine, metaphysis and distal diaphysis in long bones ``` Which is diaphyseal in location? A. Osteoblastoma – maybe more common in spine, metaphysis and distal diaphysis in long bones 2. Chondromyxoid fibroma metaphyseal 3. Osteochondroma metaphyseal 4. Chondroblastoma epiphyseal 5. Non-ossifying fibroma metaphyseal ```

Question 27

Frontal bossing is not seen with: 1. Thanatophoric dwarfism 2. Acromegaly 3. Hurler 4. Alpha thalassemia 5. Cliedo cranial dysplasia

Answer 27

*LW: Alpha thalassemia - FALSE, listed is Beta thalaemiassia. (although I am likely wrong) 1. Thanatophoric dwarfism - TRUE 2. Acromegaly - TRUE 3. Hurler - TRUE 4. Alpha thalassemia - FALSE, listed is Beta thalaemiassia. 5. Cliedo cranial dysplasia - TRUE This feature can be seen in many conditions (in alphabetical order): ``` 18q syndrome acromegaly achondroplasia ß-thalassaemia major cleidocranial dysostosis Gorlin-Goltz syndrome Greig cephalopolysyndactyly syndrome Hurler syndrome hydrocephalus (typically before the age of two) pyknodysostosis Russell-Silver dwarfism thanatophoric dysplasia ``` All can I think

Question 28

What is the commonest soft tissue sarcoma in the extremities? 1. Eosinophilic fibrosis 2. Myxoid 3. Pleomorphic 4. Synovial sarcoma

Answer 28

3. Pleomorphic most common *AJL - referring to undifferentiated pleomorphic sarcoma. 1st = UPS 2nd = liposarc

Question 29

Which sarcoma is least likely in a peripheral location in deep tissue? 1. Liposarcoma 2. Synovial sarcoma 3. Angiosarcoma

Answer 29

3. Angiosarcoma rare/. Can occur there but probably least likely. Which sarcoma is least likely in a peripheral location in deep tissue? 1. Liposarcoma second most common behind PUS 2. Synovial sarcoma 4th most common 3. Angiosarcoma rare/. Can occur there but probably least likely.

Question 30

Which is a mitochondrial inherited disorder? 1. Adrenoleukodystrophy 2. Alexander disease 3. Hurler 4. Leigh 5. Tay Sachs

Answer 30

4. Leigh - retardation ataxia, opthalmoplegia, dystopia, cranial nerve palsies Which is a mitochondrial inherited disorder? 1. Adrenoleukodystrophy storage disease - Xlinked 2. Alexander disease - sporadic, rapid neurological deterioration, macrocephaly and seizures 3. Hurler MPS - most AR (except Hunter - X-linked) 4. Leigh - retardation ataxia, opthalmoplegia, dystopia, cranial nerve palsies * **LJS - 75% actually nuclear DNA mutations but 25% mitochondrial, so still best answer 5. Tay Sachs - lysosomal storage disease, twitching, visual and hearing loss, macrocephaly

Question 31

Which is Wilms tumour not associated with? ``` A. Denys-Drash B. WAGR C. Perlman D. Hutchinson E. Beckwith–Wiedemann ```

Answer 31

*LW: D. Hutchinson - neuroblastoma mets cause hutchinson syndrome Which is Wilms tumour not associated with? A. Denys-Drash: TRUE - Wilms tumour, male pseudohermaphroditism, progressive glomerulonephritis B. WAGR: TRUE C. Perlman: TRUE: (also called renal hamartomas, nephroblastomatosis and fetal gigantism) - wiki polyhydramnios, fetal overgrowth, neonatal macrosomia, high neonatal mortality, macrocephaly, dysmorphic facial features, visceromegaly, nephroblastomatosis and a predisposition for Wilms tumor at very early age. D. Hutchinson neuroblastoma mets cause hutchinson syndrome E. Beckwith–Wiedemann: TRUE

Question 32

Which is not a feature of Tetralogy of Fallot? 1. Overriding aorta 2. VSD 3. Pulmonary stenosis 4. Mitral stenosis 5. RV hypertrophy

Answer 32

4. Mitral stenosis

Question 33

Most common childhood orbital tumour: 1. Haemangioblastoma 2. Lymphoma 3. Retinoblastoma 4. Optic sheath meningioma

Answer 33

3. Retinoblastoma

Question 34

Juvenile Idiopathic Arthritis: 1. RF negative, Anti-CCP+ 2. RF negative...

Answer 34

2. RF negative...

Question 35

Pre-eclampsias vs acute fatty liver of pregnancy. Which makes pre-eclampsia more likely? 1. Coagulopathy 2. Neurological impairment 3. Proteinuria 4. Fetal distress 5. Abnormal LFT

Answer 35

3. Proteinuria Pre-eclampsias vs acute fatty liver of pregnancy. Which makes pre-eclampsia more likely? 1. Coagulopathy both 2. Neurological impairment both 3. Proteinuria not mentioned in RP 4. Fetal distress 5. Abnormal LFT both

Question 36

Which is not risk factor for pre-eclampsia? 1. Anti-phospholipid syndrome 2. Hypertension 3. Renal problems 4. Liver problems 5. Diabetes mellitus

Answer 36

4. Liver problems Risk factors of Pre-eclampsia- diabetes mellitus - chronic hypertension - family history - nulliparity - advanced maternal age (>40 years) - obesity - Twins- autoimmune conditions

Question 37

Choriocarcinoma 1. Unlikely to recur 2. Associated with ectopic

Answer 37

2. Associated with ectopic 25%. 25% normal pregnancies, 50% from complete moles Choriocarcinoma 1. Unlikely to recur very high cure rate 2. Associated with ectopic 25%. 25% normal pregnancies, 50% from complete moles

Question 38

Which doesn’t cause Meig’s syndrome? ``` A. Brenner B. Dysgerminoma C. Granulosa D. Fibroma E. Thecoma ```

Answer 38

B. Dysgerminoma

Question 39

Which are associated? 1. Adenocarcinoma of the cervix and HPV 2. SCC of the cervix and HIV

Answer 39

1. Adenocarcinoma of the cervix and HPV

Question 40

Which is not a risk factor for gynaecomastia? 1. Germ cell tumour 2. Chronic renal failure 3. Liver failure 4. Spironolactone 5. other un recalled option

Answer 40

All are according to RP Renal failure and germ cell tumours not mentioned in robbins **LJS - agree. All listed in stat dx as well. Quick google suggests GCT and renal CRF both also known to cause gynaecomastia, but not listed in big Rob (p1050) ?poor recall *AJL - GCT cause gynaecomastia from b-HCG effect. CRF causes it from uraemic hypogonadism.

Question 41

Breast lesion triple negative (HER, ER and PR) most likely lesion: 1. Invasive tubular 2. Lobular invasive C. Mucinous D. Medullary

Answer 41

D. Medullary Breast lesion triple negative (HER, ER and PR) most likely lesion: 1. Invasive tubular - ER/PR +ve, HER2-ve 2. Lobular invasive er +ve, HER-2 -ve C. Mucinous er +ve, HER-2 -ve D. Medullary

Question 42

Lyme disease is not associated with? 1. Locomotor ataxia 2. VII palsy 3. Radiculoneuritis 4. Aseptic meningitis 5. Encephalomyelitis

Answer 42

1. Locomotor ataxia infection by Borrelia Borgdorferi- bacteria- fever, systemic symptoms- skin- Neurological- cardiac- MSK: arthritis

Question 43

Which is most likely to affect the kidneys and lungs: 1. Diabetes 2. PAN 3. Tuberous sclerosis

Answer 43

3. Tuberous sclerosis

Question 44

What is caused by the Philadelphia chromosome thing? 1. CLL 2. ALL 3. CML

Answer 44

**LJS - disagree. CML caused by Philadelphia chrm (t(9;22) causing BCR-ABL fusion gene 1. CLL

Question 45

What is associated? A. Condylomata and SCC

Answer 45

A. Condylomata and SCC condylomata = genital warts