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Blue Book Review > Renal Tumors > Flashcards

Renal Tumors Flashcards

1
Q

Average growth rate of benign renal cysts

A

2.8 mm/year

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2
Q

Oncocytoma

  1. derived from what portion of nephron
  2. Similar to which variant of RCC
  3. Associated syndrome
  4. percentage of solid renal masses
A
  1. distal tubules
  2. chromophobe
  3. Birt Hogg Dube
  4. 3-7%
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3
Q

Oncocytoma

  1. how often see RCC in ips or contra kidney?
  2. Genetic anomaly
A
  1. 30%

2. Loss of chromosome 1 and Y

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4
Q

Birt Hogg Dube

  1. Associated with which renal tumors (2)
  2. Other systemic findings (2)
A
  1. Oncocytoma, chromophobe RCC

2. cutaneous fibrofolliculomas, pulmonary cysts

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5
Q

Angiomyolipoma

  1. Derived from what cell types (3)
  2. Worst variant? findings
  3. Male or female?
  4. Associated with what syndrome? What percent with AML have it?
A
  1. perivascular epithelioid cells, smooth muscle, fat
  2. epithelioid, paucity of fat
  3. Female (80%)
  4. Tuberous Sclerosis, 20-25%
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6
Q

AML

  1. CT finding
  2. MR finding
  3. Histological stain, what other cell type has
A
  1. negative Hounsfield Units (never calcification)
  2. decreased enhancement on chemical shift imaging
  3. HMB-45, melanoma
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7
Q

3 Most common causes of retroperitoneal bleeding

A
  1. AAA
  2. RCC
  3. AML
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8
Q

AML

  1. Size cutoff for treatment
  2. When to consider earlier
A
  1. > 4 cm

2. younger female desiring pregnancy

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9
Q

Tuberous Sclerosis

  1. Findings (3)
  2. What % with TS have AML
  3. Growth rate of TS AMLs
A
  1. sebaceous adenoma, seizure, MR
  2. 80%
  3. 20%, vs 5% in normal AML
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10
Q

Tuberous Sclerosis

  1. Genetic defects (2)
  2. Medical Rx for AML associated w TS
A
  1. 9q34 (TSC1); 16p13 (TSC2)

2. everolimus

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11
Q

Mixed Epithelioid Stromal Tumor of Kidney

  1. Seen in which patients (2)
  2. Stain for receptors (2)
  3. Treatment
A
  1. Perimenopausal women, those with estrogen hormones
  2. estrogen, progesterone
  3. PN or RN
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12
Q

Papillary Adenoma

  1. Size cutoff
  2. Genetic abnormalities (2), same as
  3. % with mets at 3 cm
A
  1. 5 mm, if larger it is pap RCC
  2. trisomy 7 and 17, papillary RCC
  3. 5%
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13
Q

Reninoma

  1. arises from which cells
  2. which portion of nephron
  3. Electrolyte abnormalities (2) mediated by
  4. Typical patient
A
  1. juxtoglomerular cells
  2. distal convoluted tubule
  3. hypernatremia, hypokalemia. aldosterone
  4. young female with secondary HTN
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14
Q

Leiomyoma

1. origin within kidney (3)

A
  1. renal capsule or peripelvic smooth muscle, renal vein
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15
Q

RCC General

  1. Incidence in population
  2. % mortality
  3. Classic Triad
  4. Risk factors (4 non smoking)
  5. type of cancer cell
A
  1. 1:70
  2. 35-40%
  3. palpable mass, hematuria, flank pain
  4. HTN, ESRD, TS, obesity
  5. adenocarcinoma
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16
Q

RCC general

  1. most common subtypes (3 in order)
  2. % with mets at time of presentation
  3. Histologic good prognostic findings (3)
A
  1. clear cell, papillary, chromophobe
  2. 25-33%
  3. increased CA-IX, low vimentin, low p53
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17
Q

Familial RCC genetics

A

Autosomal Dominant

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18
Q

RCC in ESRD

  1. type
  2. when/how to screen
A
  1. papillary

2. Renal US annually after 5 years on HD

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19
Q

Renal mass, biopsy

  1. What size to consider
  2. % of mass behavior at this size
  3. Indications (4)
A
  1. <4 cm
  2. 20% benign, 60% indolent RCC, 20% aggressive RCC
  3. <4 cm, lymphoma, metastasis, abscess
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20
Q

RCC Workup

  1. Enchancement needed for + CT
  2. When to order bone scan
A
  1. > 20 HU

2. elevated AFP, bone pain, pathologic fracture

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21
Q

Multilocular Cystic RCC

  1. description
  2. Syndrome association
A
  1. cystic tumor lined with RCC clear cells, no solid

2. VHL

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22
Q

Clear Cell RCC

  1. Associated gene, Xsome, location
  2. Location in the nephron
  3. gene type
  4. gene pathway
  5. % of all RCC
A
  1. VHL; 3; 3p25-26
  2. Proximal tubules
  3. tumor suppressor
  4. VHL suppresses HIF-1 normally. HIF-1, VEGF, leads to neovascularization and tumor growth
  5. 75%
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23
Q

Papillary RCC - Type 1

  1. Associated gene, Xome, location
  2. gene type
  3. location in nephron
  4. histology
A
  1. cMet, 7q31
  2. proto-oncogene
  3. proximal tubules
  4. basophilic, blue, low grade
24
Q

Papillary RCC - Type 2

  1. Associated gene, Xsome, location
  2. gene type
  3. location in nephron
  4. histology
A
  1. fumarate hydratase, 1q42-44
  2. tumor suppressor
  3. proximal tubules
  4. eosinophilic, pink, aggressive
25
Q

Chromophobe RCC

  1. Associated gene, Xsome
  2. gene type
  3. location in nephron
  4. Similar to what benign lesion
  5. histologic findings (3)
A
  1. folliculin, 17p11.2
  2. tumor suppressor
  3. distal tubule
  4. oncocytoma
  5. hale’s colloidal, diffuse CK7 Staining, few mitochondria
26
Q

Renal Medullary Carcinoma

  1. What diagnosis commonly affected
  2. Type of patient, presentation
  3. Prognosis
A
  1. sickle cell (blacks)
  2. young, locally advanced disease at presentation
  3. poor, aggressive, 12-15 mo survival
27
Q

Renal metastases

-most common mets to the kidney (3 in order)

A
  1. lung
  2. bone
  3. liver
28
Q

Von Hippel-Lindau Disease

  1. gene location/type
  2. disease complex (5)
  3. Treatment size
A
  1. 3p25-26, tumor suppressor
  2. RCC (clear cell 50%), pheochromocytoma, retinal angioma, CNS hemangioblastoma, epididymal cystadenoma
  3. Partial Nx @ 3 cm
29
Q

Hereditary Papillary RCC

  1. Type of tumor seen
  2. Genetic abnormality (2)
A
  1. papillary type 1 RCC

2. Trisomy 7 and 17

30
Q

Hereditary Leiomyomatosis and RCC

  1. Type of RCC
  2. Associated finding
  3. treatment
A
  1. Papillary type 2
  2. cutaneous and uterine leiomyomata
  3. treat quickly, rapid mets
31
Q

Birt Hogg Dube Syndrome

  1. Gene mutation, location
  2. findings (4)
A
  1. folliculin, 17p11

2. chromophobe RCC, cutaneous fibromas, pulm cysts, pneumothorax

32
Q

Clear Cell RCC

  1. Gene, Xsome
  2. Stain (2)
A
  1. VHL, 3p24-25

2. CD10, vimentin

33
Q

Papillary type 1 RCC

  1. gene, xsome
  2. stain (2)
A
  1. c-met, 7q31 (17)

2. AMACR, basophillic/blue

34
Q

Papillary type 2 RCC

  1. gene, xsome
  2. stain (2)
A
  1. Fumarate hydratase, 1q42

2. AMACR, eosinophilic/pink

35
Q

Chromophobe RCC

  1. Xsome
  2. stain (2)
A
  1. 17p11

2. Hales, diffuse CK7

36
Q

Bellini Duct Upper tract RCC

1. Stain (2)

A

Ulex europaeus, vimentin

37
Q

AML

  1. xsomes (2)
  2. stain
A
  1. TS1/2, 9, 16

2. HMB-45

38
Q

Paraneoplastic syndromes in RCC

  1. % that get
  2. examples (5)
  3. most common
A
  1. 1/3
  2. hypercalcemia, stauffer syndrome, polycythemia, anemia, hypertension
  3. Hypercalcemia
39
Q

Hypercalcemia of RCC

  1. cause/mechanism
  2. treatment (3)
  3. confused often with
A
  1. PTH-related peptide and PTH elevated in the absence of bone mets
  2. Loop diuretic, bisphosphonates, nephrectomy
  3. HyperCa d/t bone mets
40
Q

Stauffer syndrome

  1. definition
  2. resolves with ?
A
  1. hepatic dysfunciton in RCC pt without mets

2. nephrectomy

41
Q

Anemia or polycythemia more common in RCC?

A

anemia

42
Q

RCC T staging

A

1a: <4 cm
1b: 4-7 cm
2a: 7-10 cm
2b: >10 cm
3a: renal vein, renal sinus fat
3b: IVC below diaphragm
3c: IVC above diaphragm or into wall
4: extension outside kidney

43
Q

RENAL Nephrometry Score

  1. categories
  2. Scoring
A
  1. Radius, Exophytic, Nearness to collecting system, A/P, Location relative to polar lines
  2. 4-6 is low
    7-9 moderate
    10-12 high
44
Q

When to consider ablation of a tumor (1)

A

only if < 4 cm

45
Q

Indications for partial nephrectomy (5)

A
  1. bilateral tumors
  2. Solitary kidney
  3. Tumor < 4 cm (small)
  4. Decreased renal fx
  5. comorbidities leading to decreased renal fx (HTN, DM)
46
Q

Principles of partial Nx (4)

A
  1. early vascular control
  2. diuresis with mannitol
  3. Ischemia time < 30 min
  4. adequate reconstruction
47
Q

Purpose of mannitol in partial nx (2)

A

prior to hilar clamping

  1. reduce oxidative damage/free radicals
  2. induce diuresis
48
Q

Stage 4 RCC surgery

  1. if tumor resectable and solitary met
  2. if multiple mets
  3. Which met location do not do nephrectomy
A
  1. nephrectomy and metastatectomy
  2. cytoreductive nephrectomy & medical therapy
  3. Brain met
49
Q

RCC surgery

  1. Lymph node dissection needed?
  2. When to remove adrenal (1 and 1 maybe)
A
  1. Only if gross adenopathy

2. renal tumor with direct extension (t4), question if upper pole tumor or > 7 cm

50
Q

RCC follow up after Nephrectomy

  1. when is recurrence most common
  2. MC sites of recurrence
  3. testing for surveillance (2)
A
  1. first 1-2 years
  2. lung, bone, liver
  3. Abd CT and Chest imaging
51
Q

RCC follow up (CTs, CXR)

  1. T1
  2. T2-T4
  3. Ablation
  4. Surveillance
  5. When to get first CT scan
A
  1. annually x3 years, annually x3 years
  2. semiannually x3 years, then annually x 2
  3. annually x5 years
  4. annually
  5. 3-6 months out from surgery
52
Q

Hyperfiltration injury

  1. Need to remove how much kidney?
  2. Leads to what pathologically
  3. First indicator
  4. Clinical manifestation
A
  1. 75%
  2. Focal segmental sclerosis
  3. proteinuria
  4. HTN
53
Q

Main reason for P Nx over R Nx is that radical has higher rates of (2)

A
  1. Cardiovascular disease

2. chronic renal insufficiency

54
Q

Metastatic RCC Tyrosine Kinase inhibitors

  1. List (3)
  2. Class side effects (3)
  3. Delivery
A
  1. Sunitinib
  2. Sorafinib
  3. Pazopanib
    a. hand-foot-mouth
    b. hepatotoxicity
    c fatigue/thyroid
    —Oral
55
Q

MTOR Inhibitor for metastatic RCC

  1. Drug name
  2. Who is it indicated for?
  3. Delivery
  4. Side effects (2)
A
  1. Temsirolimus
  2. Poor risk patients with advanced RCC
    - LDH, anemia, hyperCa, Performance < 70, multiple mets, long dx to rx gap
  3. IV
  4. Mucositis, rash
56
Q

Metastatic RCC cytokine therapy

  1. Durable complete remission rate?
  2. Requirements to take
A
  1. 5%

2. Excellent performance status

57
Q

Metastatic RCC VEGF ligand inhibitor

  1. Drug name/combo
  2. complications (3)
  3. Drug delivery route
  4. half life, Drug delivery issue
  5. Contraindication
A
  1. Bevacizumab +IFN alpha
  2. wound healing, hemorrhage, proteinuria/HTN
  3. IV
  4. t1/2 = 20 days. Need to stop 80-100 days prior to and after surgery (4-5 half lives)
  5. Contra in fourniers

Blue Book Review (3 decks)

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