Renal Tumors Flashcards
(57 cards)
1
Q
Average growth rate of benign renal cysts
A
2.8 mm/year
2
Q
Oncocytoma
- derived from what portion of nephron
- Similar to which variant of RCC
- Associated syndrome
- percentage of solid renal masses
A
- distal tubules
- chromophobe
- Birt Hogg Dube
- 3-7%
3
Q
Oncocytoma
- how often see RCC in ips or contra kidney?
- Genetic anomaly
A
- 30%
2. Loss of chromosome 1 and Y
4
Q
Birt Hogg Dube
- Associated with which renal tumors (2)
- Other systemic findings (2)
A
- Oncocytoma, chromophobe RCC
2. cutaneous fibrofolliculomas, pulmonary cysts
5
Q
Angiomyolipoma
- Derived from what cell types (3)
- Worst variant? findings
- Male or female?
- Associated with what syndrome? What percent with AML have it?
A
- perivascular epithelioid cells, smooth muscle, fat
- epithelioid, paucity of fat
- Female (80%)
- Tuberous Sclerosis, 20-25%
6
Q
AML
- CT finding
- MR finding
- Histological stain, what other cell type has
A
- negative Hounsfield Units (never calcification)
- decreased enhancement on chemical shift imaging
- HMB-45, melanoma
7
Q
3 Most common causes of retroperitoneal bleeding
A
- AAA
- RCC
- AML
8
Q
AML
- Size cutoff for treatment
- When to consider earlier
A
- > 4 cm
2. younger female desiring pregnancy
9
Q
Tuberous Sclerosis
- Findings (3)
- What % with TS have AML
- Growth rate of TS AMLs
A
- sebaceous adenoma, seizure, MR
- 80%
- 20%, vs 5% in normal AML
10
Q
Tuberous Sclerosis
- Genetic defects (2)
- Medical Rx for AML associated w TS
A
- 9q34 (TSC1); 16p13 (TSC2)
2. everolimus
11
Q
Mixed Epithelioid Stromal Tumor of Kidney
- Seen in which patients (2)
- Stain for receptors (2)
- Treatment
A
- Perimenopausal women, those with estrogen hormones
- estrogen, progesterone
- PN or RN
12
Q
Papillary Adenoma
- Size cutoff
- Genetic abnormalities (2), same as
- % with mets at 3 cm
A
- 5 mm, if larger it is pap RCC
- trisomy 7 and 17, papillary RCC
- 5%
13
Q
Reninoma
- arises from which cells
- which portion of nephron
- Electrolyte abnormalities (2) mediated by
- Typical patient
A
- juxtoglomerular cells
- distal convoluted tubule
- hypernatremia, hypokalemia. aldosterone
- young female with secondary HTN
14
Q
Leiomyoma
1. origin within kidney (3)
A
- renal capsule or peripelvic smooth muscle, renal vein
15
Q
RCC General
- Incidence in population
- % mortality
- Classic Triad
- Risk factors (4 non smoking)
- type of cancer cell
A
- 1:70
- 35-40%
- palpable mass, hematuria, flank pain
- HTN, ESRD, TS, obesity
- adenocarcinoma
16
Q
RCC general
- most common subtypes (3 in order)
- % with mets at time of presentation
- Histologic good prognostic findings (3)
A
- clear cell, papillary, chromophobe
- 25-33%
- increased CA-IX, low vimentin, low p53
17
Q
Familial RCC genetics
A
Autosomal Dominant
18
Q
RCC in ESRD
- type
- when/how to screen
A
- papillary
2. Renal US annually after 5 years on HD
19
Q
Renal mass, biopsy
- What size to consider
- % of mass behavior at this size
- Indications (4)
A
- <4 cm
- 20% benign, 60% indolent RCC, 20% aggressive RCC
- <4 cm, lymphoma, metastasis, abscess
20
Q
RCC Workup
- Enchancement needed for + CT
- When to order bone scan
A
- > 20 HU
2. elevated AFP, bone pain, pathologic fracture
21
Q
Multilocular Cystic RCC
- description
- Syndrome association
A
- cystic tumor lined with RCC clear cells, no solid
2. VHL
22
Q
Clear Cell RCC
- Associated gene, Xsome, location
- Location in the nephron
- gene type
- gene pathway
- % of all RCC
A
- VHL; 3; 3p25-26
- Proximal tubules
- tumor suppressor
- VHL suppresses HIF-1 normally. HIF-1, VEGF, leads to neovascularization and tumor growth
- 75%
23
Q
Papillary RCC - Type 1
- Associated gene, Xome, location
- gene type
- location in nephron
- histology
A
- cMet, 7q31
- proto-oncogene
- proximal tubules
- basophilic, blue, low grade
24
Q
Papillary RCC - Type 2
- Associated gene, Xsome, location
- gene type
- location in nephron
- histology
A
- fumarate hydratase, 1q42-44
- tumor suppressor
- proximal tubules
- eosinophilic, pink, aggressive
25
Chromophobe RCC
1. Associated gene, Xsome
2. gene type
3. location in nephron
4. Similar to what benign lesion
5. histologic findings (3)
1. folliculin, 17p11.2
2. tumor suppressor
3. distal tubule
4. oncocytoma
5. hale's colloidal, diffuse CK7 Staining, few mitochondria
26
Renal Medullary Carcinoma
1. What diagnosis commonly affected
2. Type of patient, presentation
3. Prognosis
1. sickle cell (blacks)
2. young, locally advanced disease at presentation
3. poor, aggressive, 12-15 mo survival
27
Renal metastases
| -most common mets to the kidney (3 in order)
1. lung
2. bone
3. liver
28
Von Hippel-Lindau Disease
1. gene location/type
2. disease complex (5)
3. Treatment size
1. 3p25-26, tumor suppressor
2. RCC (clear cell 50%), pheochromocytoma, retinal angioma, CNS hemangioblastoma, epididymal cystadenoma
3. Partial Nx @ 3 cm
29
Hereditary Papillary RCC
1. Type of tumor seen
2. Genetic abnormality (2)
1. papillary type 1 RCC
| 2. Trisomy 7 and 17
30
Hereditary Leiomyomatosis and RCC
1. Type of RCC
2. Associated finding
3. treatment
1. Papillary type 2
2. cutaneous and uterine leiomyomata
3. treat quickly, rapid mets
31
Birt Hogg Dube Syndrome
1. Gene mutation, location
2. findings (4)
1. folliculin, 17p11
| 2. chromophobe RCC, cutaneous fibromas, pulm cysts, pneumothorax
32
Clear Cell RCC
1. Gene, Xsome
2. Stain (2)
1. VHL, 3p24-25
| 2. CD10, vimentin
33
Papillary type 1 RCC
1. gene, xsome
2. stain (2)
1. c-met, 7q31 (17)
| 2. AMACR, basophillic/blue
34
Papillary type 2 RCC
1. gene, xsome
2. stain (2)
1. Fumarate hydratase, 1q42
| 2. AMACR, eosinophilic/pink
35
Chromophobe RCC
1. Xsome
2. stain (2)
1. 17p11
| 2. Hales, diffuse CK7
36
Bellini Duct Upper tract RCC
| 1. Stain (2)
Ulex europaeus, vimentin
37
AML
1. xsomes (2)
2. stain
1. TS1/2, 9, 16
| 2. HMB-45
38
Paraneoplastic syndromes in RCC
1. % that get
2. examples (5)
3. most common
1. 1/3
2. hypercalcemia, stauffer syndrome, polycythemia, anemia, hypertension
3. Hypercalcemia
39
Hypercalcemia of RCC
1. cause/mechanism
2. treatment (3)
3. confused often with
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
Stauffer syndrome
1. definition
2. resolves with ?
1. hepatic dysfunciton in RCC pt without mets
| 2. nephrectomy
41
Anemia or polycythemia more common in RCC?
anemia
42
RCC T staging
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
RENAL Nephrometry Score
1. categories
2. Scoring
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
When to consider ablation of a tumor (1)
only if < 4 cm
45
Indications for partial nephrectomy (5)
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
Principles of partial Nx (4)
1. early vascular control
2. diuresis with mannitol
3. Ischemia time < 30 min
4. adequate reconstruction
47
Purpose of mannitol in partial nx (2)
prior to hilar clamping
1. reduce oxidative damage/free radicals
2. induce diuresis
48
Stage 4 RCC surgery
1. if tumor resectable and solitary met
2. if multiple mets
3. Which met location do not do nephrectomy
1. nephrectomy and metastatectomy
2. cytoreductive nephrectomy & medical therapy
3. Brain met
49
RCC surgery
1. Lymph node dissection needed?
2. When to remove adrenal (1 and 1 maybe)
1. Only if gross adenopathy
| 2. renal tumor with direct extension (t4), question if upper pole tumor or > 7 cm
50
RCC follow up after Nephrectomy
1. when is recurrence most common
2. MC sites of recurrence
3. testing for surveillance (2)
1. first 1-2 years
2. lung, bone, liver
3. Abd CT and Chest imaging
51
RCC follow up (CTs, CXR)
1. T1
2. T2-T4
3. Ablation
4. Surveillance
5. When to get first CT scan
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
Hyperfiltration injury
1. Need to remove how much kidney?
2. Leads to what pathologically
3. First indicator
4. Clinical manifestation
1. 75%
2. Focal segmental sclerosis
3. proteinuria
4. HTN
53
Main reason for P Nx over R Nx is that radical has higher rates of (2)
1. Cardiovascular disease
| 2. chronic renal insufficiency
54
Metastatic RCC Tyrosine Kinase inhibitors
1. List (3)
2. Class side effects (3)
3. Delivery
1. Sunitinib
2. Sorafinib
3. Pazopanib
a. hand-foot-mouth
b. hepatotoxicity
c fatigue/thyroid
---Oral
55
MTOR Inhibitor for metastatic RCC
1. Drug name
2. Who is it indicated for?
3. Delivery
4. Side effects (2)
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
Metastatic RCC cytokine therapy
1. Durable complete remission rate?
2. Requirements to take
1. 5%
| 2. Excellent performance status
57
Metastatic RCC VEGF ligand inhibitor
1. Drug name/combo
2. complications (3)
3. Drug delivery route
4. half life, Drug delivery issue
5. Contraindication
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
