Renal Tumors Flashcards
Average growth rate of benign renal cysts
2.8 mm/year
Oncocytoma
- derived from what portion of nephron
- Similar to which variant of RCC
- Associated syndrome
- percentage of solid renal masses
- distal tubules
- chromophobe
- Birt Hogg Dube
- 3-7%
Oncocytoma
- how often see RCC in ips or contra kidney?
- Genetic anomaly
- 30%
2. Loss of chromosome 1 and Y
Birt Hogg Dube
- Associated with which renal tumors (2)
- Other systemic findings (2)
- Oncocytoma, chromophobe RCC
2. cutaneous fibrofolliculomas, pulmonary cysts
Angiomyolipoma
- Derived from what cell types (3)
- Worst variant? findings
- Male or female?
- Associated with what syndrome? What percent with AML have it?
- perivascular epithelioid cells, smooth muscle, fat
- epithelioid, paucity of fat
- Female (80%)
- Tuberous Sclerosis, 20-25%
AML
- CT finding
- MR finding
- Histological stain, what other cell type has
- negative Hounsfield Units (never calcification)
- decreased enhancement on chemical shift imaging
- HMB-45, melanoma
3 Most common causes of retroperitoneal bleeding
- AAA
- RCC
- AML
AML
- Size cutoff for treatment
- When to consider earlier
- > 4 cm
2. younger female desiring pregnancy
Tuberous Sclerosis
- Findings (3)
- What % with TS have AML
- Growth rate of TS AMLs
- sebaceous adenoma, seizure, MR
- 80%
- 20%, vs 5% in normal AML
Tuberous Sclerosis
- Genetic defects (2)
- Medical Rx for AML associated w TS
- 9q34 (TSC1); 16p13 (TSC2)
2. everolimus
Mixed Epithelioid Stromal Tumor of Kidney
- Seen in which patients (2)
- Stain for receptors (2)
- Treatment
- Perimenopausal women, those with estrogen hormones
- estrogen, progesterone
- PN or RN
Papillary Adenoma
- Size cutoff
- Genetic abnormalities (2), same as
- % with mets at 3 cm
- 5 mm, if larger it is pap RCC
- trisomy 7 and 17, papillary RCC
- 5%
Reninoma
- arises from which cells
- which portion of nephron
- Electrolyte abnormalities (2) mediated by
- Typical patient
- juxtoglomerular cells
- distal convoluted tubule
- hypernatremia, hypokalemia. aldosterone
- young female with secondary HTN
Leiomyoma
1. origin within kidney (3)
- renal capsule or peripelvic smooth muscle, renal vein
RCC General
- Incidence in population
- % mortality
- Classic Triad
- Risk factors (4 non smoking)
- type of cancer cell
- 1:70
- 35-40%
- palpable mass, hematuria, flank pain
- HTN, ESRD, TS, obesity
- adenocarcinoma
RCC general
- most common subtypes (3 in order)
- % with mets at time of presentation
- Histologic good prognostic findings (3)
- clear cell, papillary, chromophobe
- 25-33%
- increased CA-IX, low vimentin, low p53
Familial RCC genetics
Autosomal Dominant
RCC in ESRD
- type
- when/how to screen
- papillary
2. Renal US annually after 5 years on HD
Renal mass, biopsy
- What size to consider
- % of mass behavior at this size
- Indications (4)
- <4 cm
- 20% benign, 60% indolent RCC, 20% aggressive RCC
- <4 cm, lymphoma, metastasis, abscess
RCC Workup
- Enchancement needed for + CT
- When to order bone scan
- > 20 HU
2. elevated AFP, bone pain, pathologic fracture
Multilocular Cystic RCC
- description
- Syndrome association
- cystic tumor lined with RCC clear cells, no solid
2. VHL
Clear Cell RCC
- Associated gene, Xsome, location
- Location in the nephron
- gene type
- gene pathway
- % of all RCC
- VHL; 3; 3p25-26
- Proximal tubules
- tumor suppressor
- VHL suppresses HIF-1 normally. HIF-1, VEGF, leads to neovascularization and tumor growth
- 75%
Papillary RCC - Type 1
- Associated gene, Xome, location
- gene type
- location in nephron
- histology
- cMet, 7q31
- proto-oncogene
- proximal tubules
- basophilic, blue, low grade
Papillary RCC - Type 2
- Associated gene, Xsome, location
- gene type
- location in nephron
- histology
- fumarate hydratase, 1q42-44
- tumor suppressor
- proximal tubules
- eosinophilic, pink, aggressive
Chromophobe RCC
- Associated gene, Xsome
- gene type
- location in nephron
- Similar to what benign lesion
- histologic findings (3)
- folliculin, 17p11.2
- tumor suppressor
- distal tubule
- oncocytoma
- hale’s colloidal, diffuse CK7 Staining, few mitochondria
Renal Medullary Carcinoma
- What diagnosis commonly affected
- Type of patient, presentation
- Prognosis
- sickle cell (blacks)
- young, locally advanced disease at presentation
- poor, aggressive, 12-15 mo survival
Renal metastases
-most common mets to the kidney (3 in order)
- lung
- bone
- liver
Von Hippel-Lindau Disease
- gene location/type
- disease complex (5)
- Treatment size
- 3p25-26, tumor suppressor
- RCC (clear cell 50%), pheochromocytoma, retinal angioma, CNS hemangioblastoma, epididymal cystadenoma
- Partial Nx @ 3 cm
Hereditary Papillary RCC
- Type of tumor seen
- Genetic abnormality (2)
- papillary type 1 RCC
2. Trisomy 7 and 17
Hereditary Leiomyomatosis and RCC
- Type of RCC
- Associated finding
- treatment
- Papillary type 2
- cutaneous and uterine leiomyomata
- treat quickly, rapid mets
Birt Hogg Dube Syndrome
- Gene mutation, location
- findings (4)
- folliculin, 17p11
2. chromophobe RCC, cutaneous fibromas, pulm cysts, pneumothorax
Clear Cell RCC
- Gene, Xsome
- Stain (2)
- VHL, 3p24-25
2. CD10, vimentin
Papillary type 1 RCC
- gene, xsome
- stain (2)
- c-met, 7q31 (17)
2. AMACR, basophillic/blue
Papillary type 2 RCC
- gene, xsome
- stain (2)
- Fumarate hydratase, 1q42
2. AMACR, eosinophilic/pink
Chromophobe RCC
- Xsome
- stain (2)
- 17p11
2. Hales, diffuse CK7
Bellini Duct Upper tract RCC
1. Stain (2)
Ulex europaeus, vimentin
AML
- xsomes (2)
- stain
- TS1/2, 9, 16
2. HMB-45
Paraneoplastic syndromes in RCC
- % that get
- examples (5)
- most common
- 1/3
- hypercalcemia, stauffer syndrome, polycythemia, anemia, hypertension
- Hypercalcemia
Hypercalcemia of RCC
- cause/mechanism
- treatment (3)
- confused often with
- PTH-related peptide and PTH elevated in the absence of bone mets
- Loop diuretic, bisphosphonates, nephrectomy
- HyperCa d/t bone mets
Stauffer syndrome
- definition
- resolves with ?
- hepatic dysfunciton in RCC pt without mets
2. nephrectomy
Anemia or polycythemia more common in RCC?
anemia
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
RENAL Nephrometry Score
- categories
- Scoring
- Radius, Exophytic, Nearness to collecting system, A/P, Location relative to polar lines
- 4-6 is low
7-9 moderate
10-12 high
When to consider ablation of a tumor (1)
only if < 4 cm
Indications for partial nephrectomy (5)
- bilateral tumors
- Solitary kidney
- Tumor < 4 cm (small)
- Decreased renal fx
- comorbidities leading to decreased renal fx (HTN, DM)
Principles of partial Nx (4)
- early vascular control
- diuresis with mannitol
- Ischemia time < 30 min
- adequate reconstruction
Purpose of mannitol in partial nx (2)
prior to hilar clamping
- reduce oxidative damage/free radicals
- induce diuresis
Stage 4 RCC surgery
- if tumor resectable and solitary met
- if multiple mets
- Which met location do not do nephrectomy
- nephrectomy and metastatectomy
- cytoreductive nephrectomy & medical therapy
- Brain met
RCC surgery
- Lymph node dissection needed?
- When to remove adrenal (1 and 1 maybe)
- Only if gross adenopathy
2. renal tumor with direct extension (t4), question if upper pole tumor or > 7 cm
RCC follow up after Nephrectomy
- when is recurrence most common
- MC sites of recurrence
- testing for surveillance (2)
- first 1-2 years
- lung, bone, liver
- Abd CT and Chest imaging
RCC follow up (CTs, CXR)
- T1
- T2-T4
- Ablation
- Surveillance
- When to get first CT scan
- annually x3 years, annually x3 years
- semiannually x3 years, then annually x 2
- annually x5 years
- annually
- 3-6 months out from surgery
Hyperfiltration injury
- Need to remove how much kidney?
- Leads to what pathologically
- First indicator
- Clinical manifestation
- 75%
- Focal segmental sclerosis
- proteinuria
- HTN
Main reason for P Nx over R Nx is that radical has higher rates of (2)
- Cardiovascular disease
2. chronic renal insufficiency
Metastatic RCC Tyrosine Kinase inhibitors
- List (3)
- Class side effects (3)
- Delivery
- Sunitinib
- Sorafinib
- Pazopanib
a. hand-foot-mouth
b. hepatotoxicity
c fatigue/thyroid
—Oral
MTOR Inhibitor for metastatic RCC
- Drug name
- Who is it indicated for?
- Delivery
- Side effects (2)
- Temsirolimus
- Poor risk patients with advanced RCC
- LDH, anemia, hyperCa, Performance < 70, multiple mets, long dx to rx gap - IV
- Mucositis, rash
Metastatic RCC cytokine therapy
- Durable complete remission rate?
- Requirements to take
- 5%
2. Excellent performance status
Metastatic RCC VEGF ligand inhibitor
- Drug name/combo
- complications (3)
- Drug delivery route
- half life, Drug delivery issue
- Contraindication
- Bevacizumab +IFN alpha
- wound healing, hemorrhage, proteinuria/HTN
- IV
- t1/2 = 20 days. Need to stop 80-100 days prior to and after surgery (4-5 half lives)
- Contra in fourniers