Wieder's 1 - Renal Tumors

jahen86's version from 2015-05-02 01:49

Work up

Question Answer
Bosniak: simple v complex?Bosniak 1 = simple; 2-4 = complex
Bosniak 1 f/uno further w/u
Bosniak 2 f/uno further w/u
Bosniak 2F f/usurveillance periodic imaging
Bosniak 3 f/uevaluate for malignancy
Bosniak 3 f/uevaluate for malignancy
3 differentials for solid (non-cystic) renal mass?malignancy (RCC), pseudotumor, angiomyolipoma (AML)
How to differentiate between malignant solid renal mass and renal pseudotumor?DMSA renal scan or CT/MRI optimized to renal imaging
How does pseudotumor vs RCC appear on DMSA scan?pseudotumor is nl, RCC has decreased uptake
PE shows pink papules on face?adenoma sebaceum, a/w tuberous sclerosis
PE shows hypopigmented macules on trunk/buttocks?ash-leaf spots, a/w tuberous sclerosis
PE shows leathery textured plaques on lower back?Shagreen patches, a/w tuberous sclerosis
PE shows small white papules on face/upper trunk?fibrofolliculomas, a/w Birt-Hogg-Dube
renal mass w/u labs?chem10, LFTs, LDH, CBC, U/A
renal mass further imaging?CXR/Chest CT, bone XRs if pain, brain MRI if neuro deficits, mammogram in women at risk
reasons to biopsy renal masssuspicion of inflammatory/AML/lymphoma/met; surgical candidate choosing surveillance (might change decision); nonsurgical candidate choosing systemic therapy


Question Answer
most common adult malignant renal massRCC
most common pediatric malignant renal massWilms' tumor
differential for benign renal masssimple cyst (most common), papillary adenoma (most common solid), pseudotumor, AML, oncocytoma, JGA tumor, nephroma
3 most common renal metslymphoma/leukemia, lung, breast; less commonly stomach, colon, cervical, melanoma
defn Dromedary hump, most common laterality?mid lateral renal bulge thought to be from downward pressure from spleen or liver during development, most commonly left

Hereditary Syndromes

Question Answer
Birt Hogg Dube chromosome17
3 classic BHD findingsskin fibrofolliculomas, pulm cysts/spont ptx, renal tumors
classic triad of tuberous sclerosismental retardation, seizures, adenoma sebaceum (all from hamartomas)
chromosomes for tuberous sclerosis?9 or 16
urologic association for tuberous sclerosis?renal cysts, AML, RCC
VHL gene3p
VHL non-urologic manifestationshemangioblastomas (cerebe3llar, spinal, retinal)
VHL urologic manifestationsrenal cysts, clear cell RCC, pheos, epididymal cysts
protein target for pVHLtargets HIF1α at nl oxygen lvls for ubiquitination -> proteasomal degradation; at low O2 lvls & in mutated pVHL, HIF1α is unapposed and binds to HIF1β and promotes transcription of proangiomatotic proteins: VEGF, PDGF, Epo

Benign Renal Tumors

Question Answer
compare papillary adenoma v papillary RCCmicroscopically indistinguishable; are <5mm
what part of nephron does papillary adenoma & RCC arise?proximal tubule
what part of nephron does oncocytoma arise?collecting duct
compare oncocytoma v chromophobe RCCindistinguishable on imaging, may coexist together, tx as RCC
AML components?blood vessels, muscle, and fat
AML v RCC on CT scan?AML no calcifications, HU= -20 to -80 d/t fat content, enhancing, homogenous, not cystic
stain to diagnose AMLHMB-45
size cutoff for obs v tx of AML>=4cm consider treatment d/t risk of hemorrhage, otherwise surveillance


Question Answer
modifiable risk factors for RCCobesity, tobacco
nephron origination of clear cell RCCproximal tubule
nephron origination of chromophobe RCCcollecting duct
stain to differentiate chromophobe RCC from oncocytoma?Hale's colloidal iron stains RCC cytoplasm
most common RCC classification arising in pts with chronic renal failurepapillary, secondary to acquired cystic kidney dz
most common RCC classification arising in pts w/ sickle cell traitrenal medullary carcinoma
what is Stauffer's syndrome?paraneoplastic hepatitis associated w/ RCC (not from mets)
Fuhrman grading used for what types of RCC?clear cell & papillary
3 components of Fuhrman gradingnuclear size, nuclear contour, nucleoli prominence @ x100
T1 RCC?<7cm, limited to kidney
T1a RCC?<4cm, limited to kidney
T1b RCC?4-7cm, limited to kidney
T2 RCC?>7cm, limited to kidney
T2a RCC?7-10cm, limited to kidney
T2b RCC?>10cm, limited to kidney
T3 RCC?extension into major veins or perinephric tissue/fat but not adrenal or beyond Gerota's
T3a RCC?no IVC extension
T3b RCC?extends into subdiaphragmatic IVC
T3c RCC?extends into supradiaphragmatic IVC or invades IVC wall
T4tumor invasion beyond Gerota's or ipsilateral adrenal
size indication for nephron sparing surgery (i.e. PN)T1 (<7cm)
only immunotherapy that achieves durable remission in met ccRCC?high dose IL-2
mTOR inhibitors used for immunotherapy to improve overall and progression free survival?temsirolimus (only immunotherapy that works against non-ccRCC) and everolimus
mechanism + examples of tyrosine kinase inhibitors (TKIs) used to tx RCCinhibits various receptors responsible for angiogenesis/cell proliferation (VEGFR, PDGFR); ex: sunitinib, sorafenib, pazopanib
mechanism of bevacizumab w/ INF-alfa-2aneutralizes VEGF-A, improves outcomes when combined w/ INF therapy

Follow Up

Question Answer
defn hyperfiltration renal injuryprolonged glomerular hyperfiltration after removal of >75% of functional renal tissue, results in renal injury = FSGS
how to monitor for hyperfiltration injury?24hr urine for creat, protein, & volume
abd imaging f/u for T1N0 pt s/p RNx?CT/MRI/US at 3-12mo post op, no further imaging after if no major risk factors (familial, high grade, +margins)
abd imaging f/u for T1N0 pt s/p PNx?CT/MRI @ 3-12mo post op, then qYear for 3yrs.
how often CXR for T1N0 pt s/p nx?qYear x 3yrs
imaging f/u for high risk pt (T2-4, N1) s/p Nx?chest & abd CT/MRI 3-6mo post op; then chest (CXR/CT/MRI) + abd (CT/MRI/US) imaging q6mo x3yrs, then qY x2yrs (total 5yrs imaging f/u)
imaging f/u for surveillance?abd CT/MRI 6mo after dx, then qY + CXR

Recurrence & Survival

Question Answer
ECOG 0nl activity
ECOG 1symptomatic but ambulatory
ECOG 2bedridden <50% of the time
ECOG 3bedridden >50% of the time
ECOG 4completely bedridden
approx 5 yr dz specific survival T1-T2N0 s/p RN or PN>90%
approx 5 yr overall survival for T3 tumor s/p RN~50%
approx 5 yr overall survival for T4 or N1 tumors s/p RN~25%
approx 5 yr overall survival for initial M1 tumor s/p RN & resection of met<20%