Flashcards in Renal Neoplastic Disease Deck (52)
Where does a RCC originate at? How common are RCC's?
- renal cortex
- 80-85% of primary renal neoplasms
- 2% of overall cancer incidence and mortality
- highest incidence in europe and U.S.
Epidemiology of RCC?
- male predominance: RCC is approx 50% more common in men than women
- highest incidence is b/t 60-80, median age of dx: 64, median age of death: 70
RFs of RCC?
- smoking (1/3 of cases)
- acquired cystic disease of kidney
- occupational exposure, NSAID use, genetic, Hep C infection
Subtypes of RCC?
- clear cell (75-85%)
- papillary (chromophilic) - (10-15%)
- chromophobe (5-10%)
- oncocytic (3-7%)
- collecting duct (bellini's duct - very rare)
What cancer types effect cortical collecting duct intercalated cells?
- chromophobe, oncocytoma RCC
What cancer types effect medullary collecting duct?
- collecting duct carcinoma
What cancer types effect PCT?
- clear cell, and papillary RCC
clear cell carcinoma characteristics?
- arise from PCT
- typically have deletion of x-some 3p
- macroscopically they may be solid or less commonly cystic
- tends to have vascular invasion (spread to renal sinus - into renal vein then will met to liver, lungs)
Characteristics of papillary carcinoma?
- can be familial or sporadic
- though to arise from PCT
- trisomy 7 common (also 16, 17, and loss of Y)
- divided into type 1 and 2
- recent study shoows better prognosis than clear cell carcinoma
Characteristics of chromophobe RCC?
- histologically: composed of sheets of cells that are darker than clear cell carcinoma
- approx 5-10% of RCCs
- arise from intercalated cells of collecting ducts
- lower risk of disease progression and death compared to clear cell
Characteristics of collecting duct carcinoma (Bellini)?
- approx 1% of RCCs
- aggressive clinical course
- affects younger adults and african american pts more frequently
Characteristics of oncocytomas?
- 3-7% of RCCs
- originate from intercalated cells of collecting ducts
- consists of oncocytes, which are large well differentiated neoplastic cells with intensely eosinophilc granular cytoplasm
- mutations in c-met oncogene assoc with papillary RCC
- tends to be unilateral and single, but multiple and bilateral oncocytomas have been described
- these are benign, but hard to distinguish from RCC, rarely mets
Micro appearance of clear cell carcinoma?
- clear cytoplasm due to lipid and glycogen, cells may be bland or poorly differentiated, cells form solid nests, tubules: fine vasculature
Micro appearance of papillary carcinoma?
- papillae are composed of delicate vascular cores with overlying layers of tumor cells
- papillary tips may undergo necrosis and calcification (psammoma bodies)
Micro appearance of chromophobe RCC?
- sheets of pink (eosinophilic) cells with perinuclear halos
- look like oncocytoma (can't tell diff until kidney is out)
Clinical features of RCC? Presentation?
- presents late in course and most commonly dx incidentally
- classic presentation:
flank pain (constant dull ache - back or abd)
palpable renal mass
- nonspecific features: fatigue, fever, wt loss, night sweats
- sudden onset of scrotal varicocele (tumor blocking blood flow to IVC)
- paraneoplastic syndromes: due to ectopic production of various hormones
other features- anemia, hepatic dysfxn, and anorexia
Dx eval of RCC?
- CT scan (for true eval of mass, with IV contrast)
- MRI (preggos, allergic to dye, serial CT scans)
Diff dx of renal cysts?
(can have complex multiple cysts)
How common are simple cysts?
- approx 50% of persons older than 50 have 1 or more renal cysts
- almost 1/3 of persons over 50 - have at least one renal cyst that is ID on CT scan
3 major criteria for simple cyst on a U/S?
- mass is round and sharply demarcated with smooth walls
- no echoes (anechoic) within the mass
- strong posterior wall echo indicating good transmission through the cyst
What is the primary reason to investigae a renal mass?
- to exclude a malignant neoplasm
Most common sites of mets from RCC?
- lung (get CXR, UA, Cr)
- lymph nodes: intra abdominal
Imaging for assessment of mets?
- bone scan
- CT of chest
- PET (in oncologists care)
Tx of RCC?
- TOC: SURGICAL REMOVAL
- radical nephrectomy (gold std) , involvement of lymphatics in almost 25% of pts, 5% of pts have IVC involvement
- role of radiotherapy remain controversial in tx of RCC, relatively radioresistant tumor, chemo, immunotherpay limited role
What is included in radical nephrectomy (gold std of tx)
- early vascular ligation, extrafascial dissection of kidney, en bloc rescetion of adrenal gland, and extensive lymphadenectomy from the crus of diaphragm to aortic bifurcation, resection of tumor extension into IVC
Nephron- sparing surgery (partial nephrectomy) survival rates?
- equivalent survival of pts undergoing NSS versus radical nephrectomy for unilateral tumore less than 4 cm in size
- recurrence free survival after 5-10 years comparable with radical nephrectomy
- BUT risk of recurrences increases with large tumors, bilateral tumors, multifocality, sxs, and certain histologies such as papillary RCC
Staging and prognosis of RCC?
- stage 1: tumor less than 7 cm in greatest dimension and limited to the kidney, 5 yr survival rate is 95%
- stage 2: tumor larger than 7 cm in greatest dimension and limited to kidney, 5 yr survival is 88%
- stage 3: tumor in major veins or adrenal gland, tumor within Gerota's fascia or 1 regional lymph node involved, 5 year survival: 59%
- stage 4: tumor beyond Gerota's fascia or more than 1 regional lymph node involved, 5 yr survival: 20% (distal mets = poor outcome)
What is a Wilm's tumor (nephroblastoma)?
- ped tumor: most common renal malignancy in children (8:1,000,000, 500 cases/year, 7% of ped neoplasms)
- 4th most common childhood cancer
- mean age: 3.5 years (rarely seen after 15)
- more common in African Americans
Pathogenesis of Wilm's tumor?
- caused by abnorm renal development, resulting in proliferation of metanephric blastema w/o normal tubular and glomerular differentiation
- assoc with congenital syndromes (rare): WAGR syndrome, Denys-Drash syndrome, Beckwith-Wiedemann syndromes