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
RFs for Wilm's tumor?
- aniridia (iris formed partially or not all all)
- undescended testicles
Clinical features of Wilm's tumor?
- palpable mass: 90% of cases - may be large, extending into pelvis
- abdominal pain in 30%
- hematuria: 12-25%
- HTN in 25%
- 5-10% bilateral
Imaging for Wilm's tumor?
- abdominal US and contrast enhanced CT (useful to diff Wilms from other causes of abdominal masses)
How is WIlm's tumor definitively dx?
- histologic confirmation, either at time of surgical excision or by bx
How is staging based in wilms tumor?
- upon anatomic extent of tumor without consideration for genetic, histologic, or biological markers
Staging of Wilm's tumor?
- stage 1: cancer is found in one kidney only and can be completely removed by surgery
- stage 2: cancer has spread beyond the kidney to surrounding area but can be completely removed by surgery
- stage 3: cancer hasn't spread beyond the abdomen but can't be completely removed by surgery
- stage 4: cancer has spread to distant parts of the body, most commonly the lungs, liver, bone and/or brain
- stage 5: cancer is found in both kidneys at diagnosis (bilateral tumors)
Macroscopic appearance of Wilm's tumor?
- usually large single well circumscribed
- tan/white, not as much hemorrhage as RCC
- occasional cyst formation
Tx for Wilm's Tumor?
- tx decisions and eval of long term outcome are based upon Wilm's tumor stage
- surgery for stages 1-4 (radical, take out adrenal gland and surrounding fat)
- stave 5: try to take out as much of kidney as you can, need to go on transplant list
- chemo and radiation indicated
What are the benign renal tumors of the kidney? Other malignant tumors?
- papillary adenoma
transitional cell carcinoma
Characteristics of a renal papillary adenoma?
- arise from cortical renal tubules
- histologically similar to low grade papillary cancers: usually contain trisomy 7, and 17
- no unequivocal benign marker, tx as early cancer (including tumors less than 3 cm)
Where are renal fibromas/hamartomas found?
- small benign fibrous nodules in renal pyramids
Characteristics of an angiomyolipoma?
- benign tumor composed of blood vessels, smooth muscle, and adipose tissue
- present in up to half of pts with tuberous sclerosis
- easy to spot radiographically because of fat content
What is a TCC?
Transitional Cell Carcinoma
- in the collecting system
- a malignant tumor arising from transitional epithelial cells that line the urinary tract from renal calyces to ureteral orifices
- peak incidence: 60-70 y/o
- men 2x more than women
- incidence slightly higher in African Americans
Presentation and workup of TCC?
- presentation: hematuria, and renal colic (uncomfortable pain)
- workup: urine cytology, cystoscopy, CT IVP
Tx of TCC?
- radical nephrourectomy
What is a renal sarcoma? Presentation?
- renal sarcoma: rare malignant tumor derived from mesenchymal cells, these grow fast and big!
- 1-2% of all renal malignant tumors, peaks in 50s
- presentation: abdominal pain, palpable mass, gross hematuria
Dx and tx of renal sarcoma?
- dx: CT (you already know what you are looking for - abdominal mass, so go straight to CT, US won't show much) and MRI
- tx: surgical excision
Most common histologic pattern of RCC?
- Clear cell
Why has the incidence of RCC been on the rise in the US?
- because of increased use of imaging procedures for other reasons
RFs assoc with a sig increased incidence of RCC include?
Sxs of RCC?
- mass, pain due to tumor
- hematuria due to invasion of urinary tract
- metastases, paraneoplastic syndromes
Tx of RCC?
- For pts with a resectable stage 1, II, and III - surgery primary
- radical nephrectomy most widely used when there is evidence of invasion into adrenal, renal vein, or perinephric fat
- partial nephrectomy is alt for smaller tumors and is particularly valuable in pts with bilateral or multiple lesions and those with impaired renal fxn