Renal Neoplasia Flashcards
Renal Cell Carcinoma
Arises from the tubular epithelium, considered ADENOCARCINOMAS
Can affect ALL ages –> but RARE IN KIDS
Median age 60+
Comprises 2-3% of ALL CANCERS, 1-3% of visceral tumors, and 85% of ALL RENAL TUMORS
Makes up 2% of cancer deaths
M slight more than F
Risk factors for renal cell carcinoma?
SMOKING #1
Carcinogens, especially CADMIUM, ASBESTOS, PETROLEUM
Obesity
Associated with unopposed estrogen, HTN, abuse of analgesics, and those with pre-existing renal issues (horseshoe kidney, DM, CRF)
Subtypes of RCC?
CLEAR CELL RENAL CARCINOMA (most common)
PAPILLARY RENAL CELL CARCINOMA
CHROMOPHOBE RENAL CELL CARCINOMA (thought to arise from intercalated cells of the COLLECTING DUCT - the others are tubular epithelial cell origin)
CLEAR CELL RENAL CARCINOMA
Characterized by a CLEAR CYTOPLASM, variable nuclear features, and the LOSS OF CHROMOSOME 3p
Most common subtype of RCC
Nuclear features baseline of grading –> Grade 1 (round, regular) up to grade 4 (large, pleomorphic)
Arises from PROXIMAL TUBULAR EPITHELIUM
More common in the UPPER POLE of the kidney, often have CYSTIC COMPONENT – variable degrees of necrosis and hemorrhage
What syndrome is associated with Clear Cell Renal Carcinoma?
VON-HIPPEL LINDAU SYNDROME
Mutation in the tumor suppressor gene on the short arm of CHROMOSOME 3
This gene is responsible for SUPPRESSING PROLIFERATION AND TUMOR DEVELOPMENT
CCRCC development thought to be due to a somatic inactivation of the remaining wildtype VHL allele (two hit hypothesis - b/c of already lost chromosome 3)
VHL itself is associated with hemangioblastoma of the CNS
BILATERAL RENAL CARCINOMA!!!!!! Others tend to be unilateral
Also: - This is a hereditary tumor syndrome that predisposes to clear cell renal cell carcinoma (about half of patients with the syndrome), retinal angiomas, pheochromocytomas, hemangiomas of the central nervous system, pancreatic islet cell tumors and epididymal cystadenomas.
Histology of CCRCC
o Pattern may be trabecular, solid or in nests.
o Cells are round to POLYGONAL with clear to granular cytoplasm.
o Cytoplasm contains glycogen and lipid.
o Grade is dependent on the nuclear features.
PAPILLARY CARCINOMA
Less common, accounts for 15% of renal tumors
Often bilateral and frequently multifocal
Finger-Like projection growth pattern
ASSOCIATED WITH TRISOMY 7, 16 and 17!!!!
Less aggressive course
Associated with tyrosine kinase proteins
GROSS and HISTO –> multifocal hemorrhagic of cystic lesions, interstitial FOAM cells are common, PSAMMOMMA BODIES - round collections of calcium
CHROMOPHOBE CARCINOMA
Arises from the intercalated cells of the collecting duct (rather than tubular epithelium)
Accounts for 4-5% of renal cancers – characterized by cells with EOSINOPHILIC, GRANULAR CYTOPLASM often with a PERINUCLEAR HALO and Hypertrophic mitochondria
GOOD PROGNOSIS
Grossly looks like a large brownish tumor
Histo –> DISTINCT cell borders, halo around nucleus, pink granular material throughout
Presentation of RCC overall
May be ASYMPTOMATIC and only detected via CT inadvertently
Others present with HEMATURIA, UNWANTED WEIGHT LOSS, FEVER, FATIGUE, NIGHT SWEATS, ANEMIA
Liver dysfunction may be present but is due to CYTOKINE PRODUCTION of the tumor associated with increased liver enzymes (thus it is NOT due to metastasis)
If there is liver dysfunction, there is obviously a poorer outcome
Liver dysfunction associated with RCC = STAFFER SYNDROME
Treatment/Prognosis of RCC
Treatment is surgical
Very few are bilateral (2%)
If they are, it is suggestive of VON HIPPEL LINDAU
At the time of presentation, 25% of cases ALREADY HAVE METASTASES ELSEWHERE
Cancer may travel through lymphatics OR hematogeonously through the RENAL VEIN and settle in the regional lymph nodes, lung, liver, bone and brain
Another 20-30% get metastasis after surgery!
5 year survival: Stage I and II (60-100%, no metastases), III (20%), IV (5%)
UROTHELIAL CARCINOMA
Arises from the transitional epithelia
Often MULTIFOCAL, so if one tumor found there are probably others
On the cut surface of kidney, there may be a WELL-CIRCUMSCRIBED MASS sitting on the RENAL PELVIS causing obstruction
HEMATURIA is common
RECURRENCE IN BLADDER is common
POOOOOOR prognosis long term unless very very small
Nephrectomy may be necessary
BENIGN TUMORS
Renal Papillomas –> benign version of PRCC
Angiomyolipoma –> is a vascular proliferation of endothelial cells, smooth muscles and adipose tissue –> associated with TUBEROUS SCLEROSIS
ONCOCYTOMA –> occurs in the intercalated cells –> brown, homogenous and encapsulated grossly, eosinophils on microscopy
