Flashcards in Tumors of the Kidney & Urinary Tract Deck (14)
Common benign tumors of the kidney
- renal papillary adenoma
- renal fibroma or hamartoma
- metanephric adenoma
Characteristics of renal paillary adenoma
- Well circumscribed nodules within the cortex, small
- 7 – 22 % at autopsy
- Surgically removed since they are considered “early cancers.”
Characteristics of renal fibroma or hamartoma
- Renomedullary Interstitial Cell Tumor
- Fibrous, less than 1 cm, within pyramids
- no malignant tendency
Characteristics of angiomyolipoma
- Vessels, smooth muscle and fat
- 25 -50% in patients with tuberous sclerosis.
Characteristics of oncocytoma
- Eosinophilic epithelial cells, numerous mitochondria
- 5 – 15% of all renal neoplasms.
- Some cases are most common within family groups.
Clear cell carcinoma: incidence, clinical, imaging
- Incidence: most common; male > female
- Arises @ renal cortex ==> invade the renal vein + can extend into the inferior vena cava
- metastases: Regional lymph nodes + often as multiple nodules in the lungs.
- Ball-like mass of renal cortex;
- Engorged, tumor-filled renal vein with extension to inferior vena cava.
- Look for metastatic disease.
Clear cell carcinoma: pathology
- Gross: single tumor (multifocal and bilateral in Von Hippel-Lindau disease),
- spherical, yellowish gray mass, patchy
- cell types: clear, granular, and spindle.
- most = clear + granula
- Spindle cell types or sarcomas tend to grow and spread more quickly than the other kinds of RCCs.
Clear cell carcinoma: genetics
- VHL gene: both familial and sporadic tumors
- lost or mutated
- loss of sequences @ short arm chromosome 3
clear cell carcinoma: staging/grade & prognosis
- Grading = Fuhrman’s criteria (1-4)
- 1-2 = better survival vs. 3-4
- 45% 5-yr survival
- higher w/out metastasis and lower w/renal vein invations
Papillary carcinoma (Chromophillic): incidence, pathology, genetics, prognosis
- Incidence: Represent 10% to 15% of renal cancers.
- Gross: Unlike clear cell RCCs, papillary carcinomas are frequently multifocal.
- Histology: Papillary growth pattern.
- Genetics: Occurs in both familial and sporadic forms. Not associated with 3p deletions.
- sporadic: trisomies 7, 16, and 17 and loss of Y
- familial: trisomy 7 (MET locus = protooncogene)
- Prognosis: Better than clear cell RCC
Chromophobe renal carcinoma: incidence, pathology, genetics, prognosis
- Incidence: Represents 5% of renal cell cancers
- Pathology: prominent cell membranes and pale eosinophilic cytoplasm + halo around the nucleus
- Genetics: multiple chromosome losses and extreme hypodiploidy.
- Prognosis: Excellent compared with that of the clear cell and papillary cancers; similar to oncocytomas.
Collecting duct carcinoma: incidence, pathology, genetics, prognosis
- Incidence: Represents approximately 1% or less
- Pathology: Nests of malignant cells enmeshed within a prominent fibrotic stroma, typically in a medullary location.
- Genetics: chromosomal losses/deletions; no distinct pattern
- Prognosis: Associated with aggressive behavior and poor prognosis.
Types of familial RCC
- Von Hippel-Lindau (VHL) syndrome
- gene implicated in both familial and sporadic
- Hereditary papillary carcinoma
- autosomal-dominant form
- bilateral tumors w/papillary historlogy
- MET protooncogene
TNM staging system
- T=tumor size, N=lymph nodes, M=metastasis
- Stage 1: small (<7cm), limited to kidney
- Stage 2: >7cm, limited to kidney
- Stage 3:
- any size + spread to lymph node + no metastasis
- spread into fatty/tissue or large veins + no lymph node spread + no metastasis
- Stage 4: distant metastasis