kidney tumors Flashcards
renal papillary adenoma
Benign-Well circumscribed nodules within the cortex, 7 – 22 % at autopsy, small (5 mm diameter). Surgically removed since they are considered “early cancers.”
Renal Fibroma or Hamartoma (Renomedullary Interstitial Cell Tumor
Benign- Fibrous, less than 1 cm, within pyramids, no malignant tendency.
Angiomyolipoma
Benign- Vessels, smooth muscle and fat. 25 -50% in patients with tuberous sclerosis.
Oncocytoma
Benign- Eosinophilic epithelial cells, numerous mitochondria, 5 – 15% of all renal neoplasms. Some cases are most common within family groups.
Metanephric adenoma
Benign - Very rare, only one case in a child known to produce metastases
Clear cell carcinoma incidence
Most common type, accounting for 70% to 80% of renal cell cancers. Male to female ratio 3:1.
Clear cell carcinoma clinical
o Hematuria. o Renal mass may be incidental finding
o Arises in the renal cortex, has a propensity to invade the renal vein and can extend into the inferior vena cava up to the heart. o Regional lymph nodes may be enlarged. Hematogenous spread to lungs may occur, too. Metastatic disease often as multiple nodules in the lungs. o Hematuria. o Renal mass may be incidental finding
o Arises in the renal cortex, has a propensity to invade the renal vein and can extend into the inferior vena cava up to the heart. o Regional lymph nodes may be enlarged. Hematogenous spread to lungs may occur, too. Metastatic disease often as multiple nodules in the lungs.
Clear cell carcinoma imaging
o Ball-like mass of renal cortex; tumor enhances less than normal parenchyma. o Engorged, tumor-filled renal vein with extension to inferior vena cava. o Look for metastatic disease.
Clear cell carcinoma pathology
Gross: Most often as single tumor (multifocal and bilateral in Von Hippel-Lindau disease), somewhat spherical, yellowish gray mass, variegated appearance, focal hemorrhage, 20% are cystic. Histology: Clear, granular and/or spindle cells. Nuclear grade is assigned according to Furhman’s criteria (from 1-4).
Furhman criteria/grading and survival
1 - Small, round, uniform nuclei (10 microns), inconspicuous nucleoli, look like lymphocytes (very rare)
2 - Slightly irregular nuclei, see nucleoli at 40x only
3 - See nucleoli at 10x, nuclei very irregular,
4 - Mitoses; bizarre, multilobated, pleomorphic cells plus grade 3 features, macronucleoli [15% of tumors]. Survival is better in grades 1-2, than 3-4. 1 - Small, round, uniform nuclei (10 microns), inconspicuous nucleoli, look like lymphocytes (very rare)
2 - Slightly irregular nuclei, see nucleoli at 40x only
3 - See nucleoli at 10x, nuclei very irregular,
4 - Mitoses; bizarre, multilobated, pleomorphic cells plus grade 3 features, macronucleoli [15% of tumors]. Survival is better in grades 1-2, than 3-4.
Clear cell carcinoma genetics
VHL gene is implicated in disease. Most cases are sporadic, but 4% are familial and associated with Von Hippel-Lindau disease. VHL gene encodes protein that is part of a ubiquitin ligase complex involved in targeting other proteins for degradation, particularly HIF-1 (increases trxn of pro-angiogenic proteins like VEGF, TGF-B)
Clear cell carcinoma prognosis and treatment
The average 5-year survival rate is about 45% and up to 70% in the absence of distant metastases. With renal vein invasion or extension into the perinephric fat, the figure is reduced to approximately 15% to 20%. Treatment is partial or full nephrectomy.
Papillary carcinoma (Chromophilic) incidence, pathology
- Incidence: Represent 10% to 15% of renal cancers.
- Pathology: o Gross: Unlike clear cell RCCs, papillary carcinomas are frequently multifocal. o Histology: Papillary growth pattern. • Incidence: Represent 10% to 15% of renal cancers.
- Pathology: o Gross: Unlike clear cell RCCs, papillary carcinomas are frequently multifocal. o Histology: Papillary growth pattern.
Papillary carcinoma (Chromophilic) genetics and prognosis
familial or sporadic. trisomies 7, 16, and 17 and loss of Y in male patients in the sporadic form, and trisomy 7 in the familial form. Prognosis: better than clear cell carcinoma
Chromophobe Renal Carcinoma incidence, pathology, genetics, prognosis
• Incidence: 5% of renal cell cancers • Pathology: Cells with prominent cell membranes and pale eosinophilic cytoplasm, usually with a halo around the nucleus. Histologic distinction from oncocytoma can be difficult. • Genetics: multiple chromosome losses and extreme hypodiploidy. They are, like the benign oncocytoma, thought to grow from intercalated cells of collecting ducts.• Prognosis: Excellent compared with that of the clear cell and papillary cancers; similar to oncocytomas.
