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Flashcards in kidney tumors Deck (17):
1

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.”

2

Renal Fibroma or Hamartoma (Renomedullary Interstitial Cell Tumor

Benign- Fibrous, less than 1 cm, within pyramids, no malignant tendency.

3

Angiomyolipoma

Benign- Vessels, smooth muscle and fat. 25 -50% in patients with tuberous sclerosis.

4

Oncocytoma

Benign- Eosinophilic epithelial cells, numerous mitochondria, 5 – 15% of all renal neoplasms. Some cases are most common within family groups.

5

Metanephric adenoma

Benign - Very rare, only one case in a child known to produce metastases

6

Clear cell carcinoma incidence

Most common type, accounting for 70% to 80% of renal cell cancers. Male to female ratio 3:1.

7

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.

8

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.

9

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).

10

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.

11

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)

12

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.

13

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.

14

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

15

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.

16

Chromophobe RCC vs oncocytoma staining with Hales colloidal iron stain

Chromophobe RCC turns blue, oncocytoma is pale/clear

17

Collecting Duct (Bellini duct) Carcinoma incidence, pathology, prognosis

• Incidence: 1% or less of renal epithelial neoplasms. They arise from collecting duct cells in the medulla.
• Pathology: Nests of malignant cells enmeshed within a prominent fibrotic stroma, typically in a medullary location.
• Prognosis: Associated with aggressive behavior and poor prognosis. For the majority of patients surgical treatment will not result in a cure. Previously recommended chemotherapy and/or immunotherapy appear to have a limited role in treatment of this disease, and early detection may be the best method for prolonging patient survival.