Malignant Tumors of the Kidney Flashcards
What is the origin of 90% of Wilms tumors?
Sporadic and unilateral
What is WAGR Syndrome?
Wilms tumor, aniridia, genitourinary anomalies, mental retardation
What is Denys-Drash Syndrome?
Wilms Tumor, intersexual disorders, glomerular mesangial sclerosis
What is Beckwith-Wiedemann Syndrome?
Wilms tumor, overgrowth ranging from gigantism to hemihypertrophy, visceromegaly, and macroglossia
What leads to WAGR Syndrome?
Deletion of the short arm of chromosome 11 which contains the aniridia gene and Wilms tumor gene 1 (WTN1)
What genetic process leads to WAGR syndrome? What is this similar to?
Germline mutation and then loss of heterozygosity; Similar to pathogenesis of hereditary Rb
What are found in the kidneys of all children with syndromic Wilms tumors?
Nephrogenic rests - small foci of persistent primitive blastemal cells
A bulging, pale, tan, cut surface, enclosed by a thin rim of renal cortex and capusle is shown. What type of tumor is it?
Wilms tumor
Wilms tumor is shown. What three components are prominent?
- Metanephreic blastema
- Immature stroma
- immature epithelial elements
What is the most common primary cancer of the kidney?
Renal cell carcinoma
What are the three syndromes that predispose to RCC?
- von Hippel-Lindau Syndrome
- Autosomal Dominant RCC
- Hereditary Papillary RCC
What type of mutation is seen in virtually all sporadic clear cell RCCs?
Loss of one VHL allele
Islands of neoplastic cells with abundant clear cytoplasm is shown. What is the Dx?
Clear cell renal cell carcinoma
What accounts for the clear cytoplasm in clear cell RCC?
Abundant cytoplasmic lipids and glycogen
Papillary RCC is shown. What is the difference betwixt type 1 and type 2?
- Type 1: Small basophilic cells
- Type 2: Large acidophilic cells
A mixture of acidophilic granular cells and pale transparent cells with prominent cell borders is shown. What is the Dx?
Chromophobe RCC
