Renal Neoplasia PDF

# RENAL NEOPLASIA ### I. ANGIOMYOLIPOMA * A. Hamartoma comprised of blood vessels, smooth muscle, and adipose tissue * B. Increased frequency in tuberous sclerosis ### II. RENAL CELL CARCINOMA * A. Malignant epithelial tumor arising from kidney tubules ### B. Presents with classic triad of hema...

# RENAL NEOPLASIA ### I. ANGIOMYOLIPOMA * A. Hamartoma comprised of blood vessels, smooth muscle, and adipose tissue * B. Increased frequency in tuberous sclerosis ### II. RENAL CELL CARCINOMA * A. Malignant epithelial tumor arising from kidney tubules ### B. Presents with classic triad of hematuria, palpable mass, and flank pain * 1. All three symptoms rarely occur together; hematuria is the most common symptom * 2. Fever, weight loss, or paraneoplastic syndrome (e.g., EPO, renin, PTHrP, or ACTH) may also be present * 3. Rarely may present with left-sided varicocele * i. Involvement of the left renal vein by carcinoma blocks drainage of the left spermatic vein leading to varicocele * ii. Right spermatic vein drains directly into the IVC; hence, right-sided varicocele is not seen. ### C. Gross exam reveals a yellow mass; microscopically, the most common variant exhibits clear cytoplasm ### D. Pathogenesis involves loss of VHL tumor suppressor gene, which leads to: * Increased IGF-1 (promotes growth) * Increased HIF transcription factor (increases VEGF and PDGF) ### E. Tumors may be hereditary or sporadic. * 1. Sporadic tumors classically arise in adult males (average age is 60 years) as a single tumor in the upper pole of the kidney; major risk factor for sporadic tumors is cigarette smoke * 2. Hereditary tumors arise in younger adults and are often bilateral. * i. Von Hippel-Lindau disease is an autosomal dominant disorder associated with inactivation of the VHL gene leading to increased risk for hemangioblastoma of the cerebellum and renal cell carcinoma. ### F. Staging * 1. **T** - based on size and involvement of the renal vein (occurs commonly and increases risk of hematogenous spread to the lungs and bone) * 2. **N** - spread to retroperitoneal lymph nodes # III. WILMS TUMOR ### A. Malignant kidney tumor comprised of blastema, primitive glomeruli and tubules, and stromal cells * 1. Most common malignant renal tumor in children; average age is 3 years * 2. Presents as a large, unilateral flank mass with hematuria and hypertension (due to renin secretion) ### C. Most cases (90%) are sporadic; syndromic tumors may be seen with: * 1. **WAGR syndrome** - Wilms tumor, Aniridia, Genital abnormalities, and mental and motor Retardation; associated with deletion of WT1 tumor suppressor gene (located at 11p13) * 2. **Denys-Drash syndrome** - Wilms tumor, progressive renal (glomerular) disease, and male pseudohermaphroditism; associated with mutations of WT1 * 3. **Beckwith-Wiedemann syndrome** - Wilms tumor, neonatal hypoglycemia, muscular hemihypertrophy, and organomegaly (including tongue); associated with mutations in WT2 gene cluster (imprinted genes at 11p15.5), particularly IGF-2