Renal tumors

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Benign Renal Tumors - The Good Guys

  • Angiomyolipoma (AML)

    • Composed of blood vessels, smooth muscle, & fat.
    • Fat-density on CT is diagnostic.
    • Risk of spontaneous hemorrhage, especially if > 4 cm.
  • Oncocytoma

    • Gross: Mahogany-brown tumor with a central stellate scar.
    • Histo: Large eosinophilic cells packed with mitochondria.
    • Often asymptomatic and found incidentally. Gross pathology of renal oncocytoma with central scar
  • Renal Papillary Adenoma

    • Most common benign renal tumor.
    • Defined as a papillary tumor ≤ 1.5 cm.

High-Yield: Multiple and bilateral angiomyolipomas are strongly associated with Tuberous Sclerosis (TSC).

Renal Cell Carcinoma - The Clear Culprit

  • Origin: Arises from the epithelium of the proximal convoluted tubule (PCT).
  • Risk Factors: Major risks include smoking and obesity. Also associated with hypertension, male gender, and acquired cystic kidney disease.
  • Pathology & Genetics:
    • Clear Cell (75%): Most common subtype. Associated with deletion of the VHL tumor suppressor gene on chromosome 3p. Grossly appears as a golden-yellow mass due to high lipid and glycogen content. Renal Cell Carcinoma Histology (H&E)
  • Clinical Features:
    • Classic Triad (uncommon, <10%): Flank pain, palpable mass, and hematuria.
    • Paraneoplastic Syndromes: 📌 PEAR: PTHrP (hypercalcemia), Erythropoietin (polycythemia), ACTH (Cushing's), Renin (hypertension).

Invasion Tendency: RCC is notorious for invading the renal vein and inferior vena cava (IVC). This can obstruct the left gonadal vein, leading to a varicocele that fails to decompress when supine.

  • Diagnosis: Abdominal CT with contrast is the gold standard for diagnosis and staging.

RCC Genetics & Staging - Code & Climb

  • Genetic Triggers

    • VHL gene (von Hippel-Lindau): Deletion on chromosome 3p. Most common cause of sporadic & hereditary clear cell RCC.
    • MET proto-oncogene: Activating mutation on chromosome 7. Associated with hereditary papillary RCC.
    • BHD gene: Mutations linked to chromophobe RCC & oncocytomas.
  • Robson Staging

    • Stage I: Tumor confined to kidney, <7 cm.
    • Stage II: Tumor confined to kidney, >7 cm.
    • Stage III: Extends into renal vein, IVC, or adrenal gland.
    • Stage IV: Invades beyond Gerota's fascia or distant metastases.

⭐ The classic triad of hematuria, flank pain, and a palpable abdominal mass is seen in only ~10% of RCC patients.

Renal Cell Carcinoma T Staging (AJCC 8th Edition)

Wilms & Urothelial Tumors - Kid & Pelvis Invaders

  • Wilms Tumor (Nephroblastoma)

    • Most common pediatric renal tumor, typically ages 2-5.
    • Presents as a large, palpable, unilateral flank mass; may cause hypertension.
    • Histology: Triphasic (blastemal, stromal, epithelial).
    • Associated with WAGR, Denys-Drash, Beckwith-Wiedemann syndromes.
    • 📌 WAGR: Wilms, Aniridia, Genitourinary anomalies, Retardation (mental).
  • Urothelial Carcinoma (Renal Pelvis)

    • Presents with painless hematuria.
    • Risk factors: Smoking, aniline dyes, cyclophosphamide.

⭐ Field defect: Urothelial carcinoma is often multifocal, with synchronous or metachronous tumors in the ureter or bladder.

Wilms tumor gross pathology: large unilateral kidney mass

  • Renal cell carcinoma (RCC) is the most common renal malignancy in adults; classic triad is hematuria, flank pain, and a palpable mass.
  • Clear cell is the most common RCC subtype, associated with VHL gene mutations on chromosome 3p.
  • Wilms tumor (nephroblastoma) is the most common renal malignancy in children, linked to WT1/WT2 mutations.
  • Angiomyolipoma is a benign tumor strongly associated with tuberous sclerosis.
  • RCC frequently causes paraneoplastic syndromes, like polycythemia (↑EPO) and hypercalcemia (↑PTHrP).

Practice Questions: Renal tumors

Test your understanding with these related questions

A 4-year-old girl is being followed by the pediatric oncology team after her pediatrician found a palpable abdominal mass towards the right flank 2 weeks ago. Abdominal ultrasonography detected a solid mass in the right kidney without infiltration of the renal vein and inferior vena cava. The contrast-enhanced computed tomography (CT) confirmed the presence of a solitary mass in the right kidney surrounded by a pseudocapsule consisting of a rim of normal tissue, displacing it medially, and distorting the collecting system. No nodal involvement was detected. In which of the following chromosomes would you expect a genetic abnormality?

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Flashcards: Renal tumors

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The most common variant of renal cell carcinoma exhibits _____ cytoplasm

TAP TO REVEAL ANSWER

The most common variant of renal cell carcinoma exhibits _____ cytoplasm

clear cell

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