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Renal angiomyolipoma


Definition

  • A mesenchymal neoplasm composed of variable proportions of abnormal blood vessels, cells with smooth muscle features and adipose tissue; believed to be derived from perivascular epithelioid cells (PEC); hence, also named PECOMA

Clinical features

Epidemiology

  • Angiomyolipoma (AML) accounts for 1%–2% of all renal tumors in surgical specimens.

  • Can be sporadic or associated with tuberous sclerosis complex (TSC); sporadic AMLs 4- to 5-fold are more common than TSC-associated AMLs.

  • Patients with TSC develop AML earlier (mean age at diagnosis is 25–35 years with TS vs. 40–45 years without TSC).

  • More common in female patients (female-to-male ratio, 4:1 in patients without TSC)

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