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adrenal epithelioid angiomyolipoma

Wednesday 14 July 2010

Epithelioid angiomyolipoma (AML) is an uncommon renal mesenchymal tumor with malignant potential and is frequently associated with tuberous sclerosis.

Extrarenal AMLs are rare.


- adrenal mass
- epithelioid smooth muscle cells

  • slightly pleomorphic nuclei
  • sometimes with prominent nucleoli
  • eosinophilic cytoplasm resembling oncocytic tumors.


Epithelioid cells were positive for melanoma (HMB45 and positive MelanA) and smooth muscle markers (alpha-smooth muscle-specific actin), but not for epithelial markers (cytokeratin, EMA).

Differential diagnosis

- renal cell carcinoma
- adrenal gland carcinoma
- metastatic carcinoma


- Monotypic epithelioid angiomyolipoma of the adrenal gland: an unusual site for a rare extrarenal tumor. D’Antonio A, Caleo A, Caleo O, De Dominicis G, Boscaino A. Ann Diagn Pathol. 2009 Oct;13(5):347-50. PMID: 19751913