Epithelioid angiomyolipoma (AML) is an uncommon renal mesenchymal tumor with malignant potential and is frequently associated with tuberous sclerosis.
Extrarenal AMLs are rare.
Synopsis
adrenal mass
epithelioid smooth muscle cells
- slightly pleomorphic nuclei
- sometimes with prominent nucleoli
- eosinophilic cytoplasm resembling oncocytic tumors.
Immunochemistry
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
References
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