title: Malignant PEComa of the adrenal gland.
abstract: Perivascular epithelioid cell neoplasms, also known as PEComas, are unique mesenchymal tumors exhibiting perivascular epithelioid cell differentiation, characterized by a mixed myogenic and melanocytic phenotype. PEComas arising in visceral organs outside of the kidney, liver, and lung are rare, and often pose problems in diagnosis. Examples of this neoplasm originating in the adrenal gland are limited. The present report details the clinical and pathologic features of an unusual case of a pure epithelioid PEComa (epithelioid angiomyolipoma) of the adrenal gland exhibiting clinically malignant behavior in the form of pulmonary metastases, a feature not previously described in tumors of this site. The diagnosis was supported by immunohistochemical studies demonstrating expression of myoid and melanocytic antigens. The present case serves to emphasize the potential of PEComa for clinically aggressive behavior and the importance of distinguishing this tumor from other epithelioid neoplasms that are more commonly encountered in the adrenal gland.
authors.affiliation: Department of Pathology, City of Hope National Medical Center, 1500 East Duarte Road, Duarte, CA 91010, United States. pheji@hotmail.com
authors.forename: Sean K
authors.initials: SK
authors.lastname: Lau
mesh_terms: D000310:Adrenal Gland Neoplasms / Q000235:genetics / Q000276:immunology / Q000473:pathology*; D000951:Antigens, Neoplasm / Q000032:analysis; D014408:Biomarkers, Tumor / Q000032:analysis; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008175:Lung Neoplasms / Q000556:secondary; D008875:Middle Aged; D054973:Perivascular Epithelioid Cell Neoplasms / Q000235:genetics / Q000276:immunology / Q000473:pathology* / Q000556:secondary; D020133:Reverse Transcriptase Polymerase Chain Reaction
chemical_list: D000951:Antigens, Neoplasm; D014408:Biomarkers, Tumor