title: Rhabdoid Myomelanocytic Tumor (PEComa) of the Ovary: A Clinically Benign Case Followed for 7 Years.
abstract: A 3.0 × 2.5 cm rhabdoid myomelanocytic tumor was incidentally found in the left ovary of a 43-year-old black woman. The tumor cells were cytologically bland with minimal proliferation rate, multifocally weakly or moderately expressed TFE3, strongly expressed smooth muscle markers and SMARCB1/INI1, and focally expressed HMB45. They contained numerous paranuclear whorls of intermediate filaments that were verified by ultrastructure. No other lines of differentiation were detected within the tumor. Neither translocation nor increased number of copies of the TFE3 gene at Xp11.22 was detected by fluorescence in situ hybridization. The patient remains well, free of tumor, 7 years after surgery. A rhabdoid variant of myomelanocytic tumor is a rarity, with only a single case described previously.
authors.affiliation: Baylor Scott & White Health/Texas A&M Health Science Center College of Medicine, Temple, TX, USA.
authors.forename: Debby
authors.initials: D
authors.lastname: Rampisela
authors.affiliation: Charles University, Pilsen, Czech Republic.
authors.forename: Petr
authors.initials: P
authors.lastname: Grossmann
authors.affiliation: Baylor Scott & White Health/Texas A&M Health Science Center College of Medicine, Temple, TX, USA ldonner@sw.org.
authors.forename: Ludvik R
authors.initials: LR
authors.lastname: Donner
mesh_terms: D000328:Adult; D014408:Biomarkers, Tumor / Q000032:analysis; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007150:Immunohistochemistry; D010051:Ovarian Neoplasms / Q000175:diagnosis / Q000473:pathology*; D054973:Perivascular Epithelioid Cell Neoplasms / Q000175:diagnosis / Q000473:pathology*
chemical_list: D014408:Biomarkers, Tumor
keywords: TFE3-positive tumors; ovarian myomelanocytic tumors; perivascular epithelioid cell tumors; rhabdoid tumors