Rhabdoid Myomelanocytic Tumor (PEComa) of the Ovary: A Clinically Benign Case Followed for 7 Years. (August 2016)

• Record Type: Journal Article
• Authors: Rampisela, Debby; Grossmann, Petr; Donner, Ludvik R.
• Is Part Of: International journal of surgical pathology. Volume 24:Number 5(2016)
• Page Start: 431
• Page End: 435
• Publication Date: 2016-08
• Subjects: perivascular epithelioid cell tumors -- rhabdoid tumors -- ovarian myomelanocytic tumors -- TFE3-positive tumors; Pathology, Surgical -- Periodicals; 617.07
• DOI: 10.1177/1066896916635815
• Languages: English
• ISSNs: 1066-8969

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.