Sclerosing variant of perivascular epithelioid cell tumor in the female genital organs

Yuichi Yamada, Hidetaka Yamamoto, Yoshihiro Ohishi, Kenichi Nishiyama, Masao Fukuhara, Toshiaki Saitou, Masazumi Tsuneyoshi, Yoshinao Oda

Research output: Contribution to journalArticlepeer-review

24 Citations (Scopus)


Perivascular epithelioid cell tumor (PEComas), other than angiomyolipoma, clear cell 'sugar' tumor of the lung, and lymphangioleiomyomatosis, is an uncommon mesenchymal neoplasm that arises in the soft tissue and visceral organs. We report herein two cases of sclerosing PEComa; a distinctive variant of PEComa, which is characterized by extensive stromal hyalinization, occurring in the uterus and broad ligament. The patients were 34- and 51-year-old females with no family history of tuberous sclerosis complex. Macroscopically, the tumors had white to gray cut surfaces and were microscopically composed of predominantly spindle- to polygon-shaped cells with clear to slightly eosinophilic cytoplasm and pleomorphic nuclei focally arranged in a perivascular pattern, accompanied by marked stromal hyalinization. These tumor cells were immunohistochemically positive for HMB45 and α-smooth muscle actin. Although this variant of PEComa is very rare, this entity should be considered as a potential primary neoplasm of the female genital organs.

Original languageEnglish
Pages (from-to)768-772
Number of pages5
JournalPathology International
Issue number12
Publication statusPublished - Dec 2011

All Science Journal Classification (ASJC) codes

  • Pathology and Forensic Medicine


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