multifocal pecoma (pecomatosis) of the female genital tract and pelvis a case report and review of the literature多病灶的pecoma(pecomatosis)女性生殖道和骨盆病例报告和文献之回顾.pdfVIP

Yang et al. Diagnostic Pathology 2012, 7:23 /content/7/1/23 CASE REPORT Open Access Multifocal PEComa (PEComatosis) of the female genital tract and pelvis: a case report and review of the literature * Wang Yang , Gao Li and Zheng Wei-qiang Abstract Perivascular epithelioid cells (PECs) are constantly present in a group of tumors called PEComas, including angiomyolipoma (AML), clear-cell “sugar” tumor (CCST) of the lung and extrapulmonary sites, lymphangioleiomyomatosis, clear-cell myomelanocytic tumor of the falciform ligament/ligamentum teres and rare clear-cell tumors of other anatomic sites. PECs have distinctive morphologic, immunohistochemical, ultrastructural and genetic characteristics, including an epithelioid appearance with a clear to granular cytoplasm, a round to oval, centrally located nucleus, and an inconspicuous nucleolus. PECs also express melanocytic and myogenic markers like HMB45 and smooth muscle actin. PEComa is rare in human, and multifocal PEComas (PEComatosis) is extremely rare. Up to now there have been only less than 5 cases described in the literature. Here we report a 46- year old Chinese woman who had PEComatosis arising from the genital tract and pelvis. To our knowledge, this is the first case of PEComatosis ever reported in China. Virtual Slides: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/ vs/1293097548652023. Keywords: Perivascular epithelioid cell tumor (PEComa), PEComatosis, Immunohistochemistry Perivascular epithelioid cell tumor (PEComa) refers to a Here we describe a 46-year-old Chinese woman who family of neoplasms showing at least partial morphologi- had multifocal perivascular epithelioid cell tumor cal or immunohistochemical evidences of a p