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Open Access Case Report

Microscopic endometrial perivascular epithelioid cell nodules: a case report with the earliest presentation of a uterine perivascular epithelioid cell tumor

Chia-Lang Fang12, Yun-Ho Lin13 and Wei-Yu Chen124*

Author Affiliations

1 Department of Pathology, School of Medicine, College of Medicine, Taipei Medical University, Taipei, Taiwan

2 Department of Pathology, Wan Fang Hospital, Taipei Medical University, Taipei, Taiwan

3 Deparment of Pathology, Taipei Medical University Hospital, Taipei, Taiwan

4 Department of Pathology, School of Medicine, College of Medicine, Taipei Medical University, 250 Wu-Hsing St, Taipei 11031, Taiwan

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Diagnostic Pathology 2012, 7:117  doi:10.1186/1746-1596-7-117

Published: 3 September 2012

Abstract

Perivascular epithelioid cell (PEC) tumors (PEComas) are a family of related mesenchymal tumors composed of PECs which co-express melanocytic and smooth muscle markers. Although their distinctive histologic, immunohistochemical, ultrastructural, and genetic features have been clearly demonstrated, their histogenesis and normal counterpart remain largely unknown. Precursor lesions of PEComas have rarely been reported. We herein describe a tuberous sclerosis patient with microscopic PEC nodules in the endometrium of adenomyosis, pelvic endometriosis, an ovarian endometriotic cyst, and the endometrium of the uterine cavity. The nodules showed a mixture of spindle-shaped and epithelioid cells concentrically arranged around small arteries. The cells exhibited uniform nuclei, light eosinophilic cytoplasm, and immunoreactivity with HMB-45 and CD10. Some nodules revealed continuity with a PEComa in the myometrium. These findings support microscopic endometrial PEC nodules possibly being precursor lesions of uterine PEComas. The wide distribution of the nodules in the pelvis may be related to the multicentricity of PEComas in tuberous sclerosis patients. Owing to the immunoreactivity with CD10, microscopic endometrial PEC nodules may be misinterpreted as endothelial stromal cells unless melanocytic markers are stained. To the best of our knowledge, this is a case with the earliest manifestation of PEC lesions occurring in the endometrium.

The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/9658280017862643 webcite

Keywords:
Perivascular epithelioid cell; PEComa; Lymphangioleiomyomatosis; CD10; Adenomyosis; Endometriosis; Tuberous sclerosis