Uterine PEComas: A Morphologic, Immunohistochemical, and Molecular Analysis of 32 Tumors

The American Journal of Surgical Pathology
Jennifer A BennettEsther Oliva

Abstract

Uterine perivascular epithelioid cell tumors (PEComas) are rare neoplasms that may show overlapping morphology and immunohistochemistry with uterine smooth muscle tumors. In this study, we evaluated the morphologic, immunohistochemical, and molecular features of 32 PEComas, including 11 with aggressive behavior. Two distinct morphologies were observed: classic (n=30) and those with a lymphangioleiomyomatosis appearance (n=2). In the former, patients ranged from 32 to 77 (mean: 51) years and 13% had tuberous sclerosis. Tumors ranged from 0.2 to 17 (mean: 5.5) cm with 77% arising in the corpus. Epithelioid cells were present in 100% and a spindled component was seen in 37%. Nuclear atypia was low (53%), intermediate (17%), or high (30%). Mitoses ranged from 0 to 36 (mean: 6) and 0 to 133 (mean: 19) per 10 and 50 high-power fields, with atypical mitoses present in 30%. Thin and delicate vessels were noted in 100%, clear/eosinophilic and granular cytoplasm in 93%, stromal hyalinization in 73%, necrosis in 30%, and lymphovascular invasion in 10%. All tumors were positive for HMB-45, cathepsin K, and at least one muscle marker, with most expressing melan-A (77%) and/or MiTF (79%). A PSF-TFE3 fusion was identified in one while another...Continue Reading

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Citations

Mar 5, 2019·The American Journal of Surgical Pathology·Sarah ChiangCristina R Antonescu
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Feb 12, 2021·Archives of Gynecology and Obstetrics·Jiao WangDandan Wang
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Aug 7, 2019·Gynecologic Oncology Reports·Rodger RothenbergerCaroline Billingsley
Dec 17, 2020·Seminars in Diagnostic Pathology·Jennifer A Bennett, Esther Oliva
May 1, 2021·Journal of Clinical Medicine·Marek KrawczykMichał Grąt
Jun 17, 2021·Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc·Jennifer A BennettEsther Oliva
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Sep 26, 2021·Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc·Sarah ChiangJason A Konner

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Methods Mentioned

BETA
hysterectomy
Fluorescence

Software Mentioned

Isis
SAS
Stata

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