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2018, Volume 34, Number 1, Page(s) 082-086
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DOI: 10.5146/tjpath.2014.01274 |
Perivascular Epithelioid Cell Tumor (PEComa) of the Uterine Cervix in a Patient with Tuberous Sclerosis Complex: A Literature Review |
Handan ÇELİK1, Mehmet KEFELİ2, Mehmet ÇETİNKAYA1, Levent YILDIZ2 |
1Departments of Obstetrics and Gynecology, Ondokuz Mayıs University, Faculty of Medicine, SAMSUN, TURKEY
2Departments of Pathology, Ondokuz Mayıs University, Faculty of Medicine, SAMSUN, TURKEY |
Keywords:
Perivascular epithelioid cell neoplasm, Uterine cervical neoplasm, Tuberous sclerosis |
Perivascular epithelioid cell tumors (PEComa) are a rare type of
mesenchymal tumor arising from perivascular epithelial cells. These
tumor cells are a co-expression of both melanocytic and myogenic
antigens, such as HMB 45 and smooth muscle actin, and at least in
some patients, are located around vessels. PEComas has been reported
at various sites, including visceral organs, soft tissue, the prostate
gland and broad ligaments. In the female reproductive system, the
uterine corpus is the most common site of involvement. Some cases
are related to tuberous sclerosis complex. Cervical PEComa with
tuberous sclerosis complex is presented in the case of a 41 year-old
and the literature is reviewed. There have been only eight cases of
cervical PEComas and only one other case associated with tuberous
sclerosis complex reported to date.
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