Turkish Journal of Pathology

Türk Patoloji Dergisi

Turkish Journal of Pathology

Turkish Journal of Pathology

2018, Vol 34, Num, 1     (Pages: 082-086)

Perivascular Epithelioid Cell Tumor (PEComa) of the Uterine Cervix in a Patient with Tuberous Sclerosis Complex: A Literature Review

Handan ÇELİK 1, Mehmet KEFELİ 2, Mehmet ÇETİNKAYA 1, Levent YILDIZ 2

1 Departments of Obstetrics and Gynecology, Ondokuz Mayıs University, Faculty of Medicine, SAMSUN, TURKEY
2 Departments of Pathology, Ondokuz Mayıs University, Faculty of Medicine, SAMSUN, TURKEY

DOI: 10.5146/tjpath.2014.01274
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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.

Keywords : Perivascular epithelioid cell neoplasm, Uterine cervical neoplasm, Tuberous sclerosis