Perivascular epithelioid cell tumors of the uterine cervix

• Published in: Pathology, research and practice Vol. 212; no. 8; pp. 667 - 671
• Main Authors: Kudela, E., Biringer, K., Kasajova, P., Nachajova, M., Adamkov, M.
• Format: Journal Article
• Language: English
• Published: Germany Elsevier GmbH 01-08-2016
• Subjects: Biomarkers, Tumor - metabolism; Cervix; Cervix Uteri - metabolism; Cervix Uteri - pathology; Cervix Uteri - surgery; Female; Humans; Hysterectomy; Ovariectomy; PEComa; Perivascular Epithelioid Cell Neoplasms - metabolism; Perivascular Epithelioid Cell Neoplasms - pathology; Perivascular Epithelioid Cell Neoplasms - surgery; Prognosis; Tuberous sclerosis; Uterine Cervical Neoplasms - metabolism; Uterine Cervical Neoplasms - pathology; Uterine Cervical Neoplasms - surgery; Tuberous sclerosis; Cervix; PEComa
• ISSN: 0344-0338; 1618-0631
• DOI: 10.1016/j.prp.2016.03.010

The World Health Organization (WHO) defines PEComas as mesenchymal tumors composed of histologically and immunohistochemically distinctive perivascular cells.
Uterus is the most common site of a subgroup of PEComas not otherwise specified(NOS). PEComas of the uterine cervix are extremely rare, and only thirteen cases have been described in the English literature to date. In this review, we summarize the available data concerning diagnostics, immunohistochemical analysis, genetics and treatment of cervical PEComas. Radical hysterectomy with bilateral salpingooophorectomy is the best surgical approach available. Adjuvant therapy in its present form is not efficient. Therefore, further studies are needed to evaluate the newest treatment strategies.