PEComa-A Rare Uterine Neoplasm: A Case Report

Roya  Padmehr; Saeid  Arasteh; Soheila  Aminimoghaddam; Ali  Rahbari; Mehrdad  Bohloli; Seyed Mohammad  Mir Eskandari; Hamid  Mohabbat Dar; Morvarid  Ahmad Beigi; Negin  Talebi Biderouni

doi:10.18502/jri.v23i3.10015

Roya Padmehr Reproductive Biotechnology Research Center, Avicenna Research Institute, ACECR, Tehran, Iran
Saeid Arasteh Reproductive Biotechnology Research Center, Avicenna Research Institute, ACECR, Tehran, Iran
Soheila Aminimoghaddam Department of Gynecology Oncology, School of Medicine, Iran University of Medical Sciences, Tehran, Iran
Ali Rahbari Assistance Professor of Pathology, Arak University of Medical Sciences, Arak, Iran
Mehrdad Bohloli Department of Surgery, Jam General Hospital, Tehran, Iran
Seyed Mohammad Mir Eskandari Assistance Professor of Anesthesiology and Intensive Care, Tehran University of Medical Sciences, Tehran, Iran
Hamid Mohabbat Dar Shahid Beheshti University of Medical Sciences, Tehran, Iran
Morvarid Ahmad Beigi Reproductive Biotechnology Research Center, Avicenna Research Institute, ACECR, Tehran, Iran
Negin Talebi Biderouni Reproductive Biotechnology Research Center, Avicenna Research Institute, ACECR, Tehran, Iran

DOI: https://doi.org/10.18502/jri.v23i3.10015

Keywords: Case report, Pathology, PEComa, Perivascular epithelioid cell tumor.

Abstract

Background: Perivascular Epithelioid Cell Tumors (PEComas) are rare mesen-chymal tumors originating from perivascular epithelioid cells. The second common affected organ is uterine. Most of PEComas are benign and patients have good prognosis. At the present time, surgery is the main treatment and adjuvant chemo-therapy is used in malignant cases, although the best diagnostic and management method is yet to be discovered considering the rarity of this neoplasm.

Case Presentation: The patient was a 53 year old lady with a history of two vaginal deliveries and no previous surgery. She had severe pelvic pain and underwent MRI with the primary impression of sarcoma. In MRI, she had a 7 cm mass in lower segment of uterus. The patient underwent laparoscopic hysterectomy, bilateral oophorectomy, lymphadenectomy, and omental biopsy in Jam Hospital. Pathologic report of the patient revealed malignant PEComa without lymph node and omentum involvement.

Conclusion: Diagnosis of PEComa before surgery is difficult and its differential diagnoses form uterine leiomyoma or leiomyosarcoma. Final diagnosis can be made after surgical biopsy and immunohistochemistry evaluation. Surgery is still the main treatment and adjuvant therapy is used in high risk patients.