Journal of Reproduction & Infertility

Journal of Reproduction & Infertility

PEComa-A Rare Uterine Neoplasm: A Case Report

Authors
Roya Padmehr 1
Saeid Arasteh 1
Soheila Aminimoghaddam 2
Ali Rahbari 3
Mehrdad Bohloli 4
Seyed Mohammad Mir Eskandari 5
Hamid Mohabbat Dar 6
Negin Talebi Biderouni 1
1 Reproductive Biotechnology Research Center, Avicenna Research Institute, ACECR, Tehran, Iran
2 Department of Gynecology Oncology, School of Medicine, Iran University of Medical Sciences, Tehran, Iran
3 Assistance Professor of Pathology, Arak University of Medical Sciences, Tehran, Iran
4 Department of Surgery, Jam General Hospital, Tehran, Iran
5 Assistance Professor of Anesthesiology and Intensive Care, Tehran University of Medical Sciences, Tehran, Iran
6 Shahid Beheshti University of Medical Sciences, Tehran, Iran
10.18502/jri.v23i3.10015
Abstract
Background: Perivascular Epithelioid Cell Tumors (PEComas) are rare mesenchymal 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 chemotherapy 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.
Keywords
case report
Pathology
PEComa
Perivascular epithelioid cell tumor
Journal of Reproduction & Infertility
Volume 23, Issue 3
Summer 2022
Pages 224-227