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
  1. Pea M, Bonetti F, Zamboni G, Martignoni G, Riva M, et al. Melanocyte-marker-HMB-45 is regularly expressed in angiomyolipoma of the kidney. Pathology. 1991;23(3):185-8.
  2. Pea M, Bonetti F, Zamboni G, Martignoni G, Fiore-Donati L, Doglioni C. Clear cell tumor and angiomyolipoma. Am J Surg Pathol. 1991;15(2): 199-202.
  3. Machado I, Cruz J, Lavernia J, Rayon JM, Poveda A, Llombart-Bosch A. Malignant PEComa with metastatic disease at diagnosis and resistance to several chemotherapy regimens and targeted therapy (m-TOR inhibitor). Int J Surg Pathol. 2017;25(6): 543-9.
  4. Musella A, De Felice F, Kyriacou AK, Barletta F, Di Matteo FM, Marchetti C, et al. Perivascular epithelioid cell neoplasm (PEComa) of the uterus: a systematic review. Int J Surg 2015;19:1-5.
  5. Baird DD, Dunson DB, Hill MC, Cousins D, Schectman JM. High cumulative incidence of uterine leiomyoma in black and white women: ultrasound evidence. Am J Obstet Gynecol. 2003; 188(1):100-7.
  6. Sadigh S, Shah P, Weber K, Sebro R, Zhang PJ. Primary malignant perivascular epithelioid cell neoplasm (PEComa) of the bone mimicking granular cell tumor in core biopsy: a case report and literature review. Oncol Lett. 2018;15(3):2946-52.
  7. Conlon N, Soslow RA, Murali R. Perivascular epithelioid tumours (PEComas) of the gynaecological tract. J Clin Pathol. 2015;68(6):418-26.
  8. Sanfilippo R, Jones RL, Blay JY, Le Cesne A, Provenzano S, Antoniou G, et al. Role of chemotherapy, VEGFR inhibitors, and mTOR inhibitors in advanced perivascular epithelioid cell tumors (PEComas). Clin Cancer Res. 2019;25(17): 5295-300.
  9. Pan CC, Chung MY, Ng KF, Liu CY, Wang JS, Chai CY, et al. Constant allelic alteration on chromosome 16p (TSC2 gene) in perivascular epithelioid cell tumour (PEComa): genetic evidence for the relationship of PEComa with angiomyolipoma. J Pathol. 2008;214(3):387-93.
  10. Prasad SR, Sahani DV, Mino-Kenudson M, Narra VR, Humphrey PA, Menias CO, et al. Neoplasms of the perivascular epithelioid cell involving the abdomen and the pelvis: cross-sectional imaging findings. J Comput Assist Tomogr. 2007;31(5): 688-96.
  11. Giannella L, Carpini GD, Montik N, Verdecchia V, Puccio F, Di Giuseppe J, et al. Ultrasound features of a uterine perivascular epithelioid cell tumor (PEComa): case report and literature review. Diagnostics (Basel). 2020;10(8):553.
  12. Pattamapaspong N, Khunamornpong S, Phongnarisorn C, Pojchamarnwiputh S. Malignant perivascular epithelioid cell tumor of the round ligament mimics leiomyoma on computed tomography. Singapore Med J. 2009;50(7):e239-42.
  13. Fletcher C, Bridge JA, Hogendoorn P, Mertens F. WHO classification of tumors of soft tissue and bone. 4th ed. Lyon: IARC Press; 2013. 467 p.
  14. Fadare O. Perivascular epithelioid cell tumor (PEComa) of the uterus: an outcome-based clinicopathologic analysis of 41 reported cases. Adv Anat Pathol. 2008;15(2):63-75.
  15. Jeon IS, Lee SM. Multimodal treatment using surgery, radiotherapy, and chemotherapy in a patient with a perivascular epithelioid cell tumor of the uterus. J Pediatr Hematol Oncol. 2005;27(12):681-4.
  16. Vang R, Kempson RL. Perivascular epithelioid cell tumor (PEComa’) of the uterus: a subset of HMB-45-positive epithelioid mesenchymal neoplasms with an uncertain relationship to pure smooth muscle tumors. Am J Surg Pathol. 2002;26(1):1-13.
Journal of Reproduction & Infertility
Volume 23, Issue 3
Summer 2022
Pages 224-227