Benign Perivascular Epithelioid Cell Tumor of the Mesentery Misdiagnosed as a Uterine Fibroid: A Case Report and Review of Literature

Authors

  • Omar Al-Nahhas Department of Emergency, Tawam Hospital, Alain, United Arab Emirates https://orcid.org/0000-0003-3428-2678
  • Aysha Simran Haris Department of Emergency, Sheikh Zayed Military Hospital, Abu Dhabi, United Arab Emirates https://orcid.org/0009-0004-2577-0260
  • Yousif Basim Raad Department of Emergency, Tawam Hospital, Alain, United Arab Emirates
  • Khaled Alaboud Alkheder Department of Emergency, Tawam Hospital, Alain, United Arab Emirates https://orcid.org/0009-0005-7189-5694
  • Kenichi Miyata University Surgery Center Merced, California, United States of America

DOI:

https://doi.org/10.3889/oamjms.2024.11842

Keywords:

Benign PEComa, Perivascular epithelioid cell tumor, Mesenteric PEComa

Abstract

BACKGROUND: Perivascular epithelioid cell tumor (PEComa) is a type of mesenchymal tumor composed of histologically and immunohistochemically distinctive perivascular epithelioid cells. Benign mesenteric PEComa is a rare entity as there has been only one case reported so far. We are presenting a case of benign mesenteric PEComa in a 30-year-old female that presented with abdominal pain and was diagnosed by microscopic morphological examination and immunohistochemical staining. The aim of this case report is to make health-care professionals aware of the diagnostic criteria in similar presentations, treatment modalities offered for this patient, and others reported over the years for malignant as well as benign PEComas and also shedding the light on a new presentation and diagnosis.

CASE REPORT: We reported a case of a 30-year-old female who presented with abdominal pain radiating to the back and rectum. After a thorough physical examination which was unremarkable, a CT scan was done which showed a mesenteric mass. This mass was then resected and sent for histopathology which revealed a PEComa. We report the history, clinical findings, laboratory reports, and gross imaging of a 30-year-old female who presented to our clinic and was diagnosed with a very rare benign mesenteric PEComa.

CONCLUSION: PEComa is a rare tumor, and as PEComas arising from the mesentery being one of the rarer kinds with only 10 cases reported and this case being the 11th, out of those ten cases, only two of them were found to be benign which makes the case we are reporting the second benign mesenteric PEComa reported in English Literature.

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References

Fletcher C, Unni K, Mertens F. Pathology and Genetics of Tumours of Soft Tissue and Bone; 2006. Lyon: IARC Press.

Armah HB, Parwani AV. Malignant perivascular epithelioid cell tumor (PEComa) of the uterus with late renal and pulmonary metastases: A case report with review of the literature. Diagn Pathol. 2007;2(1):45. https://doi.org/10.1186/1746-1596-2-45 PMid:180531815 DOI: https://doi.org/10.1186/1746-1596-2-45

Folpe AL, Mentzel T, Lehr HA, Fisher C, Balzer BL, Weiss SW. Perivascular epithelioid cell neoplasms of soft tissue and gynecologic origin: A clinicopathologic study of 26 cases and review of the literature. Am J Surg Pathol. 2005;29(12):1558-75. https://doi.org/10.1097/01.pas.0000173232.22117.37 PMid:16327428 DOI: https://doi.org/10.1097/01.pas.0000173232.22117.37

Wejman J, Nowak K, Gielniewska L, Komorowska M, Dąbrowski W. PEComa of the mesentery coexisting with colon cancer: A case report. Diagn Pathol. 2015;10(1):31. https://doi.org/10.1186/s13000-015-0265-5 PMid:25896860

Fu X, Jiang JH, Gu X, Li Z. Malignant perivascular epithelioid cell tumor of mesentery with lymph node involvement: A case report and review of literature. Diagn Pathol. 2013;8(1):60. https://doi.org/10.1186/1746-1596-8-60 PMid:23587410 DOI: https://doi.org/10.1186/1746-1596-8-60

Kapur S, Patel NK, Levin MB, Huang R. Malignant mesenteric perivascular epithelioid cell neoplasm presenting as an intra- abdominal fistula in a 49-year-old female. Case Rep Oncol Med. 2014;2014:534175. https://doi.org/10.1155/2014/534175 PMid:25114821 DOI: https://doi.org/10.1155/2014/534175

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. https://doi.org/10.1097/PAP.0b013e31816613b0 PMid:18418088 DOI: https://doi.org/10.1097/PAP.0b013e31816613b0

Bonetti F, Pea M, Martignoni G, Doglioni C, Zamboni G, Capelli P, et al. Clear cell (“sugar”) tumor of the lung is a lesion strictly related to angiomyolipoma-the concept of a family of lesions characterized by the presence of the perivascular epithelioid cells (PEC). Pathology. 1994;26:230-236. https://doi.org/10.1080/00313029400169561 PMid:7991275 DOI: https://doi.org/10.1080/00313029400169561

Wejman J, Nowak K, Gielniewska L, Komorowska M, Dąbrowski W. PEComa of the mesentery coexisting with colon cancer: A case report. Diagn Pathol. 2015;10:31. https://doi.org/10.1186/s13000-015-0265-5 PMid:25896860 DOI: https://doi.org/10.1186/s13000-015-0265-5

Singer E, Yau S, Johnson M. Retroperitoneal PEComa: Case report and review of literature. Urol Case Rep. 2018;19:9-10. https://doi.org/10.1016/j.eucr.2018.03.012 PMid:29888175 DOI: https://doi.org/10.1016/j.eucr.2018.03.012

Shi HY, Wei LX, Sun L, Guo AT. Clinicopathologic analysis of 4 perivascular epithelioid cell tumors (PEComas) of the gastrointestinal tract. Int J Surg Pathol. 2010;18(4):243-7. https://doi.org/10.1177/1066896908330481 PMid:191244500. DOI: https://doi.org/10.1177/1066896908330481

Shi Y, Geng J, Xie H, Wang B. Malignant perivascular epithelioid cell tumor arising in the mesentery: A case report. Oncol Lett. 2015;9(5):2189-92. https://doi.org/10.3892/ol.2015.3037 PMid:261370387 DOI: https://doi.org/10.3892/ol.2015.3037

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Published

2024-03-15

How to Cite

1.
Al-Nahhas O, Haris AS, Raad YB, Alkheder KA, Miyata K. Benign Perivascular Epithelioid Cell Tumor of the Mesentery Misdiagnosed as a Uterine Fibroid: A Case Report and Review of Literature. Open Access Maced J Med Sci [Internet]. 2024 Mar. 15 [cited 2024 Dec. 3];12(1):136-40. Available from: https://oamjms.eu/index.php/mjms/article/view/11842

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Case Report in Surgery

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