Journal of Medical Case Reports (Oct 2024)

Conservative treatment in uterine perivascular epithelioid cell tumor of uncertain malignant potential: a case report

  • Ines Zemni,
  • Ines Houissa,
  • Nadia Boujelbene,
  • Saida Sakhri,
  • Imene Sassi,
  • Tarek Ben Dhiab

DOI
https://doi.org/10.1186/s13256-024-04781-z
Journal volume & issue
Vol. 18, no. 1
pp. 1 – 5

Abstract

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Abstract Introduction Perivascular epithelioid cell tumors are uncommon mesenchymal tumors. The genital tract is the most common extrarenal location. Preoperative diagnosis is rarely achieved owing to non-specific symptoms and imaging features. Consensus on treatment strategies remains elusive. Case presentation We report the case a 38 year-old north African woman with a primary sterility, who was diagnosed with a uterine Perivascular epithelioid cell tumor of uncertain malignant potential on a resection specimen of an intracavity polypoid mass. Immunohistochemistry confirmed the diagnosis and we opted for conservative surgery to preserve the patient’s fertility desires. Conclusion Uterine perivascular epithelioid cell tumor is a rare entity that warrants consideration in the differential diagnosis of uterine tumors. Treatment modalities, follow-up protocols, and prognosis remain ambiguous. Given their unpredictable behavior, accurate diagnosis and long-term monitoring are imperative.

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