Journal of Investigative Medicine High Impact Case Reports (Jun 2022)

A Rare Cause of Chronic Hip Pain From PEComa: An Aggressive Mesenchymal Sarcoma

  • Samender Randhawa MD,
  • Jessica Pinsker BS,
  • Madhurya Amirapu MBBS,
  • Metlapalli Venkata Sravanthi MBBS,
  • Prashanth Ashok Kumar MBBS,
  • Komal Akhtar MD

DOI
https://doi.org/10.1177/23247096221103385
Journal volume & issue
Vol. 10

Abstract

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Perivascular epithelioid cell tumors (PEComas) are related to the tuberous sclerosis complex (TSC) and are commonly benign. When malignant, they can be aggressive with local invasion and metastatic spread. Conventional PEComas do not have TFE3 gene rearrangement and are associated with TSC with a preference for an occurrence at a younger age. We report a case of a young male who had progressive chronic hip pain and was found to have a TFE3 -associated PEComa in his pelvic region.