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

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 pr...

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Bibliographic Details
Published inJIM - high impact case reports Vol. 10; p. 23247096221103385
Main Authors Randhawa, Samender, Pinsker, Jessica, Amirapu, Madhurya, Sravanthi, Metlapalli Venkata, Ashok Kumar, Prashanth, Akhtar, Komal
Format Journal Article
LanguageEnglish
Published Los Angeles, CA SAGE Publications 2022
Sage Publications Ltd
SAGE Publishing
Subjects
Basic Helix-Loop-Helix Leucine Zipper Transcription Factors - genetics
Case Report
Case reports
Hip joint
Humans
Male
Pain
Perivascular Epithelioid Cell Neoplasms - complications
Perivascular Epithelioid Cell Neoplasms - diagnosis
Perivascular Epithelioid Cell Neoplasms - genetics
Sarcoma
Tuberous sclerosis
Tumors
epithelioid
sarcoma
metastatic
PEComa
mesenchymal
tumor
TFE3
sirolimus
hip pain
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Summary: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.
Bibliography:ObjectType-Case Study-2
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ISSN:2324-7096
2324-7096
DOI:10.1177/23247096221103385