Pecoma Arising in the Buttock Region: A Call for Increased Awareness of This Tumor

Document Type

Conference Proceeding

Publication Date

8-2021

Publication Title

American Journal of Dermatopathology

Abstract

Introduction: Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal tumors that have a perivascular distribution. To our knowledge, this is the only reported instance of PEComa in the buttock region presenting with symptoms of discomfort.

Methods: We report a 49-year-old female who presented with a 2-month history of an enlarging mass on her right buttock and discomfort while sitting. Imaging revealed a soft tissue mass with central necrosis, and a small mass in the left upper lobe of the lung. Biopsy with pathology demonstrated high-grade malignancy and epithelioid cells that had clear to eosinophilic cytoplasm. Final pathology during surgical resection confirmed the diagnosis of a PEComa. Neoadjuvant radiation therapy was administered, followed by surgical resection. Repeat imaging after intervention revealed development of diffuse bilateral nodules, necessitating chemotherapy.

Results: Follow-up imaging showed mixed response with substantial improvement in the intrathoracic disease burden, but progression of disease elsewhere.

Conclusions: Early diagnosis and treatment is crucial in improving outcomes in patients with PEComa.

Volume

43

Issue

Supplement

First Page

1

Last Page

14

Comments

Poster Abstracts Presented at the 24th Virtual Joint Meeting of the International Society of Dermatopathology, April 12-16, 2021

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