Pecoma Arising in the Buttock Region: A Call for Increased Awareness of This Tumor
American Journal of Dermatopathology
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.
Rehman R, Aoun M, Levitin R, Quinn TJ, Kabolizadeh P. Pecoma arising in the buttock region: a Call for increased awareness of this tumor. American Journal of Dermatology 2021 Aug; v.43 Supp.; p.1-14.