Primary Adrenal Epithelioid Sarcoma in a Child: A Case Report with Literature Review

Introduction: Epithelioid sarcoma is a malignant mesenchymal neoplasm with evidence of epithelial differentiation. All the cases reported in the solid organs are of “proximal type” occurring in adults. We report a primary epithelioid sarcoma arising in the adrenal gland of a young male. Case report: An 11-year-old male patient presented with right loin pain. Imaging revealed a 10.8 × 10.8 × 13.5 cm complex cystic mass with obscured right adrenal gland. Clinical and radiological studies did not reveal metastases. Histologic features were those of proximal type epithelioid sarcoma with extensive central necrosis. Immunohistochemistry showed strong positivity for pancytokeratin, vimentin, and CD34. Nuclear expression of SMARCB1 (INI-1) protein was lost. Conclusion: Proximal type of epithelioid sarcoma can arise from solid organs such as the adrenal.