TY - JOUR
T1 - Primary Adrenal Epithelioid Sarcoma in a Child
T2 - A Case Report with Literature Review
AU - Monappa, Vidya
AU - Singh, Varun Kumar
AU - Chawla, Arun
PY - 2020/1/1
Y1 - 2020/1/1
N2 - 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.
AB - 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.
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U2 - 10.1080/15513815.2020.1745972
DO - 10.1080/15513815.2020.1745972
M3 - Article
C2 - 32275460
AN - SCOPUS:85083550297
JO - Pediatric Pathology and Laboratory Medicine
JF - Pediatric Pathology and Laboratory Medicine
SN - 1551-3815
ER -