Indication

Epithelioid sarcoma

Epithelioid sarcoma is a rare, malignant soft tissue neoplasm displaying both epithelial and mesenchymal differentiation, most frequently presenting in adolescents and young adults. Read more about Epithelioid sarcoma

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About Epithelioid sarcoma.

Epithelioid sarcoma is a rare, malignant soft tissue neoplasm displaying both epithelial and mesenchymal differentiation, most frequently presenting in adolescents and young adults.

It predominantly arises in the subcutaneous or deep dermal tissues of the distal extremities—particularly the hands, forearms, and feet—or less commonly as a more aggressive proximal variant in the pelvic girdle, trunk, or genital regions.

Clinically, it typically presents as an indolent, firm, painless mass or ulcerating cutaneous nodule that mimics chronic inflammatory or benign processes.

Pathophysiologically, the malignancy is strongly characterized by the loss of the INI-1/SMARCB1 tumor suppressor protein and exhibits a high propensity for local infiltrative recurrence, regional lymph node metastasis, and hematogenous dissemination, primarily to the lungs.

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