Alveolar soft part sarcoma

Alveolar soft part sarcoma is an exceedingly rare, indolent, but clinically aggressive malignant neoplasm of soft tissue that most commonly affects adolescents and young adults It classically arises within the deep connective tissues or skeletal muscles of the extremities—predominantly the lower limbs—in adults, while frequently presenting in the head and neck region, particularly the orbit or tongue, in children Histopathologically, tumors demonstrate organoid or nested pseudoalveolar architect...

Alveolar soft part sarcoma is an exceedingly rare, indolent, but clinically aggressive malignant neoplasm of soft tissue that most commonly affects adolescents and young adults Histopathologically, tumors demonstrate organoid or nested pseudoalveolar architectures of large cells accompanied by prominent vascularity, predisposing patients to early hematogenous metastases, most commonly to the lungs, brain, and bones

It classically arises within the deep connective tissues or skeletal muscles of the extremities—predominantly the lower limbs—in adults, while frequently presenting in the head and neck region, particularly the orbit or tongue, in children Pathophysiologically, it is defined by an unbalanced translocation, der(17)t(X;17)(p11.2;q25), which creates the pathogenic *ASPSCR1–TFE3* gene fusion and leads to dysregulated transcription

Pathophysiologically, it is defined by an unbalanced translocation, der(17)t(X;17)(p11.2;q25), which creates the pathogenic *ASPSCR1–TFE3* gene fusion and leads to dysregulated transcription Histopathologically, tumors demonstrate organoid or nested pseudoalveolar architectures of large cells accompanied by prominent vascularity, predisposing patients to early hematogenous metastases, most commonly to the lungs, brain, and bones