alveolar soft part sarcoma

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  • Clinical

    • age: adolescents, young adults
    • site: extremities, head/neck
  • Histology

    • architecture: nests, alveolar structures, thin fibrovascular septa. In children can be sheets.
    • cytology: large epithelioid cells, pink granular cytoplasm. Can also be clear.
    • cells often have inclusions
  • Stains

    • variable: desmin, myoD1, S100, NSE
    • negative: myogenin, synaptophysin, chromogranin, CK, EMA
  • Molecular

    • der(17)t(X;17), TFE3/ASPL fusion