Alveolar soft part sarcoma: an unusually long interval between presentation and brain metastasis

Abstract

While alveolar soft part sarcoma is an uncommon soft tissue tumor known for late metastases to lung, bone, and brain, and interval of 33 years between primary presentation and development of brain metastasis has not been described. We document a patient with a removal of an alveolar soft part sarcoma from the pectoralis major muscle at the age of 10 years, a lung metastasis at the age of 31 years, and brain and renal masses at the age of 43 years. The patient received surgical resections each time, but never radiotherapy or chemotherapy. He is currently alive and well. Immunohistochemistry, karyotypic analysis, flow cytometry, and gene expression were analyzed on primary tumor and established cell cultures in the hopes of further elucidating the histogenesis of this unusual neoplasm.