title: Alveolar soft-part sarcoma responsive to intensive chemotherapy.
abstract: Alveolar soft-part sarcoma (ASPS) is a very rare malignant tumor, usually of the extremities, and is seen most often in adolescents and young adults. Surgical excision of the primary and pulmonary metastases has resulted in prolonged survival in some patients, but adjuvant radiation and/or chemotherapy are generally thought to be ineffective. The authors describe a 13-year-old patient with ASPS of an extremity who presented with multiple bilateral pulmonary metastases at diagnosis. Following intensive multiagent chemotherapy, pulmonary metastases showed in vivo evidence of tumor death. The patient has remained disease-free for 10 years following treatment.
authors.affiliation: Department of Pediatrics, Marshfield Clinic, WI 54449, USA. nickerson.harlan@marshfieldclinic.org
authors.forename: H James
authors.initials: HJ
authors.lastname: Nickerson
authors.forename: Teresa
authors.initials: T
authors.lastname: Silberman
authors.forename: F Stig
authors.initials: FS
authors.lastname: Jacobsen
authors.forename: Bruce R
authors.initials: BR
authors.lastname: Krawisz
authors.forename: Hope S
authors.initials: HS
authors.lastname: Maki
authors.forename: Carola A S
authors.initials: CA
authors.lastname: Arndt
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D004317:Doxorubicin / Q000008:administration & dosage; D005260:Female; D006801:Humans; D007866:Leg; D015080:Mesna / Q000008:administration & dosage; D009362:Neoplasm Metastasis / Q000473:pathology; D012509:Sarcoma / Q000188:drug therapy* / Q000473:pathology*; D016896:Treatment Outcome; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D014750:Vincristine; D004317:Doxorubicin; D015080:Mesna