title: Primary alveolar soft-part sarcoma of the liver: anomalous presentation of a rare disease.
abstract: Alveolar soft-part sarcoma is a highly vascular soft-tissue tumor that is uniformly malignant. It comprises less than 1 per cent of all soft-tissue sarcomas. Patients with alveolar soft-part sarcoma most frequently are aged 15 to 35 years, and the soft tissues of the lower extremities typically are affected. In the pediatric population, it most frequently occurs in the head and neck and particularly affects the tongue and orbit. Alveolar soft-part sarcoma has been described as a primary lesion in the trunk, upper extremities, and retroperitoneum; more novel locations include the mediastinum, female genital tract, stomach, bone, and larynx. Numerous case reports describe alveolar soft-part sarcoma in diverse anatomic locations, but this report is, to our knowledge, the first documentation of primary alveolar soft-part sarcoma of the liver. We describe a 47-year-old woman with such a manifestation. Despite surgical resection and numerous chemotherapeutic regimens, this patient had widespread metastasis and died approximately 2 years after the diagnosis was established.
authors.affiliation: Department of Surgery, Mayo Clinic, Jacksonville, Florida 32224, USA.
authors.forename: Kyle K
authors.initials: KK
authors.lastname: Shaddix
authors.forename: G Peter
authors.initials: GP
authors.lastname: Fakhre
authors.forename: William W
authors.initials: WW
authors.lastname: Nields
authors.forename: Jeffery L
authors.initials: JL
authors.lastname: Steers
authors.forename: Winston R
authors.initials: WR
authors.lastname: Hewitt
authors.forename: David M
authors.initials: DM
authors.lastname: Menke
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use; D017809:Fatal Outcome; D005260:Female; D006498:Hepatectomy; D006801:Humans; D008113:Liver Neoplasms / Q000175:diagnosis* / Q000188:drug therapy / Q000601:surgery; D008875:Middle Aged; D018234:Sarcoma, Alveolar Soft Part / Q000175:diagnosis* / Q000188:drug therapy / Q000601:surgery
chemical_list: D000970:Antineoplastic Agents