title: Pediatric angiosarcoma of the heart: a unique presentation and metastatic pattern.
abstract: We report the seventh case of angiosarcoma of the heart in a child. The patient was a 23-month-old female who presented for lower extremity limping and underwent open surgical biopsy of the femur. Immediately postoperatively, she developed pericardial tamponade, and a bulky intracardiac mass was discovered as the underlying cause. The mass was composed of highly pleomorphic tumor cells reactive for the endothelial markers CD31, CD34, and factor VIII-related antigen (FVIII-RA). Staging evaluation revealed widespread metastases involving the brain, ovaries, and bone marrow. She died of complications of metastatic disease 8 months following initial presentation. Unusual features of this case include the young age of the patient, left-sided nature of the cardiac tumor, presentation secondary to metastatic disease, and the pattern of metastases. The literature on cardiac angiosarcoma, which is limited to six case reports in the pediatric population, is also reviewed.
authors.affiliation: Department of Pathology, University of Virginia Health System, Charlottesville 22908, USA.
authors.forename: A M
authors.initials: AM
authors.lastname: Booth
authors.forename: R D
authors.initials: RD
authors.lastname: LeGallo
authors.forename: M H
authors.initials: MH
authors.lastname: Stoler
authors.forename: P E
authors.initials: PE
authors.lastname: Waldron
authors.forename: L A
authors.initials: LA
authors.lastname: Cerilli
mesh_terms: D000951:Antigens, Neoplasm / Q000032:analysis; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D014408:Biomarkers, Tumor / Q000032:analysis; D019046:Bone Marrow Neoplasms / Q000737:chemistry / Q000556:secondary* / Q000628:therapy; D001932:Brain Neoplasms / Q000737:chemistry / Q000556:secondary* / Q000628:therapy; D004317:Doxorubicin / Q000008:administration & dosage; D017809:Fatal Outcome; D005260:Female; D006325:Heart Atria / Q000473:pathology; D006338:Heart Neoplasms / Q000737:chemistry / Q000473:pathology* / Q000628:therapy; D006352:Heart Ventricles / Q000473:pathology; D006394:Hemangiosarcoma / Q000737:chemistry / Q000556:secondary* / Q000628:therapy; D006801:Humans; D007069:Ifosfamide / Q000008:administration & dosage; D007223:Infant; D010051:Ovarian Neoplasms / Q000737:chemistry / Q000556:secondary* / Q000628:therapy; D014750:Vincristine / Q000008:administration & dosage
chemical_list: D000951:Antigens, Neoplasm; D014408:Biomarkers, Tumor; D014750:Vincristine; D004317:Doxorubicin; D007069:Ifosfamide