title: Primary cardiac angiosarcoma in a 25-year-old man: excision, adjuvant chemotherapy, and multikinase inhibitor therapy.
abstract: Primary cardiac tumors do not occur frequently, and only one quarter of them, chiefly sarcomas, are malignant. Patients with angiosarcoma typically have a shorter survival time than do patients with other sarcomas, and the prognosis for survival depends strictly on the stage of the disease at the time of diagnosis and the possibility of complete surgical excision. Chemotherapy and radiotherapy have well-established postoperative roles because of the high probability of metastasis. We report the case of a 25-year-old man who presented with pericardial effusion and echocardiographic evidence of an intracavitary right atrial mass but without the bulky, infiltrative growth typical of this location of the disease. Malignancy was suggested by the clinical presentation, the location of the mass in the right side of the heart, and the absence of conditions favoring thrombus formation. After complete surgical excision, the mass was confirmed to be an angiosarcoma. Conventional adjuvant chemotherapy and maintenance therapy with inhibitors of CD117 (c-kit) and vascular endothelial growth factor relieved the patient's clinical symptoms and enabled his long-term, disease-free survival. In addition to reporting this case, we discuss aspects of the diagnosis and treatment of angiosarcoma.
authors.affiliation: Department of Cardiovascular Surgery and Transplants, Monaldi Hospital, 80131 Naples, Italy.
authors.forename: Renato
authors.initials: R
authors.lastname: Bellitti
authors.forename: Marianna
authors.initials: M
authors.lastname: Buonocore
authors.forename: Nicolina
authors.initials: N
authors.lastname: De Rosa
authors.forename: Franco Enrico
authors.initials: FE
authors.lastname: Covino
authors.forename: Beniamino
authors.initials: B
authors.lastname: Casale
authors.forename: Pasquale
authors.initials: P
authors.lastname: Santè
mesh_terms: D000328:Adult; D000970:Antineoplastic Agents / Q000627:therapeutic use; D001549:Benzamides / Q000627:therapeutic use*; D001706:Biopsy; D006348:Cardiac Surgical Procedures; D017024:Chemotherapy, Adjuvant; D017548:Echocardiography, Transesophageal; D006338:Heart Neoplasms / Q000201:enzymology / Q000473:pathology / Q000628:therapy*; D006394:Hemangiosarcoma / Q000201:enzymology / Q000473:pathology / Q000628:therapy*; D006801:Humans; D000068877:Imatinib Mesylate; D008297:Male; D010879:Piperazines / Q000627:therapeutic use*; D047428:Protein Kinase Inhibitors / Q000627:therapeutic use; D019009:Proto-Oncogene Proteins c-kit / Q000037:antagonists & inhibitors / Q000378:metabolism; D011743:Pyrimidines / Q000627:therapeutic use*; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D042461:Vascular Endothelial Growth Factor A / Q000037:antagonists & inhibitors / Q000378:metabolism
chemical_list: D000970:Antineoplastic Agents; D001549:Benzamides; D010879:Piperazines; D047428:Protein Kinase Inhibitors; D011743:Pyrimidines; C467484:VEGFA protein, human; D042461:Vascular Endothelial Growth Factor A; D000068877:Imatinib Mesylate; D019009:Proto-Oncogene Proteins c-kit
keywords: Antineoplastic combined chemotherapy protocols/therapeutic use; chemotherapy, adjuvant; disease-free survival; heart neoplasms/drug therapy/epidemiology/surgery; prognosis; sarcoma/surgery/therapy; treatment outcome