title: Cardiac angiosarcoma management and outcomes: 20-year single-institution experience.
abstract: OBJECTIVE
  To identify the clinicopathologic characteristics, treatments, and outcomes of a series of patients with primary cardiac angiosarcoma (AS).
  
  
  METHODS
  This retrospective case series was set in a tertiary referral center with a multidisciplinary clinic. Consecutive patients with institutionally confirmed pathologic diagnosis of cardiac AS from January 1990 to May 2011 were reviewed. Main outcome measures included patient demographics, tumor characteristics, management strategies, disease response, and survival.
  
  
  RESULTS
  Data from 18 patients (78 % male) were reviewed. Sixteen patients (89 %) had AS originating in the right atrium. At diagnosis, eight patients (44 %) had localized/locally advanced disease and ten patients (56 %) had metastatic disease. Initial treatment strategies included resection (44 %), chemotherapy (39 %), and radiotherapy (11 %). Of the eight patients with localized/locally advanced AS, two underwent macroscopically complete resection with negative microscopic margins, one underwent macroscopically complete resection with positive microscopic margins, one underwent macroscopically incomplete resection, two received chemotherapy followed by surgery and intraoperative radiotherapy, one received chemotherapy alone, and one died before planned radiotherapy. Median follow-up was 12 months. Median overall survival (OS) was 13 months for the entire cohort; median OS was 19.5 months for those presenting with localized/locally advanced AS and 6 months for those with metastatic disease at presentation (p = 0.08). Patients who underwent primary tumor resection had improved median OS compared with patients whose tumors remained in situ (17 vs. 5 months, p = 0.01).
  
  
  CONCLUSIONS
  Cardiac AS is associated with poor prognosis. Resection of primary tumor should be attempted when feasible, as OS may be improved. Nevertheless, most patients die of disease progression.
authors.affiliation: Division of Surgical Oncology, Brigham and Women's Hospital, Center for Sarcoma and Bone Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA. n.lookhong@utoronto.ca
authors.forename: Nicole J
authors.initials: NJ
authors.lastname: Look Hong
authors.forename: Prakash K
authors.initials: PK
authors.lastname: Pandalai
authors.forename: Jason L
authors.initials: JL
authors.lastname: Hornick
authors.forename: Prem S
authors.initials: PS
authors.lastname: Shekar
authors.forename: David C
authors.initials: DC
authors.lastname: Harmon
authors.forename: Yen-Lin
authors.initials: YL
authors.lastname: Chen
authors.forename: James E
authors.initials: JE
authors.lastname: Butrynski
authors.forename: Elizabeth H
authors.initials: EH
authors.lastname: Baldini
authors.forename: Chandrajit P
authors.initials: CP
authors.lastname: Raut
mesh_terms: D000328:Adult; D000368:Aged; D003131:Combined Modality Therapy; D005260:Female; D005500:Follow-Up Studies; D006338:Heart Neoplasms / Q000401:mortality* / Q000473:pathology / Q000628:therapy*; D006394:Hemangiosarcoma / Q000401:mortality* / Q000473:pathology / Q000628:therapy*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009362:Neoplasm Metastasis; D009364:Neoplasm Recurrence, Local / Q000401:mortality* / Q000473:pathology / Q000628:therapy*; D009367:Neoplasm Staging; D011379:Prognosis; D012189:Retrospective Studies; D015996:Survival Rate; D055815:Young Adult