title: Primary and metastatic cardiac sarcomas: a 12-year experience at a German heart center.
abstract: BACKGROUND
  Primary and metastatic cardiac sarcomas represent a heterogeneous group of rare neoplasms with varying clinical course and diverse histogenetic line of differentiation. To date, there exist no uniform guidelines for their surgical and/or oncological treatment.
  
  
  METHODS
  We evaluated retrospectively all patients undergoing cardiac surgery for primary or secondary cardiac sarcoma in the period 1999-2011 at the Erlangen Heart Centre to analyze their clinicopathological spectrum, treatment and outcome.
  
  
  RESULTS
  Five patients (3 women & 2 men; mean age: 46 years; range: 28-81 years) had primary cardiac sarcomas (6.7% of primary cardiac tumors) and 4 had cardiac metastasis from soft tissue sarcoma (1 case each of osteosarcoma, myxoid liposarcoma, alveolar soft part sarcoma and pleomorphic spindle cell sarcoma). Primary sarcomas were located in the left atrium (n=3), left ventricle (n=1) and right atrium (n=1). Histological types were myxosarcoma (3), pleomorphic undifferentiated sarcoma (1) and angiosarcoma (1). Four patients died at 2-64 months (mean, 24.5 months). Sarcoma metastasis to the heart developed at a mean of 109.5 months from initial diagnosis (range, 5-240 months). Three of them died of disease at a mean of 14 months after cardiac surgery and one is disease free 34 months after heart transplantation for metastasis.
  
  
  CONCLUSIONS
  Primary and metastatic cardiac sarcomas are very heterogeneous in their histological appearance, clinical presentation and course of the disease. Radical surgery combined with chemoradiation is promising in patients with resectable disease and may significantly prolong survival. Cardiac transplantation represents an emerging strategy for patients with isolated unresectable cardiac involvement.
authors.affiliation: Institute of Pathology, Friedrich-Alexander-University, Erlangen, Germany. abbas.agaimy@uk-erlangen.de
authors.forename: Abbas
authors.initials: A
authors.lastname: Agaimy
authors.forename: Johannes
authors.initials: J
authors.lastname: Rösch
authors.forename: Michael
authors.initials: M
authors.lastname: Weyand
authors.forename: Thomas
authors.initials: T
authors.lastname: Strecker
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D055424:Cardiac-Gated Imaging Techniques; D059186:Chemoradiotherapy, Adjuvant; D005260:Female; D005858:Germany; D006338:Heart Neoplasms / Q000145:classification / Q000401:mortality / Q000473:pathology* / Q000556:secondary / Q000628:therapy; D016027:Heart Transplantation; D006801:Humans; D008297:Male; D059146:Metastasectomy; D008875:Middle Aged; D012189:Retrospective Studies; D012509:Sarcoma / Q000145:classification / Q000401:mortality / Q000473:pathology* / Q000556:secondary / Q000628:therapy; D016019:Survival Analysis; D013997:Time Factors; D036542:Tomography, Spiral Computed; D016896:Treatment Outcome
keywords: Cardiac tumors; angiosarcoma; cardiac sarcoma; cardiac surgery; myxosarcoma; pathology