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Int J Clin Exp Pathol 2012;5(9):928-938

Original Article
Primary and metastatic cardiac sarcomas: a 12-year experience at a german heart center

Abbas Agaimy, Johannes Rösch, Michael Weyand, Thomas Strecker

Institute of Pathology and Center of Cardiac Surgery, Friedrich-Alexander-University, Erlangen, Germany

Received August 5, 2012; Accepted September 17, 2012; Epub October 20, 2012; Published October 30, 2012

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.

Keywords: Cardiac tumors, cardiac sarcoma, pathology, cardiac surgery, myxosarcoma, angiosarcoma

Address all correspondence to:
Dr. Abbas Agaimy,
Institute of Pathology, Friedrich-Alexander-University
Krankenhausstrasse 8-10, 91054
Erlangen, Germany.
Phone: +49-9131-85-22288; Fax: +49-9131-85-24745
E-mail: abbas.agaimy@uk-erlangen.de