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Epithelioid Hemangioendothelioma of the Lung Presenting With Pneumonia and Heart Rhythm Disturbances in a Teenage Girl

Reich, Susanne MD*; Ringe, Hannelore MD*; Uhlenberg, Birgit MD; Fischer, Berit MD; Varnholt, Verena MD*

Journal of Pediatric Hematology/Oncology:
doi: 10.1097/MPH.0b013e3181d413b2
Original Articles

The epithelioid hemangioendothelioma (EHE) is a rare low-grade tumor of vascular origin that may arise at any site. However, lung and liver represent the 2 main locations. Symptoms of the pulmonary EHE are usually nonspecific and mild. Distant metastases of PEHE are frequent. However, heart metastases have only been reported in connection with primary EHE of the liver. We describe the case of a 15-year-old girl presenting with an abscess forming pneumonia and severe rhythm disturbances associated with an EHE of the lung. The untypical fulminant clinical course, the surgical interventions, and the involvement of the heart as a life threatening complication, eventually on the basis of cardiac metastases of PEHE, are emphasized.

Author Information

*Pediatric Intensive Care Unit

Clinic of Pediatric Neurology

Clinic of Pediatric Surgery, Charité—Universitätsmedizin Berlin, Germany

Reprints: Susanne Reich, MD, Klinik für Allgemeine Pädiatrie, Charité—Universitätsmedizin, Augustenburger Platz 1, D-13353 Berlin, Germany (e-mail:

Received for publication October 19, 2009; accepted January 9, 2010

© 2010 Lippincott Williams & Wilkins, Inc.