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J Thorac Cardiovasc Surg 2008;135:949-950
© 2008 The American Association for Thoracic Surgery


Brief Communication

Primary intimal pulmonary artery sarcoma: A diagnostic challenge

Hans Scheffel, MDa, Paul Stolzmann, MDa, André Plass, MDb, Achim Weber, MDc, René Prêtre, MDb, Borut Marincek, MDa, Hatem Alkadhi, MDa,*

a Institute of Diagnostic Radiology, University Hospital Zurich, Zurich, Switzerland
b Clinic for Cardiovascular Surgery, University Hospital Zurich, Zurich, Switzerland
c Institute of Surgical Pathology, University Hospital Zurich, Zurich, Switzerland

Received for publication November 9, 2007; accepted for publication November 12, 2007.

* Address for reprints: Hatem Alkadhi, MD, Institute of Diagnostic Radiology, Department of Medical Radiology, Rämistrasse 100, 8091 Zurich, Switzerland. (Email: hatem.alkadhi@usz.ch).

The first 20% of the full text of this article appears below.

Primary pulmonary artery sarcoma (PAS), which was first described by Mandelstamm in 1923,1Go is a rare entity that is often misdiagnosed as chronic pulmonary thromboembolism.2Go The differentiation of these 2 diseases with similar clinical presentations is fundamental for the initiation of adequate therapy, which completely differs between the 2 conditions.3Go PAS often has a fatal outcome because of insidious intravascular tumor growth with extension into the distal pulmonary artery system. Surgery and adjacent irradiation or chemotherapy are necessary to optimize the survival time that ranges between 14 and 18 months.4Go Computed tomography (CT) can serve as one component in the diagnostic workup of PAS by providing characteristic imaging features that favor a PAS diagnosis over that of a thromboembolism.5Go We present the CT findings of a patient with primary intimal PAS of the pulmonary trunk along with the intraoperative images and histopathologic findings.

Clinical Summary

A 38-year-old female patient with recurrent atypical chest pain was admitted to the University Hospital Zurich. Her medical history was remarkable with regard to a long-standing pulmonary arterial hypertension. She had . . . [Full Text of this Article]







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