gms | German Medical Science

Gemeinsame Jahrestagung der Deutschen, Österreichischen und Schweizerischen Gesellschaft für Thoraxchirurgie

24.-26.10.2013, Basel, Schweiz

Pulmonary artery sarcoma mimicking chronic thromboembolic pulmonary hypertension (CTEPH)

Meeting Abstract

  • M. Zaatar - Department of Thoracic Surgery, Kerckhoff-Clinic, Bad Nauheim
  • C. Wiedenroth - Department of Thoracic Surgery, Kerckhoff-Clinic, Bad Nauheim
  • T. Kramm - Department of Thoracic Surgery, Kerckhoff-Clinic, Bad Nauheim
  • S. Beqiri - Department of Thoracic Surgery, Kerckhoff-Clinic, Bad Nauheim
  • S. Guth
  • E. Mayer

Deutsche Gesellschaft für Thoraxchirurgie. Österreichische Gesellschaft für Thoraxchirurgie. Schweizerische Gesellschaft für Thoraxchirurgie. Gemeinsame Jahrestagung der Deutschen, Österreichischen und Schweizerischen Gesellschaft für Thoraxchirurgie. Basel, Schweiz, 24.-26.10.2013. Düsseldorf: German Medical Science GMS Publishing House; 2013. DocP12

doi: 10.3205/13dgt064, urn:nbn:de:0183-13dgt0649

Veröffentlicht: 14. Oktober 2013

© 2013 Zaatar et al.
Dieser Artikel ist ein Open Access-Artikel und steht unter den Creative Commons Lizenzbedingungen ( Er darf vervielfältigt, verbreitet und öffentlich zugänglich gemacht werden, vorausgesetzt dass Autor und Quelle genannt werden.



Objective: Pulmonary artery sarcoma is a rare malignancy which is often mistaken for pulmonary thromboembolism. The presentation is one of central pulmonary artery obstruction and progressive right-heart failure.

Methods: A 43-year-old woman presented to her general practitioner with chronic cough and moderate dyspnea on exertion. She received a symptomatic treatment. Two weeks later she was admitted to a hospital as an emergency with massive dyspnea. A central bilateral pulmonary artery embolism was diagnosed by computed tomography (CT) and was immediately treated with intravenous heparin followed by oral anticoagulation. In the clinical follow-up the CT showed an enlargment of the “thromboembolic mass”. A thrombolysis with rt-PA was unsuccessful. The pulmonary artery mass remained extending from the right ventricular outflow tract to both pulmonary artery branches (Figure 1 [Fig. 1]). The echocardiography showed an increased right ventricular afterload with a systolic pulmonary artery pressure of > 90 mmHg. The patient was referred to our hospital with the presumptive diagnosis of CTEPH for further diagnostic and surgery.

After discussion in our interdisciplinary CTEPH-board, CTEPH was withdrawn and surgery recommended for the assumed diagnosis of pulmonary artery sarcoma. Surgery was performed in September 2012. Via median sternotomy cardio-pulmonary bypass with deep hypothermia was initiated. The left main pulmonary artery was incised and the tumor resected by endarterectomy in an interrupted circulatory arrest time of 23 minutes. A right pneumonectomy was mandatory due to tumor invasion of the media layer of the pulmonary artery. The histopathological examination confirmed the diagnosis of a pulmonary artery sarcoma.

Results: The postoperative course was uneventful. The patient was discharged on post-operative day 12. Nine months postoperatively the patient is alive in reduced clinical condition (ECOG 2), and in complete remission. Adjuvant chemotherapy was recommended but was not performed due to the performance status.

Conclusion: In patients with suspected chronic thromboembolic pulmonary hypertension, these key-points guide to the diagnosis pulmonary artery sarcoma: central pulmonary artery mass, increasing volume despite sufficient anticoagulation and contrast medium uptake. Surgical resection with or without adjuvant chemotherapy currently offers the only chance for long-term survival.