Acute Pulmonary Artery Obstruction as the Primary Manifestation of a Rapidly Growing Intimal Sarcoma in a 54-Year-Old Patient

Abstract

Pulmonary artery sarcoma is a rare malignant neoplasm that is often misdiagnosed and most often only recognized postmortem during the autopsy. We present the case of a male patient with a rapidly progressive pulmonary tumor who underwent urgent pneumonectomy for increasing symptoms of chest pain and septic clinical picture. Histological analysis revealed the diagnosis of a pulmonary artery sarcoma. Despite an R1-resection and adjuvant chemotherapy, the patient is in good clinical health and free of tumor relapse 1 year after the surgery.

Keywords

thoracic surgery - bronchial disease - chest wall - histology - lung cancer treatment - lung cancer - diagnosis

• References

• 1 Mandelstamm M. Über primäre Neubildungen des Herzens. Virchows Arch Pathol Anat 1923; 245: 43-54
• 2 El-Sayed Ahmed MM, Aftab M, Al-Najjar RM, de la Cruz KI, Benjamin RS, Hallman CH. Pulmonary artery sarcoma mimicking pulmonary embolism. Tex Heart Inst J 2014; 41 (5) 515-517
• 3 Grimer R, Judson I, Peake D, Seddon B. Guidelines for the management of soft tissue sarcomas. Sarcoma 2010; 2010: 506182
• 4 Nonomura A, Kurumaya H, Kono N , et al. Primary pulmonary artery sarcoma. Report of two autopsy cases studied by immunohistochemistry and electron microscopy, and review of 110 cases reported in the literature. Acta Pathol Jpn 1988; 38 (7) 883-896
• 5 Blackmon SH, Rice DC, Correa AM , et al. Management of primary pulmonary artery sarcomas. Ann Thorac Surg 2009; 87 (3) 977-984
• 6 Mussot S, Ghigna MR, Mercier O , et al. Retrospective institutional study of 31 patients treated for pulmonary artery sarcoma. Eur J Cardiothorac Surg 2013; 43 (4) 787-793
• 7 Coli A, Parente P, Bigotti G. Pulmonary artery sarcoma: an insidious tumor still diagnosed too late. Analysis of the literature and report of a case. J Exp Clin Cancer Res 2007; 26 (1) 151-156
• 8 Cox JE, Chiles C, Aquino SL, Savage P, Oaks T. Pulmonary artery sarcomas: a review of clinical and radiologic features. J Comput Assist Tomogr 1997; 21 (5) 750-755
• 9 Wong HH, Gounaris I, McCormack A , et al. Presentation and management of pulmonary artery sarcoma. Clin Sarcoma Res 2015; 5 (1) 3
• 10 Mellwig KP, Schmidt H, Gleichmann U, Raute-Kreinsen U. Metastasizing round-cell sarcoma of the right atrium [in German]. Dtsch Med Wochenschr 1991; 116 (40) 1505-1508