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: bronchial disease; chest wall; diagnosis; histology; lung cancer; lung cancer treatment; thoracic surgery.

Fig. 1

CT scan preoperatively showing a rapid progress of the tumor mass, here it already shows the typical polypoid growth pattern, like a bunch of grapes. CT, computed tomography.

Fig. 2

Resected specimen showing the tumor mass infiltrating the pulmonary artery.

Fig. 3

Histological section of the myxoid tumor mass (EVG-stain, threefold enlargement), with canaliculary expansion inside the pulmonary vessels, spindle-cell formation with fibroelastic components. EVG, Elastic Van Gieson.