Tohoku J. Exp. Med., 2015 December, 237(4)

Primary Pulmonary Angiosarcoma Presenting with Hemoptysis and Ground-Glass Opacity: A Case Report and Literature Review

IKUKO SHIMABUKURO,¹ KAZUHIRO YATERA,² SHINGO NOGUCHI,¹ YUKIKO KAWANAMI,² TAKASHI IWANAMI,³ CHINATSU NISHIDA,² KEI YAMASAKI,² TOSHINORI KAWANAMI,² HIROSHI ISHIMOTO,² TOMOKO SO,³ HIDETAKA URAMOTO,³ CHIHARU YOSHII,¹ FUMIHIRO TANAKA³ and HIROSHI MUKAE²

¹Department of Respiratory Medicine, Wakamatsu Hospital of the University of Occupational and Environmental Health, Japan, Kitakyushu, Fukuoka, Japan
²Department of Respiratory Medicine, University of Occupational and Environmental Health, Japan, Kitakyushu, Fukuoka, Japan
³Second Department of Surgery, University of Occupational and Environmental Health, Japan, Kitakyushu, Fukuoka, Japan

Angiosarcoma originates from the vascular endothelium and accounts for only 1-2% of all sarcomatous malignancies. The skin is one of the most common primary sites. However, primary pulmonary angiosarcoma is rare, and only 31 cases of primary pulmonary angiosarcoma have been reported. A 79-year-old Japanese female developed bloody sputum, and chest X-ray and chest computed tomography (CT) showed consolidation with ground-glass opacity (GGO) on the right middle and lower lung fields. The bronchoscopic findings demonstrated bleeding from the right B⁴ and B⁵, and bronchial arterial embolization was subsequently performed. However, the CT findings demonstrated new pulmonary nodules with GGO in the left lung, and she presented with hemosputum and a progression of anemia. Right hemothorax also occurred, and surgical lung resection was performed to control the bleeding. The pathological analysis of the resected lung revealed the focal growth of atypical alveolar epithelioid-like cells that were positive for CD31 and negative for epithelial membrane antigen, and these atypical cells were replacing the endothelium of the pulmonary artery. Based on these findings, the patient was diagnosed with pulmonary angiosarcoma, which might originate from the endothelium of the pulmonary artery. No abnormal findings were observed except for the pulmonary involvement on systemic CT scanning. However, the patient passed away due to respiratory failure with compression of the brainstem caused by brain metastasis three months after the first visit. Early surgical resection is the important treatment for patients with angiosarcoma. In addition, we review the literature reporting the patients with primary pulmonary angiosarcoma.

keywords —— angiosarcoma; hemoptysis; lung cancer; pathology; thoracic surgery

© 2015 Tohoku University Medical Press

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Tohoku J. Exp. Med., 2015, 237, 273-278

Correspondence: Kazuhiro Yatera, M.D., Ph.D., Department of Respiratory Medicine, University of Occupational and Environmental Health, Japan, 1-1 Iseigaoka, Yahatanishiku, Kitakyushu, Fukuoka 807-8555, Japan.

e-mail: yatera@med.uoeh-u.ac.jp