title: Primary Pulmonary Angiosarcoma Presenting with Hemoptysis and Ground-Glass Opacity: A Case Report and Literature Review.
abstract: 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 B(4) and B(5), 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.
authors.affiliation: Department of Respiratory Medicine, Wakamatsu Hospital of the University of Occupational and Environmental Health, Japan.
authors.forename: Ikuko
authors.initials: I
authors.lastname: Shimabukuro
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Yatera
authors.forename: Shingo
authors.initials: S
authors.lastname: Noguchi
authors.forename: Yukiko
authors.initials: Y
authors.lastname: Kawanami
authors.forename: Takashi
authors.initials: T
authors.lastname: Iwanami
authors.forename: Chinatsu
authors.initials: C
authors.lastname: Nishida
authors.forename: Kei
authors.initials: K
authors.lastname: Yamasaki
authors.forename: Toshinori
authors.initials: T
authors.lastname: Kawanami
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Ishimoto
authors.forename: Tomoko
authors.initials: T
authors.lastname: So
authors.forename: Hidetaka
authors.initials: H
authors.lastname: Uramoto
authors.forename: Chiharu
authors.initials: C
authors.lastname: Yoshii
authors.forename: Fumihiro
authors.initials: F
authors.lastname: Tanaka
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Mukae
mesh_terms: D000368:Aged; D000740:Anemia / Q000209:etiology; D001932:Brain Neoplasms / Q000556:secondary; D001981:Bronchial Arteries / Q000601:surgery; D004621:Embolization, Therapeutic; D017809:Fatal Outcome; D005260:Female; D006394:Hemangiosarcoma / Q000150:complications* / Q000473:pathology* / Q000601:surgery; D006469:Hemoptysis / Q000209:etiology*; D006491:Hemothorax / Q000209:etiology; D006801:Humans; D008175:Lung Neoplasms / Q000150:complications* / Q000473:pathology* / Q000601:surgery; D018396:Mucin-1 / Q000032:analysis; D019408:Platelet Endothelial Cell Adhesion Molecule-1 / Q000032:analysis / Q000378:metabolism
chemical_list: D018396:Mucin-1; D019408:Platelet Endothelial Cell Adhesion Molecule-1