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.

Hemoptysis in a Patient with Multifocal Primary Pulmonary Angiosarcoma

“…However, the above number is certainly to low, as at least several reports have not been identified by the authors of this paper [7,8]. The mean age of patients affected by PPA was 55,9 years (range, 23-82 years), with significant men predominance [6]. The early diagnosis is very difficult due to non-specific signs and symptoms presented by the patients.…”

“…According to one recent paper only 32 case reports had been published to 2015 [6]. However, the above number is certainly to low, as at least several reports have not been identified by the authors of this paper [7,8].…”

“…The data coming from the analysis of 24 PPA cases suggests the median survival time of 3 months (range 0.5 -39 months) [7]. Similarly, almost one third of patients reviewed by Shimabukuro et al died within the first two months after establishing the diagnosis [6]. There have been only few reports of survival time longer than 12 months [5,11,23].…”

“…The majority of reports emphasize the following symptoms: (1) hemoptysis (the most common sign related to the vessel injury [7,9], (2) cough, dyspnea (related to local growth) [7], (3) chest pain [1], (4) non-specific symptoms: weight loss, malaise, fatigue, fever [5,7,10]. Nevertheless, some patients may present the tration [6,11,12]. Pulmonary angiosarcomas are often associated with ground-glass opacity (halo sign) and mediastinal lymphadenopathy [12].…”

Primary Pulmonary Epithelioid Angiosarcoma: A Rare Case Presentation of Bilateral Pneumothoraces

“…Angiosarcoma is a rare malignant neoplasm with a very poor prognosis that originates from the vascular endothelium and accounts for only 1%-2% of all sarcomatous malignancies [1]. It arises most often in the deep soft tissues, although other primary sites have been reported, including the adrenals, thyroid, skin, and bone [2].…”