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Year : 2018  |  Volume : 14  |  Issue : 9  |  Page : 533-535

Primary pulmonary epithelioid angiosarcoma: A case report and literature review

1 Department of Respiratory Medicine, The Second Hospital of Shandong University, Jinan, China
2 Department of Thoracic, The Second Hospital of Shandong University, Jinan, China

Date of Web Publication29-Jun-2018

Correspondence Address:
Wei Wang
Department of Respiratory Medicine, The Second Hospital of Shandong University, Jinan
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-1482.176419

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 > Abstract 

Primary pulmonary epithelioid angiosarcoma is an extremely rare malignancy. Herein, we report the case of an elderly Chinese patient with primary pulmonary epithelioid angiosarcoma. The 72-year-old man presented with a 1-month history of persistent hemoptysis and left chest pain and weight loss of 3 kg. A chest computed tomography (CT) scan revealed two masses (maximum size 3.0 cm × 2.0 cm and 0.8 cm × 0.5 cm) in right lower lobe. We performed a left thoracotomy for tumor resection. Pathological examination showed that there was a significant amount of hemorrhage, fibrinous exudates, degeneration, and necrosis. With immunohistochemical analysis, tumor cells had strong expression of CD34, FLI-1, vimentin. Morphological and immunohistochemical findings supported the diagnosis of epithelioid angiosarcoma.

Keywords: Angiosarcoma, epithelioid, immunohistochemistry, pathology, pulmonary

How to cite this article:
Zhang Y, Huang X, Peng C, Wang Y, Wu Q, Wu Z, Shao H, Wang W. Primary pulmonary epithelioid angiosarcoma: A case report and literature review. J Can Res Ther 2018;14, Suppl S2:533-5

How to cite this URL:
Zhang Y, Huang X, Peng C, Wang Y, Wu Q, Wu Z, Shao H, Wang W. Primary pulmonary epithelioid angiosarcoma: A case report and literature review. J Can Res Ther [serial online] 2018 [cited 2019 Oct 16];14:533-5. Available from: http://www.cancerjournal.net/text.asp?2018/14/9/533/176419

 > Introduction Top

Epithelioid angiosarcoma is an extremely rare malignant vascular tumor with a very poor prognosis. Most angiosarcomas occur in skin, spleen, liver, breast, heart, bone, and gastrointestinal tract have been well-documented.[1] Primary epithelioid angiosarcoma of the lung is extremely rare, and only a few primary cases have been reported.[1],[2] Here, we report a case of primary epithelioid angiosarcoma of the lung with reviewing the relevant literature.

 > Case Report Top

A 72-year-old man, with a 1-month history of persistent hemoptysis and left chest pain, was admitted to our hospital in May 2014. He complained about shortness of breath on exertion and weight loss. Computed tomography (CT) scan revealed a 3.0 cm oval-shaped mass in right lower lobe [Figure 1]. His medical history included cerebral hemorrhage with left ineffective limbs, and he had 50 years of smoking history. At admission, the patient presented with pulse rate of 84 beats/min, blood pressure of 154/71 mm Hg, and oxygen saturation of approximately 97%. We preoperatively diagnosed lung malignant tumor.
Figure 1: Computed tomography scan

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The patient underwent a tumor resection with thoracotomy under general anesthesia. By surgical exploration, two masses of sized 3.0 cm × 2.0 cm × 1.8 cm and 0.8 cm × 0.5 cm× 0.5 cm were observed in dorsal segment of right lower lobe. The masses extensively adhered to the parietal pleura with obscure boundaries. The surface of the masses exhibited calcification, and the tissue appeared hard and thickened. Wedge resection was done for frozen pathology, and the result showed malignant tumor; we decided to the left lower lobe and lymph nodes resection.

The postoperative course was uneventful. The patient recovered with no major complication and was discharged from the hospital 10 days after the operation. Pathological examination revealed that the tumors mainly consisted of fibrinous exudates and hemorrhagic and degenerated necrotic tissue, in addition to a small amount of hyperplastic and degenerated fibrous connective tissue. Immunohistochemical analysis showed tumor cells had strong expression of CD34, FLI-1, vimentin [Figure 2].
Figure 2: In hematoxylin and eosin stain (a) cells have large nuclei, and exhibit epithelioid features and there are strongly immunoreactive for CD34 (b) and FLI-1 (c) and vimentin (d) at the immunohistochemical examination. These histological results were suggestive for a pulmonary epithelioid angiosarcoma

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Neither postoperative radiotherapy nor chemotherapy was performed in the patient due to his old age. Currently, 1 year following surgery, the patient is alive and exhibits no evidence of disease recurrence.

 > Discussion Top

Angiosarcoma is a rare malignant tumor derived from vascular endothelium, accounting for only 1–2% of all soft-tissue sarcomas.[1],[2] Epithelioid angiosarcoma involving the lung is a rare disorder with low incidence, especially its primary form. Pulmonary angiosarcomas are usually the result of metastatic disease (pulmonary metastases occur in 60–80% of cutaneous and cardiac angiosarcomas).[3]

Epidemiologically, there is no gender priority and the average age of occurrence is 45 years. Predisposing factors are unknown, may be related to certain stimulating factors such as lucite plombage, chronic empyema, and tuberculous pyothorax.[4] Patients with primary angiosarcoma can present with chest pain, hemoptysis, dyspnea, cough, and weight loss. However, up to 20% of cases are asymptomatic and are detected as an incidental finding at autopsy.[2],[5]

The common radiographic presentation may be multiple peripheral pulmonary nodules, solitary mass with adjacent alveolar pattern and/or variable degree of consolidation, and ground-glass opacity.[6] However, neither chest radiography nor CT scan is able to differentiate epithelioid angiosarcoma of the lung and any other pulmonary malignant neoplasms.

Definitive diagnosis is made on the basis of histopathological and immunohistochemical findings. The microscopic feature is always misleading except some subtle clues such as red blood cells containing intracytoplasmic lumina. Immunohistochemically, the neoplastic cells showed reactivity for endothelial cell markers (CD31, CD34, factor VIII, FLI-1, and Ulex europaeus agglutinin I), epithelial markers (cytokeratins and sometimes epithelial membrane antigen), and vimentin.

Treatment options for pleural angiosarcoma include surgery, chemotherapy, and radiation. Surgery is the best treatment for patients with localized lesions. Vascular embolization can decrease the size of the tumor and control bleeding, and it may be performed before surgery. The effect of radiation therapy is very poor, and the curative effect of chemotherapy needs further evaluation. Wilson et al. have mentioned complete radiographic response of disease by a chemotherapeutic combination of gemcitabine and taxotere.[7] Prognosis of pulmonary angiosarcoma has been shown to be poor with almost all patients dead within months of initial presentation.[8] Our experience with this case suggests that comprehensive and sufficient sample collection and meticulous histological examination are required for accurate diagnosis of this malignancy.

Financial support and sponsorship

The study was funded by the foundation of the Second Hospital of Shandong University.

Conflicts of interest

There are no conflicts of interest.

 > References Top

Patel AM, Ryu JH. Angiosarcoma in the lung. Chest 1993;103:1531-5.  Back to cited text no. 1
López L, Iriberri M, Cancelo L, Gómez A, Uresandi F, Atxotegui V. Persistent hemoptysis secondary to extensive epithelioid angiosarcoma. Arch Bronconeumol 2004;40:188-90.  Back to cited text no. 2
Chen YB, Guo LC, Yang L, Feng W, Zhang XQ, Ling CH, et al. Angiosarcoma of the lung: 2 cases report and literature reviewed. Lung Cancer 2010;70:352-6.  Back to cited text no. 3
Patsios D, de Perrot M, Tsao MS, Weisbrod G. Epithelioid angiosarcoma of the lung: A rare late complication of Lucite plombage. Br J Radiol 2006;79:e36-9.  Back to cited text no. 4
Adem C, Aubry MC, Tazelaar HD, Myers JL. Metastatic angiosarcoma masquerading as diffuse pulmonary hemorrhage: Clinicopathologic analysis of 7 new patients. Arch Pathol Lab Med 2001;125:1562-5.  Back to cited text no. 5
Eichner R, Schwendy S, Liebl F, Huber A, Langer R. Two cases of primary pulmonary angiosarcoma as a rare cause of lung haemorrhage. Pathology 2011;43:386-9.  Back to cited text no. 6
Wilson R, Glaros S, Brown RK, Michael C, Reisman D. Complete radiographic response of primary pulmonary angiosarcomas following gemcitabine and taxotere. Lung Cancer 2008;61:131-6.  Back to cited text no. 7
Ozcelik C, Onat S, Yaldiz M, Ozcelik Z. Primary epithelioid angiosarcoma of the lung presenting as pulmonary hemorrhage. Asian Cardiovasc Thorac Ann 2006;14:69-71.  Back to cited text no. 8


  [Figure 1], [Figure 2]


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