Pulmonary Angiosarcoma Presenting as Spontaneous Recurrent Hemothorax
Andrea Campione, MD,
Giuseppe Forte, MD1,
Luca Luzzi, MD,
Alberto Comino, MD1,
Alberto Gorla, MD,
Alberto Terzi, MD
Thoracic Surgery Unit
1 Department of Pathology, Hospital of Cuneo, Cuneo, Italy
Andrea Campione, MD, Tel: +390171642286, Fax: +390171642491, Email: campioneandrea{at}libero.it, Thoracic Surgery Hospital of Cuneo, Via Coppino 26 12100, Cuneo, Italy.
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ABSTRACT
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An incidental diagnosis of pulmonary angiosarcoma was made after surgical exploration for repeated episodes of bleeding in an 85-year-old woman. Spontaneous hemothorax is uncommon and deserves detailed investigation.
Key Words: Hemangiosarcoma • Hemothorax • Lung Neoplasms
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INTRODUCTION
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Spontaneous hemothorax is uncommon and usually related to metastatic malignant pleural disease, anticoagulant drugs, thrombocytopenia, primary vascular bleeding from intrathoracic aneurysmal rupture, or arteriovenous malformation.1 Hemothorax can be the presenting symptom in cardiac angiosarcoma, Ewing sarcoma of the diaphragm, or metastatic and primary pleural angiosarcomas.1
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CASE REPORT
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An 85-year-old white woman was referred to our hospital with productive cough and worsening dyspnea. Her history included slow neurological decline and consequent repeated trauma. Physical examination on admission revealed dullness on right chest percussion, without adenopathy, palpable tumors, or any sign of heart failure. Chest radiography showed a massive right pleural effusion. Laboratory data were normal except for a moderate degree of anemia (hemoglobin, 9 g·dL–1). Pleural puncture revealed bloody effusion, with a pleural hematocrit close to 50% of the peripheral blood hematocrit. A chest drain was inserted and 3,000 mL of hemorrhagic fluid was removed. After complete clinical and radiological resolution of the pleural effusion, the drain was removed and discharge of the patient was planned, but a chest radiograph surprisingly revealed recurrent right pleural effusion associated with extrapleural hemorrhage (Figure 1A
). An urgent computed tomography scan suggested bleeding from a small intrathoracic vessel (Figure 1B), and although laboratory and hemodynamic parameters were stable, an urgent thoracotomy was performed. Large clots and a small quantity of blood were removed. No active bleeding site was detected in the pleural cavity, but there was an area of pulmonary infarction with adhesion to the thoracic wall, which was resected. Microscopic examination of the wedge resection specimen revealed lung involvement by a malignant neoplasm exhibiting proliferation of variably sized vascular channels with irregular branching, epithelioid-like endothelial lining of vascular spaces, abundant cytoplasm with pleomorphic nuclei and prominent nucleoli, and mitotic figures in atypical fashion. On immunohistochemical examination, the neoplastic cells were positive for CD31, vimentin, Ki-67 proliferation marker, and cytokeratin AE1/AE3, while they were negative for CD34, factor VIII-related antigen, CEA, and calretinin (Figure 2). All these pathologic features were distinctive of epithelioid angiosarcoma.2 The postoperative course was uneventful, and the patient was transferred to a rehabilitation center. Adjuvant chemotherapy with epirubicin and ifosfamide was excluded because of her advanced age and performance status. She died 3 months later in another hospital, probably after developing respiratory failure, but no postmortem examination was performed.
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Figure 1. Chest radiograph and computed tomography scan performed before the scheduled discharge of the patient.
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Figure 2. Specimen stained with hematoxylin-eosin (left) and with anti-CD31 antibodies (right). Original magnification, 400x and 200x respectively.
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DISCUSSION
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Primary intrathoracic angiosarcomas are rare.3,4 Angiosarcoma is the most common primary cardiac tumor, usually presenting as a right atrial mass.3 The presence of such tumors in the lung is usually due to metastasis from a primary site, but there have been cases in which no other primary site has been detected.3 The rare cases of pulmonary angiosarcoma have presented with diffuse pulmonary hemorrhage, hemoptysis, multiple bilateral nodules, or massive spontaneous hemothorax secondary to metastatic lung involvement.1 Moriya and colleagues1 reviewed cases of bleeding from lung and pleural angiosarcomas, but they did not assess the incidence of primary pulmonary angiosarcoma, which is extremely rare, with less than 11 cases reported in the literature.5
History of irradiation and chronic tuberculous pyothorax can be predisposing factors, but none of these were present in our case, which was unique for the recurrent episodes of intrapleural bleeding, absence of hemoptysis, and the solitary pulmonary origin of the lesion. A presumed diagnosis of primary angiosarcoma of the lung was supported by the absence of diffuse lung and brain involvement on chest and brain computed tomography, the presence of unilateral hemothorax, and the absence of detectable soft tissue masses. Immunohistochemical examination was essential for the diagnosis, with the unusual characteristic of negative CD34 and factor VIII-related antigen staining, as previously reported by Lund.2 Spontaneous hemothorax is uncommon and deserves detailed investigation.
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REFERENCES
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- Moriya Y, Sugawara T, Arai M, Tsuda Y, Uchida K, Noguchi T, et al. Bilateral massive bloody pleurisy complicated by angiosarcoma. Intern Med 2007;46:125–8.[Medline]
- Lund L, Amre R. Epithelioid angiosarcoma involving the lungs. Arch Pathol Lab Med 2005;129:7–10.
- Salter DM. Pulmonary and thoracic sarcomas. Curr Diagn Pathol 2006;12:409–17. Available at: http://www.journals.elsevierhealth.com/periodicals/ycdip/issues/contents?issue_key=S0968-6053(06)X0037-6. Accessed September 10, 2008.
- Sheppard MN, Hansell DM, Du Bois RM, Nicholson AG. Primary epithelioid angiosarcoma of the lung poresenting as pulmonary hemorrhage. Human Pathol 1997;28:383–5.[Medline]
- Pandit SA, Fiedler PN, Westcott JL. Primary angiosarcoma of the lung. Ann Diagn Pathol 2005;9(5):302–4.[Medline]
Asian Cardiovasc Thorac Ann 2009;
17:84-85
© 2009 by SAGE Publications
DOI: 10.1177/0218492309102544
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ABSTRACT
INTRODUCTION
