Asian Cardiovasc Thorac Ann 2006;14:69-71
© 2006 Asia Publishing EXchange Ltd
Primary Epithelioid Angiosarcoma of the Lung Presenting as Pulmonary Hemorrhage
Cemal Ozcelik, MD,
Serdar Onat, MD,
Mehmet Yaldiz, MD1,
Zerrin Ozcelik, PhD2
Department of Thoracic Surgery
1 Department of Pathology
2 Department of Pharmacology, Dicle University School of Medicine, Diyarbakir, Turkey
For reprint information contact: Cemal Ozcelik, MD Tel: 90 412 248 8001 Fax: 90 412 248 8520 Email: cozcelik{at}dicle.edu.tr, Department of Thoracic Surgery, Dicle University School of Medicine, Diyarbakir 21280, Turkey.
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ABSTRACT
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Pulmonary angiosarcomas are usually secondary tumors, and only a few primary cases have been reported. We report a unique case of epithelioid angiosarcoma presenting as a solitary mass in the right upper lobe with pulmonary hemorrhage. Because of its epithelioid histology, this tumor may resemble a carcinoma or a variety of vascular lesions with epithelioid endothelial cells. Therefore, the diagnosis of epithelioid angiosarcoma should be based on immunohistochemical staining.
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INTRODUCTION
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Primary pulmonary angiosarcoma is extremely rare. An angiosarcoma found in the lung most likely represents metastasis from a primary tumor of the pulmonary arteries, the heart, or a distant site.1–3 Although the clinical manifestations and the course of angiosarcomas in the lung are not well known, it is clear from reported cases that these angiosarcomas are associated with pulmonary hemorrhage and generally have a poor prognosis.4,5 We report a rare instance of primary epithelioid angiosarcoma of the lung presenting as a solitary mass in the right upper lobe with pulmonary hemorrhage in that lobe.
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CASE REPORT
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A 62-year-old man presented with cough, hemoptysis, right-sided chest pain, fatigue, and weight loss of 5-month duration. His past medical history and physical examination were unremarkable, except for exposure to asbestos. He had initially been treated for pneumonia with various antibiotics in another medical center but had remained symptomatic.
The patient’s laboratory test results were normal except for a hematocrit of 27%, hemoglobin level of 9.6 mg·dL–1, and erythrocyte sedimentation rate of 120 mm·h–1. Pulmonary function studies showed a forced expiratory volume in 1 second of 3.06 L (91% of predicted value) and forced vital capacity of 3.88 L (91% of predicted value). The chest radiograph revealed nonhomogeneous opacity at the lower half of the right upper lobe (Figure 1A
). Computed tomography of the chest showed a nodular lesion of 35 mm in diameter in the posterior segment of the right upper lobe, surrounded by thickened, irregular lung interstitium. The lesion had a regular contour showing areas of low density within the mass (Figures 1B and 1C). Bronchoscopy and brush biopsy did not contribute to the diagnosis. A metastatic survey, including computed tomographic scan of the head and abdomen and total body bone scan revealed no evidence of metastatic disease.
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Figure 1. (A) Chest radiograph showing nonhomogeneous opacity at the lower half of the right upper lobe. (B, C) Computed tomographic scans of the chest revealing a nodular lesion in the posterior segment of that lobe.
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The patient underwent exploratory thoracotomy. An enlarged hilar lymph node was sent for frozen section analysis, and the result was consistent with neoplasm. Right upper lobectomy was performed. Frozen sections of the bronchial stump did not show any tumor. Routine mediastinal lymph node dissection was performed. The patient recovered well from the operation.
Histology of the excised lung tissue revealed a vascular tumor composed of epithelioid cells with vesicular nuclei and prominent nucleoli that lined irregular vascular spaces as well as forming solid areas, and showing pleomorphism with rare mitotic activity (Figure 2A). There was extensive intra-alveolar hemorrhage. No squamous differentiation was seen. On immunohistochemical testing using a standard avidin-biotin technique, the tumor cells stained positively for the cytokeratin marker CAM 5.2 and the endothelial markers factor VIII, CD31, and CD34 (Figures 2B and 2C). A diagnosis of epithelioid angiosarcoma was made.
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Figure 2. (A) Tumor section showing epithelioid cells (hematoxylin and eosin stain, original magnification x 100). (B, C) Malignant epithelioid tumor cells (arrow pointing to one cell in C) stained positively for CD31 (original magnification x 100 in B, x 400 in C).
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As a result of positive signs of bronchial margin involvement as well as invasion of the visceral pleura and a hilar lymph node, the patient was offered re-operation. On his refusal, he was referred for chemotherapy and radiation therapy following recovery from the operation. He subsequently refused adjuvant therapy and died 5 months later.
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DISCUSSION
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Angiosarcoma constitutes less than 1% of all sarcomas.2 The sites most frequently involved are the skin and subcutaneous tissue, liver, breast, and heart.2,3 Angiosarcoma occurring in the lung usually represents metastasis from the heart, the pulmonary arterial trunk, or an extrathoracic organ.3 Angiosarcoma of pulmonary origin is very rare, being reported only in a handful of cases.1,2,4–7
A case of angiosarcoma with an epithelioid morphology – or epithelioid angiosarcoma – has been reported that arose in the lung as a bilateral multinodular infiltrate and presented with pulmonary hemorrhage.4 The patient received steroid therapy for idiopathic pulmonary hemorrhage. He developed respiratory failure and required ventilation. He subsequently underwent open lung biopsy and began palliative therapy for probable adenocarcinoma but died 2 days later. A diagnosis of epithelioid angiosarcoma was made by immunohistochemistry after autopsy examination. As far as we know, our patient is the 2nd reported case of epithelioid angiosarcoma of the lung.
Predisposing factors for angiosarcomas include Thorotrast, polyvinyl chloride, and phenylethylhydrazine for liver tumors, postmastectomy and postirradiation states for skin and chest wall lesions, and chronic empyema for pleural space angiosarcomas.6 Our patient had been exposed to asbestos.
Primary pulmonary angiosarcomas and metastatic angiosarcomas in the lung have similar symptomatology and radiologic features.3,4 Primary tumors may occur either as multifocal lesions or as a solitary nodule. The few reported cases of angiosarcoma involving the lung presented with intractable hemoptysis or massive intraparenchymal or intrapleural hemorrhage.4,5 Patients usually present with chest pain, hemoptysis, dyspnea, cough, and malaise.7 When alveolar hemorrhage is encountered, angiosarcoma, either primary or metastatic to the lung, should be considered.
Our patient’s presentation was typical of angiosarcoma, with diffuse alveolar hemorrhage and a nodular lesion in the right upper lobe. The solitary form of primary pulmonary angiosarcoma has been described in a few patients. The size of the lesion may vary from a small nodule to a large mass invading the mediastinum or the chest wall.2,7
Differential diagnosis between pulmonary angiosarcomas and other masses, especially lung carcinoma and a variety of vascular lesions with epithelioid endothelial cells, is not possible without biopsy. The distinction between benign and malignant vascular lesions can be quite challenging, even on histopathological studies, and requires immunohistochemical analysis in most patients. Among the various immunohistochemical markers used for classification, factor VIII-related antigen and CD31 are considered specific for tumors derived from the endothelium.1,4
In the few reported cases of pulmonary angiosarcoma, the prognosis was poor with all patients dead within months of initial presentation.4 Therapeutic modalities such as radiation therapy, chemotherapy, and surgical intervention have all been attempted, but none of them have been shown to be dramatically effective.3 Due to the rarity of pulmonary angiosarcoma, an optimal therapeutic strategy is not available. However, there is a tendency for surgical intervention in all reported cases.3 All long-term survivors of angiosarcoma have undergone either complete resection or multidisciplinary therapy.8
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ABSTRACT
INTRODUCTION
