Abstract
Primary pleural angiosarcoma is a rare and clinically aggressive tumor. Patients usually present with chest pain, dyspnea, hemoptysis and/or cough. Radiologic studies reveal diffuse pleural thickening and pleural effusion with or without mass lesion. The clinical and radiological features both resemble those of mesothelioma, and its definite diagnosis requires careful histologic examination. However, frequent epithelioid feature and immunoreactivity to cytokeratin in primary pleural angiosarcoma further complicate the pathologic diagnosis. The use of proper immunohistochemical stains is often needed to support endothelial differentiation in the tumor cells and to exclude metastatic carcinoma and mesothelioma. We report the case of a 49-year-old male patient with primary pleural angiosarcoma, who presented with initial hemothorax, followed by a rapid progress to an inoperable status.
Highlights
Angiosarcoma is an uncommon malignant tumor of endothelial differentiation
We describe a case of Primary pleural angiosarcoma (PPA), to highlight its aggressive clinical behavior and the diagnostic pitfalls
In the context of refractory bloody pleural effusion of uncertain etiology, we suggest that surgical biopsy or excision should be considered even without mass lesion
Summary
Angiosarcoma is an uncommon malignant tumor of endothelial differentiation. It accounts for about 1% of all soft tissue malignancies and most commonly arises in skin, soft tissue, breast, liver, bone and spleen [1]. He had a 10-year history of asthma under regular medical treatment and was an exsmoker (half package-per-day for 20 years in the past) who quit 10 years ago. Thoracoscopic examination revealed diffuse blood clot coating and thickening of the visceral and parietal pleura over the right hemithorax. He received thoracoscopic decortication of the pleura. The patient received radiotherapy (3750 cGy with 15 fractions), five cycles of chemotherapy with mesna, ifosfamide, doxorubicin and dacarbazine, two cycles of cisplatin and doxorubicin and three cycles of doxorubicin, etoposide and thalidomide He was alive with the disease nine months after the diagnosis was made
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