Pleural angiosarcoma presenting with spontaneous hemothorax

Abstract

Angiosarcomas (ASs) are very rare and constitute 1-2% of soft tissue malignancies. Primary pleural AS (PPAS) is a very rare neoplasm, with only 50 cases reported in the literatüre, and is a tumor with a high tendency for local recurrence and metastasis, with an aggressive course and a generally poor prognosis unless diagnosed early. It originates from the endothelial cells of small blood vessels and therefore can affect many organs. The etiology and definitive method in the treatment is still unclear. Patients usually present with nonspecific symptoms such as cough, dyspnea, chest pain, and hemoptysis. Recurrent exudative or hemorrhagic pleural effusion may develop due to its pleural location. The diagnosis can be made by histopathological and immunohistochemical examinations of excisional biopsy specimens. The effectiveness of chemotherapy and radiotherapy is weak and can be applied for palliative purposes. Surgical approach can be used for diagnostic and palliative purposes. Due to the high degree of malignancy and insidious course of PPAS, patients usually die within months after diagnosis. In these patients, surgical exploration is important for the diagnosis and palliative/definitive treatment of the disease. We present a 61-year-old male patient who presented with dyspnea, chest pain, and massive pleural effusion findings in the left hemithorax and was diagnosed with PPAS as a result of pleural biopsy.

Keywords: Pleural nodules; Primary pleural angiosarcoma; Spontaneous hemothorax.

Fig. 1

Radiopacity area that starts in the middle zone of the right hemithorax on the chest X-ray and progresses rapidly in the follow-up (the time between two chest X-rays is 11 days)

Fig. 2

A, B Tumor characterized by solid islands formed by atypical cells with epithelioid appearance on a hemorrhagic background. C Membrane positivity with CD31 in immunohistochemical examination. D Nuclear positivity with ERG