Bilateral Spontaneous Hemothorax: A Rare Case of Primary Pleural Angiosarcoma and Literature Review

Introduction and case report: Angiosarcomas, rare soft tissue malignancies originating from endothelial cells, represent only 1–2% of all soft tissue sarcomas. Primary pleural angiosarcoma (PPA) is exceptionally rare, with only 43 reported cases since 1943. There are many diagnostic and therapeutic challenges due to the rarity of these tumors. We present the case of a 72-year-old man presenting with back pain, dyspnea and anemia. Conventional imaging revealed bilateral pleural effusion and a thickened parietal pleura, while contrast chest MR was able to identify pleural sites of contrast enhancement. Left chest tube placement evidenced a hemothorax, and the cytology result was negative. A thoracoscopic approach was chosen, allowing us to perform different parietal pleural biopsies. Radiological and pathological features led to the diagnosis of epithelioid PPA. Despite pleural drainage and blood transfusions, the patient died only 4 days after diagnosis. Objectives: To present a literature review, evaluating the disease epidemiology and the clinical, diagnostic and therapeutic features of PPA. Methods: We reviewed cases of PPA in the literature (1954–2024) by searching the PubMed database for the terms “pleural angiosarcoma” and “pleura + angiosarcoma”. Results: We found a total of 47 cases that were described between 1987 and 2024 with sufficient data to be included in our review. PPA was found to be a challenging diagnosis, found mostly in older Caucasian males. The cytology is mostly indeterminant, and an endoscopic approach is usually needed. Radical surgery is the most common treatment option, and chemotherapy and radiation therapy are also often used. However, the prognosis is poor. Conclusions: PPA is very rare, and complex cases such as this one showcase the importance of innovative approaches like MRI and emphasize the significance of multidisciplinary collaboration for optimal patient management. Bilateral spontaneous hemothorax, as seen in this case, is uncommon and poses additional challenges in disease management. Further research to advance the diagnostic capabilities and treatment efficacy is needed.