Primary Pleural Angiosarcoma is a highly aggressive malignancy occurring almost exclusively in older men, with a median survival of less than six months despite aggressive multimodal therapy.
Primary pleural angiosarcoma is a rare, aggressive malignancy with a dismal prognosis, emphasizing the need for early biopsy, accurate immunophenotyping, and multidisciplinary management.
The Primary Pleural Angiosarcoma (PPA) is a highly aggressive and rare endothelial cell malignancy of the pleura. The nonspecific presenting features, coupled with its tendency to mimic mesothelioma, metastatic carcinomas, or benign pleural diseases, make its diagnosis particularly challenging. Delayed diagnosis, often till late in the course of the disease, and poor outcomes are common. The hallmark clinical presentations are haemorrhagic pleural effusion, chest pain, and progressive dyspnoea-all nondiagnostic. The present narrative review aimed to synthesise and critically evaluate the literature on PPA, focusing on diagnostic strategies, histopathological features, therapeutic modalities, and outcome patterns. From a qualitative analysis of published case reports and review articles published between 1988 and 2025, which were chosen for their extensive clinical, pathological, and Immunohistochemical (IHC) documentation pertinent to PPA, it is found that PPA arises almost exclusively in older men, characterised by rapidly progressive respiratory symptoms and a high frequency of haemothorax. Again, imaging studies are nondiagnostic, and cytology is of low sensitivity. The differential diagnosis is established by pleural biopsy, confirmed by IHC analysis with endothelial markers such as CD31, ERG, and FLI-1. Despite aggressive multimodal therapy comprising surgical resection, chemotherapy, and radiotherapy, median survival is woeful, less than six months from presentation in most series. In conclusion, PPA represents a frontier in both diagnosis and therapy within thoracic oncology. Early biopsy, accurate immunophenotyping, and multidisciplinary input are essential in maximising patient outcomes. Due to its rarity and dismal prognosis, more comprehensive and focused molecular studies are urgently needed to enhance diagnostic precision and therapeutic outcomes.
Hatwar et al. (Fri,) conducted a review in Primary Pleural Angiosarcoma. Primary Pleural Angiosarcoma is a highly aggressive malignancy occurring almost exclusively in older men, with a median survival of less than six months despite aggressive multimodal therapy.