A case of primary pleural epithelioid hemangioendothelioma achieving stable disease with paclitaxel treatment: A case report and literature review

Pleural epithelioid hemangioendothelioma (EHE) is exceptionally rare, and the prognosis for pleural EHE is grim, typically resulting in a survival of less than 1 year. In this case report, we present the case of a 45-year-old woman who endured 1 month of right-sided pleuritic chest pain, was diagnosed with right pleural EHE, and achieved an 11-month progression-free survival following paclitaxel treatment. AbstractEpithelioid hemangioendothelioma (EHE) is a rare vascular neoplasm with a clinical behaviour that falls between a benign hemangioma and a high-grade angiosarcoma. Pleural EHE is exceptionally rare, and its prognosis is grim, with most patients experiencing survival of less than 1 year. Here, we present a case of pleural EHE in a 45-year-old woman with a month-long history of right-sided pleuritic chest pain. Chest computed tomography revealed consolidation, atelectasis of the right lung, right pleural thickening, and pleural effusion. She underwent video-assisted thoracoscopic surgery for decortication and was diagnosed with conclusively pleural EHE, showing a CAMTA1 rearrangement. Paclitaxel treatment, administered once weekly on days 1, 8 and 15 of a 28-day  cycle, resulted in a stable disease after 12 cycles. Managing patients with pleural EHE is challenging because there are still no established standard treatments. Our case achieved 11-month progression-free survival following paclitaxel treatment.
Source: Respirology Case Reports - Category: Respiratory Medicine Authors: Tags: CASE REPORT Source Type: research