Primary posterior mediastinal epithelioid angiosarcoma presenting as epigastric pain: long-term survival achieved via multimodal salvage therapy after early recurrence.

We report a rare case of primary posterior mediastinal epithelioid angiosarcoma (EA) in a 68-year-old male who presented with atypical upper abdominal pain. Contrast-enhanced chest computed tomography revealed a left posterior mediastinal mass, and histopathology with immunohistochemistry (CD31+, CD34+, FLI-1+) confirmed the diagnosis. Since preoperative imaging suggested a benign tumor, the patient underwent macroscopic complete resection via video-assisted thoracoscopic surgery (VATS). Histopathology with immunohistochemistry (CD31+, CD34+, FLI-1+, Ki-67 ~ 25%) unexpectedly confirmed the diagnosis of EA. However, within one month, he developed symptomatic hemorrhagic pleural effusion, and repeat exploration confirmed local recurrence with pleural metastasis. Given the aggressive nature of EA and early relapse, a multimodal salvage regimen was initiated, consisting of systemic paclitaxel (80 mg/m² on days 1, 8, 15 every 4 weeks, six cycles) and intrapleural cisplatin instillation (40 mg weekly for two doses). Remarkable clinical response was achieved, with resolution of pleural effusion and sustained disease control. The patient has remained alive with good quality of life for over 20 months post-recurrence. This case highlights that even in rapidly recurrent mediastinal EA, intensive multimodal therapy can achieve long-term survival. Our experience supports the integration of systemic chemotherapy with locoregional control strategies in managing aggressive sarcomas.