Chou Chun-Ying, Hu Hsiang-Wei, Chen Tom Wei-Wu, Lin Shu-Yung
Department of Internal Medicine National Taiwan University Hospital Taipei Taiwan.
Department of Pathology National Taiwan University Hospital Taipei Taiwan.
Respirol Case Rep. 2024 Mar 28;12(4):e01341. doi: 10.1002/rcr2.1341. eCollection 2024 Apr.
Epithelioid 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.
上皮样血管内皮瘤（EHE）是一种罕见的血管肿瘤，其临床行为介于良性血管瘤和高级别血管肉瘤之间。胸膜EHE极为罕见，其预后严峻，大多数患者生存期不足1年。在此，我们报告一例45岁女性胸膜EHE病例，该患者有长达1个月的右侧胸膜炎性胸痛病史。胸部计算机断层扫描显示右肺实变、肺不张、右侧胸膜增厚和胸腔积液。她接受了电视辅助胸腔镜手术进行胸膜剥脱术，最终被确诊为胸膜EHE，显示有CAMTA1重排。紫杉醇治疗在28天周期的第1、8和15天每周给药一次，12个周期后病情稳定。管理胸膜EHE患者具有挑战性，因为目前仍没有既定的标准治疗方法。我们的病例在紫杉醇治疗后实现了11个月的无进展生存期。
