Henriquez Clarissa Rodriguez, Cazes Aurélie, Fabiani Jean-Noël, Bruneval Patrick
Service d'anatomie et cytologie pathologiques, hôpital européen Georges-Pompidou, 20, rue Leblanc, 75908 Paris cedex 15, France.
Ann Pathol. 2011 Jun;31(3):218-21. doi: 10.1016/j.annpat.2011.02.015. Epub 2011 May 18.
Epithelioid hemangioendothelioma is a rare endothelial cell-derived tumor of intermediate grade malignancy. It has an unpredictable outcome, independently of the usual histoprognostic criteria. Thirty-three cases with intravascular localisation are described in the literature. We describe a case occurring in a 31-year-old male, which developed an inferior veina cava tumor, with recurrence after incomplete surgical resection and chemotherapy. He was operated on again under extracorporeal circulation and complete surgical resection was then performed. Histologic examination found an intravascular epithelioid hemangioendothelioma developing in the vascular lumen without vascular wall infiltration. This entity represents a challenge not only for the vascular surgeon, but also for the pathologist given its difficult diagnosis.
上皮样血管内皮瘤是一种罕见的源自内皮细胞的中度恶性肿瘤。其预后不可预测，与通常的组织学预后标准无关。文献中描述了33例血管内定位的病例。我们报告一例发生在一名31岁男性的病例，该患者出现下腔静脉肿瘤，在手术切除不完全及化疗后复发。随后他在体外循环下再次接受手术，并进行了完整的手术切除。组织学检查发现血管腔内发生血管内上皮样血管内皮瘤，无血管壁浸润。鉴于其诊断困难，该实体不仅对血管外科医生，而且对病理学家都是一个挑战。
