Neves Nuno, Lima-Rodrigues Francisco, Ribeiro-Silva Manuel, Cacho-Rodrigues Pedro, Eloy Catarina, Paiva Maria Emília, Pinto Rui
Spine Group, Orthopedic Department, Hospital de São João, Porto Medical School, Porto.
Acta Reumatol Port. 2010 Jul-Sep;35(3):370-4.
Epithelioid hemagioendothelioma (EH) is a rare vascular tumor with an intermediate biological behavior between hemangioma and angiosarcoma. Vertebral location is even more rare, and because the number of reported cases of EH is small and the follow-up periods short, the best surgical treatment, the role of radiotherapy and chemotherapy, as well as the definitive prognosis are still not established. The authors report a case of EH which presented as a vertebral fracture with neurological impairment, where a percutaneous biopsy was inconclusive. Treatment included vertebrectomy, with complete excision of the lesion, spinal canal decompression and vertebral stabilization. Anatomopathological study revealed an epithelioid vascular neoplasm with low mitotic index, and tumor cells reactive to vimentin, CD31 and CD34, leading to the diagnosis of Grade I Epithelioid Hemangioendothelioma. Because of the wide resection achieved and the low aggressiveness of the lesion, no adjuvant radio or chemotherapy was undertaken, and at 6 years follow-up there are no signs of recurrence or metastasis.
上皮样血管内皮瘤（EH）是一种罕见的血管肿瘤，其生物学行为介于血管瘤和血管肉瘤之间。发生于椎体的情况更为罕见，由于报道的EH病例数量少且随访期短，最佳手术治疗方法、放疗和化疗的作用以及确切预后仍未确定。作者报告了1例表现为伴有神经功能障碍的椎体骨折的EH病例，经皮活检结果不明确。治疗包括椎体切除术，完整切除病变、椎管减压和椎体稳定术。解剖病理学研究显示为一种有丝分裂指数低的上皮样血管肿瘤，肿瘤细胞对波形蛋白、CD31和CD34呈反应性，从而诊断为I级上皮样血管内皮瘤。由于实现了广泛切除且病变侵袭性低，未进行辅助放疗或化疗，随访6年无复发或转移迹象。
