Valera-Melé Marc, Darriba Allés Juan Vicente, Ruiz Juretschke Fernando, Sola Vendrell Emma, Hernández Poveda José Manuel, Montalvo Afonso Antonio, Casitas Hernando Vicente, García Leal Roberto
Department of Neurosurgery, Hospital General Universitario Gregorio Marañón, Calle Doctor Esquerdo 46, 28007 Madrid, Spain.
Department of Neurosurgery, Hospital General Universitario Gregorio Marañón, Calle Doctor Esquerdo 46, 28007 Madrid, Spain.
Neurocirugia (Engl Ed). 2021 Mar 22. doi: 10.1016/j.neucir.2021.02.002.
Angiosarcoma is an infrequent tumor among sarcomas, especially presenting as a primary tumor within the central nervous system, which can lead to a rapid neurological deterioration and death in few months. We present a 41-year old man with a right frontal enhancing hemorrhagic lesion. Surgery was performed with histopathological findings suggesting a primary central nervous system angiosarcoma. He was discharged uneventfully and received adjuvant chemotherapy and radiotherapy. At 5 months, the follow-up MRI showed two lesions with an acute subdural hematoma, suggesting a relapse. Surgery was again conducted finding tumoral membranes attached to the internal layer of the duramater around the right hemisphere. The patient died a few days later due to the recurrence of the subdural hematoma. This case report illustrates a rare and lethal complication of an unusual tumor. The literature reviewed shows that gross-total resection with adjuvant radiotherapy seems to be the best treatment of choice.
血管肉瘤是肉瘤中一种罕见的肿瘤,尤其是作为原发性肿瘤出现在中枢神经系统内,这可能导致在几个月内迅速出现神经功能恶化并死亡。我们报告一名41岁男性,其右侧额叶有一个强化的出血性病变。进行了手术,组织病理学检查结果提示为原发性中枢神经系统血管肉瘤。他顺利出院,并接受了辅助化疗和放疗。5个月时,随访磁共振成像(MRI)显示有两个病变伴有急性硬膜下血肿,提示复发。再次进行手术,发现肿瘤包膜附着于右半球周围硬脑膜的内层。患者几天后因硬膜下血肿复发死亡。本病例报告说明了一种罕见肿瘤的致命并发症。回顾的文献表明,根治性手术切除联合辅助放疗似乎是最佳的治疗选择。