Chamadoira Clara, Pereira Paulo, Silva Pedro S, Castro Ligia, Vaz Rui
Servicio de Neurocirugía, Hospital São João, Porto. Portugal.
Servicio de Neurocirugía, Hospital São João, Porto. Portugal.
Neurocirugia (Astur). 2014 Jul-Aug;25(4):179-82. doi: 10.1016/j.neucir.2014.01.001. Epub 2014 Mar 24.
Epithelioid sarcomas are rare mesenchymal neoplasms mainly arising in the limbs of young adults. We report the case of a 24-year-old male presenting low back pain radiating to both lower limbs, constipation and urinary retention. The MRI scan showed an intraspinal lesion extending from L4 to S2. Surgery resulted in gross total removal of the extradural lesion and partial removal of the intradural component. The immunohistological study of the lesion was consistent with an epithelioid sarcoma. The patient was submitted to radiotherapy and chemotherapy, but a local recurrence of the lesion and dissemination along the neuraxis were observed 3 months after surgery. Despite treatment, the patient died 4 months after the surgical procedure due to multiorgan failure. Despite there being isolated reports of epithelioid sarcomas appearing in the spine, this is, to our knowledge, the first case with intradural extension.
上皮样肉瘤是一种罕见的间叶组织肿瘤，主要发生于年轻成年人的四肢。我们报告一例24岁男性病例，该患者表现为下背部疼痛并放射至双下肢，伴有便秘和尿潴留。磁共振成像（MRI）扫描显示一个从L4延伸至S2的椎管内病变。手术实现了硬膜外病变的大体全切及硬膜内成分的部分切除。病变的免疫组织学研究结果符合上皮样肉瘤。患者接受了放疗和化疗，但术后3个月观察到病变局部复发并沿神经轴扩散。尽管接受了治疗，患者在手术操作后4个月因多器官功能衰竭死亡。尽管有孤立的报道称上皮样肉瘤出现在脊柱，但据我们所知，这是首例有硬膜内延伸的病例。
