Hospital: Hospital de Valme.
Nº: C2019-403
Aut@r o Autores: S. Rico, B. Fernández, A. Gil, A. Bello.
Presentación
We present the case of a 21-year-old man, without history of interest, who came several times to the emergency department of our hospital in the last 3 months for a low back pain with intermittent irradiation to the left leg. In the last one he also presented fever, headhache and vomiting. With yhe susppicion of acute meningitis and with a normal CT scan, lumbar puncture was performed, which was hematic twice. Thoraco-lumbar MRI was performed and compatible image with a tumor in conus medullary and subarachnoid hemorrhage were visualized. The lesion was excised with the anatomopathological diagnosis of undifferentiated intramedullary tumor, type Ewing sarcoma.
Discusión
Intramedullary Ewing´s sarcoma is a soft tissue tumor that derive from the neural crest (neuroectodermal differentiation) and they are extremely rare and staged differently, like soft tissue sarcomas. It affects mostly males under age 20. To make the definitive diagnosis, positive inmunohistochemistry studies are necessary for vimentin and CD99.9. Like another intradural tumors the acute presentation is rare. The most common presenting symptoms are low back and occasionally present as a subarachnois haemorrhage. Vascular proliferation could be a complementary explanation for the hemorrrhage. Extraosseous intramedullar Ewing usually shows like a mass well circumscribed with hypoisointense signal on T1-weighted images and hyperintensity on T2-weighted sequences, with a clear and homogeneous enhancement after administration of gadolinium They may exhibit leptomeningeal spread and have a poor prognosis. Although myxopapillary ependymoms are more frequent, have worse behavior in young people and may present with hemorrhages, the possibility, although remote of poorly differentiated tumors, requires at least not to delay surgery.
Conclusión
Intramedullary spinal tumors represent only the 4-10% of all central nervous systems tumors and of these the majority are glial neoplasms. Nonetheless, the presence of an intramedullary tumor in a young patient who debuts with acute symptoms due to subarachnoid bleeding makes it necessary to think about a more aggressive entity and realization of an early diagnosis.
Bibliografía
- Pancucci G, Simal-Julian JA, Plaza-Ramirez E, García-Marcos R, Mayordomo-Aranda E, Botella-Asunción C. Primary extraosseous intradural spinal Ewing’s sarcoma: report of two cases. Acta Neurochir 2013, 155:1229–1234 . - Yong Yan, Tao Xu, Ju