Tumor atípico teratoide rabdoide

Abstract

The atypical teratoid rhabdoid tumor (TA/RT), is a malignant embryonal lession that corresponds to the 1-3% of the central nervous system tumors in children younger than 18 years, but up to 20% of malignant tumors of the CNS in patients younger than 3 years of age, generally are infratentorial, whose prognosis is bad in the short term, with a mortality rate of 70% per year and it increases in patients with metastatic disease at the time of diagnosis, tumor recurrence is 80% per year. The clinical presentation, is highly variable, since it depends to the site where the lesion is located, although in pediatric patients the lesion is on infratentorial region, so generated alternate syndromes, symptoms of injury of cranial nerves, cerebellar symptoms and 33% of these patients have hydrocephalus. On histopathologic examination, cells are seen with a rhabdoid tumor and mesenquima component, in addition to small cells. The immunohistochemistry is essential in these lesions. We have carried out many studies in the last two decades in an attempt to increase the survival in these patients, without much success for the moment, however it has been observed that the most important component in the treatment is radiation therapy since the beginning of this early during the first month of postoperative increases the survival rate, back to this is mandatory start handling of chemotherapy.