The prognosis for<unk>  has been very poor, although some indications exist that an IRSIII-based therapy can produce long-term survival (60 to 72 months). Two-year survival is less than 20%, average survival postoperatively is 11 months, and doctors often recommend palliative care, especially with younger children because of the poor outcomes. Recently, a protocol used by a multicenter trial reported in the Journal of Clinical Oncology resulted in a 70% survival rate at 2–3 years, with most relapses occurring within months, leading to hope that a point exists beyond which patients can be considered cured. Patients with metastasis (disseminated tumor), larger tumors, tumors that could not be fully removed, or tumor recurrence, and who were younger than 36 months had the worst outcomes (i.e., shorter survival times). A retrospective survey from 36<unk>  cases at St. Jude Children's Hospital from 1984 to 2003 showed that the two-year event-free survival (EFS) for children under three was 11%, and the overall survival (OS) rate was 17%. For children aged 3 years or older, the EFS was 78% and the OS 89%. A retrospective register at the Cleveland Children's hospital on 42<unk>  patients found median survival time is 16.25 months and a survival rate around 33%. One-quarter of these cases did not show the mutation in the INI1/hSNF5 gene. The longest-term survivals reported in the literature are: * (a) Hilden and associates reported a child who was still free from disease at 46 months from diagnosis. * (b) Olson and associates reported a child who was disease free at five years from diagnosis based on the IRS III protocol. * (c) In 2003, Hirth reported a patient who had been disease-free over six years. * (d) Zimmerman in 2005 reported 50-to-72 month survival rates on four patients using an IRS III-based protocol. Two of these long-term survivors had been treated after an<unk>  recurrence. * (e) A NYU study (Gardner 2004) has four of 12 longer-term<unk>  survivors; the oldest was alive at 46 months after diagnosis. * (f) Medical University of Vienna, 2013, reported a 16-year survivor, among other long-term survivors Cancer treatments in long-term survivors who are children usually cause a series of negative effects on physical well being, fertility, cognition, and learning. Metastasis Metastatic spread is noted in roughly one-third of the<unk>  cases at the time of diagnosis, and tumors can occur anywhere throughout the CNS. The ASCO study of the 188 documented<unk>  cases prior to 2004 found 30% of the cases had metastasis at diagnosis. Metastatic spread to the meninges (leptomenigeal spread sometimes referred to as sugar coating) is common both initially and with relapse. Average survival times decline with the presence of metastasis. Primary CNS tumors generally metastasize only within the CNS. One case of metastatic disease to the abdomen via ventriculoperitoneal shunt has been reported with<unk>  . Metastatic dissemination via this mechanism has been reported with other brain tumors, including germinomas, medulloblastomas, astrocytomas, glioblastomas, ependymomas, and endodermal sinus tumors. Guler and Sugita separately reported cases of lung metastasis without a shunt.
Atypical teratoid rhabdoid tumor
Malignant rhabdoid tumour
Pediatric ependymoma
Neurothekeoma
Endodermal sinus tumor
Sialoblastoma
Ameloblastoma
Primitive neuroectodermal tumor