Children Older Than 3 Years
        Standard treatment options
Children Younger Than 3 Years

Children Older Than 3 Years

Standard treatment options

Attempting aggressive surgical resection is the first step in the management of newly diagnosed supratentorial primitive neuroectodermal tumors (SPNET), although studies have yet to demonstrate that the extent of resection is predictive of outcome.[1-3] After surgery, children with SPNET usually receive treatment similar to that received by children with poor-risk medulloblastoma. Best results have been obtained after radiation to the entire neuroaxis with local boost radiation therapy, as given for medulloblastoma.[3] However, the local boost radiation therapy may be problematic because of the size of the tumor and its location in the cerebral cortex. The chemotherapeutic approaches during and after radiation therapy are similar to those used for children with poor-risk medulloblastoma. Overall, 3- to 5-year survival rates of 40% to 50% have been noted.[1-3] Patients with disseminated disease at the time of diagnosis have overall poorer survival, with reported survival rates at 5 years ranging from 20% to 30%.[1-3]

Treatment of children younger than 3 years with supratentorial primitive neuroectodermal tumors is similar to that outlined for children with medulloblastoma. With the use of chemotherapy alone, outcome has been variable, with survival rates at 5 years ranging between 0% and 40%.[4-6]

References

1. Cohen BH, Zeltzer PM, Boyett JM, et al.: Prognostic factors and treatment results for supratentorial primitive neuroectodermal tumors in children using radiation and chemotherapy: a Childrens Cancer Group randomized trial. J Clin Oncol 13 (7): 1687-96, 1995.  [PUBMED Abstract]
   
   
2. Reddy AT, Janss AJ, Phillips PC, et al.: Outcome for children with supratentorial primitive neuroectodermal tumors treated with surgery, radiation, and chemotherapy. Cancer 88 (9): 2189-93, 2000.  [PUBMED Abstract]
   
   
3. Timmermann B, Kortmann RD, Kühl J, et al.: Role of radiotherapy in the treatment of supratentorial primitive neuroectodermal tumors in childhood: results of the prospective German brain tumor trials HIT 88/89 and 91. J Clin Oncol 20 (3): 842-9, 2002.  [PUBMED Abstract]
   
   
4. Geyer JR, Sposto R, Jennings M, et al.: Multiagent chemotherapy and deferred radiotherapy in infants with malignant brain tumors: a report from the Children's Cancer Group. J Clin Oncol 23 (30): 7621-31, 2005.  [PUBMED Abstract]
   
   
5. Marec-Berard P, Jouvet A, Thiesse P, et al.: Supratentorial embryonal tumors in children under 5 years of age: an SFOP study of treatment with postoperative chemotherapy alone. Med Pediatr Oncol 38 (2): 83-90, 2002.  [PUBMED Abstract]
   
   
6. Grill J, Sainte-Rose C, Jouvet A, et al.: Treatment of medulloblastoma with postoperative chemotherapy alone: an SFOP prospective trial in young children. Lancet Oncol 6 (8): 573-80, 2005.  [PUBMED Abstract]
   
   

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