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Childhood Central Nervous System Embryonal Tumors (PDQ®)
Health Professional VersionEn españolLast Modified: 04/03/2008



Purpose of This PDQ Summary






General Information






Cellular Classification of Central Nervous System Embryonal Tumors






Staging of Medulloblastoma






Treatment Option Overview






Treatment for Newly Diagnosed Childhood Medulloblastoma






Staging of Pineoblastoma






Treatment Options for Newly Diagnosed Pineoblastoma and Pineal Parenchymal Tumors of Intermediate Differentiation






Staging of Supratentorial Primitive Neuroectodermal Tumors






Treatment Options for Newly Diagnosed Supratentorial Primitive Neuroectodermal Tumors







Medulloepithelioma and Ependymoblastoma






Recurrent Childhood Central Nervous System Embryonal Tumors






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Changes to This Summary (04/03/2008)






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Medulloepithelioma and Ependymoblastoma

Although medulloepithelioma and ependymoblastoma identified as histologically discrete tumors within the World Health Organization classification system, there are few data on which to base treatment for these embryonal tumors.[1] Both tumors are rare and tend to arise most commonly in infants and young children. Dissemination may occur, and the tumors are staged in the same way as medulloblastoma. Treatment considerations are usually the same as those for children with poor-risk medulloblastoma and for children younger than 3 years at diagnosis with other embryonal tumors. Prognosis is poor, with 5-year survival rates ranging between 0% and 30%.[2,3]

References

  1. Kleihues P, Cavenee WK, eds.: Pathology and Genetics of Tumours of the Nervous System. Lyon, France: International Agency for Research on Cancer, 2000. 

  2. 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]

  3. Mason WP, Grovas A, Halpern S, et al.: Intensive chemotherapy and bone marrow rescue for young children with newly diagnosed malignant brain tumors. J Clin Oncol 16 (1): 210-21, 1998.  [PUBMED Abstract]

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