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Staging of CNS Embryonal Tumors


    Staging of Pineoblastoma

    Staging for children with pineoblastomas is the same as that performed for children with medulloblastoma.[4] Dissemination at the time of diagnosis occurs in 10% to 30% of patients.[4] Because of the location of the tumor, total resections are uncommon, and most patients have only a biopsy or a subtotal resection before postsurgical treatment.[4,5] Similar to other central nervous system (CNS) primitive neuroectodermal tumors (PNETs), all pineoblastomas are treated as high-risk embryonal tumors. Prognosis is worse for patients with disseminated disease at the time of diagnosis.[4,5]

    Staging of CNS PNETs

    Patients with CNS PNETs are staged in a fashion similar to that used for children with medulloblastoma. CNS PNETs may be disseminated at the time of diagnosis, although the incidence of dissemination may be somewhat less than that of medulloblastomas or pineoblastomas, with dissemination at diagnosis being documented in approximately 10% to 20% of patients.[6,7] CNS PNETs are often amenable to resection; in series, 50% to 60% of patients were totally or near-totally resected.[6,7]

    Staging of Other CNS Embryonal Tumors

    Dissemination of both medulloepitheliomas and ependymoblastomas may occur, and the tumors are staged in the same way as medulloblastoma.


    1. Fouladi M, Gajjar A, Boyett JM, et al.: Comparison of CSF cytology and spinal magnetic resonance imaging in the detection of leptomeningeal disease in pediatric medulloblastoma or primitive neuroectodermal tumor. J Clin Oncol 17 (10): 3234-7, 1999.
    2. Zeltzer PM, Boyett JM, Finlay JL, et al.: Metastasis stage, adjuvant treatment, and residual tumor are prognostic factors for medulloblastoma in children: conclusions from the Children's Cancer Group 921 randomized phase III study. J Clin Oncol 17 (3): 832-45, 1999.
    3. Yao MS, Mehta MP, Boyett JM, et al.: The effect of M-stage on patterns of failure in posterior fossa primitive neuroectodermal tumors treated on CCG-921: a phase III study in a high-risk patient population. Int J Radiat Oncol Biol Phys 38 (3): 469-76, 1997.
    4. Jakacki RI, Zeltzer PM, Boyett JM, et al.: Survival and prognostic factors following radiation and/or chemotherapy for primitive neuroectodermal tumors of the pineal region in infants and children: a report of the Childrens Cancer Group. J Clin Oncol 13 (6): 1377-83, 1995.
    5. 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.
    6. 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.
    7. 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.

    This information is produced and provided by the National Cancer Institute (NCI). The information in this topic may have changed since it was written. For the most current information, contact the National Cancer Institute via the Internet web site at http:// cancer .gov or call 1-800-4-CANCER.

    WebMD Public Information from the National Cancer Institute

    Last Updated: February 25, 2014
    This information is not intended to replace the advice of a doctor. Healthwise disclaims any liability for the decisions you make based on this information.

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