Note: Some citations in the text of this section are followed by a level of evidence. The PDQ Editorial Boards use a formal ranking system to help the reader judge the strength of evidence linked to the reported results of a therapeutic strategy. (Refer to the PDQ summary on Levels of Evidence for more information.)
There is no consensus as to the optimal treatment of newly diagnosed craniopharyngioma. Little data exist to compare the different modalities in terms of recurrence rate or quality of...
Added text to state that ATRX is involved in epigenetic gene silencing and telomere length; ATRX mutation without MYCN amplification is associated with age at diagnosis in adolescents and young adults with metastatic neuroblastoma (cited Cheung et al. as reference 48).
Added Schleiermacher et al. as reference 56.
Revised text to state that before resection of the primary tumor, bone should be assessed by metaiodobenzylguanidine (MIBG) scan, which is applicable to all sites of disease, and by technetium 99 scan if the results of the MIBG scan are negative or unavailable. Also added text to state that additional imaging of isolated or equivocal positive lesions is recommended if the primary tumor does not take up MIBG (cited Taggart et al. as reference 5).
Revised text for stage 4S to state that it is considered localized primary tumor, as defined for stage 1, 2A, or 2B, with dissemination limited to skin, liver, and/or bone marrow (limited to infants younger than 18 months).
Treatment Option Overview
Added text to state that the Children's Oncology Group (COG) study COG-P9641 demonstrated excellent survival in patients with asymptomatic, low-risk, stage 2A or 2B disease with favorable histology (cited Strother et al. as reference 3).
Revised text to state that for children with recurrent or refractory neuroblastoma, 131 I-MIBG is an effective palliative agent and should be considered (cited Johnson et al. as reference 19 and level of evidence 3iiiA).
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