Childhood Craniopharyngioma Treatment (PDQ®)–Health Professional Version

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Changes to This Summary (09/28/2017)

The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

This summary was comprehensively reviewed.

General Information About Childhood Craniopharyngioma

Revised text to state that craniopharyngiomas without calcification may be confused with other tumor types, including germ cell tumors, hypothalamic/chiasmatic astrocytomas, or Langerhans cell histiocytosis. Biopsy or resection is required to confirm the diagnosis.

Newly Diagnosed Childhood Craniopharyngioma Treatment

Added text to state that an understanding of the complex variations in how the tumors grow anatomically may help facilitate gross-total resection (cited Morisako et al. as reference 7).

Revised text to state that newer radiation technologies such as intensity-modulated photon therapy and proton therapy may reduce the radiation dose to uninvolved parts of the brain and spare normal tissue.

Revised text to state that tumor progression remains a concern, and it is usually not possible to repeat a full course of standard fractionated radiation.

Recurrent Childhood Craniopharyngioma Treatment

Added text to state that case reports demonstrating dramatic tumor response to BRAFinhibitors in adults with papillary craniopharyngiomas suggest that there may be a role for BRAF inhibitor therapy in the rare setting of a child with a papillary craniopharyngioma (cited Aylwin et al. and Roque et al. as references 15 and 16, respectively).

Late Effects in Patients Treated for Childhood Craniopharyngioma

Added text to state that in one series of 261 patients diagnosed with craniopharyngiomas before 2000, hypothalamic involvement was associated with lower overall survival, impaired quality-of-life, and severe obesity (cited Sterkenburg et al. as reference 1 and level of evidence 3iA).

Added text to state that children who undergo complete resection or subtotal resection may develop obesity, suggesting that a predilection to obesity may be a component of the disease itself, as opposed to the result of direct hypothalamic injury (cited Tan et al. as reference 10 and level of evidence 3iC).

This summary is written and maintained by the PDQ Pediatric Treatment Editorial Board, which is editorially independent of NCI. The summary reflects an independent review of the literature and does not represent a policy statement of NCI or NIH. More information about summary policies and the role of the PDQ Editorial Boards in maintaining the PDQ summaries can be found on the About This PDQ Summary and PDQ® - NCI's Comprehensive Cancer Database pages.

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