Childhood Central Nervous System Embryonal Tumors Treatment (PDQ®)–Health Professional Version

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Changes to This Summary (08/17/2018)

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.

General Information About Central Nervous System (CNS) Embryonal Tumors

Added text to state that a large study of over 1,000 patients demonstrated germline mutations in approximately 5% of all patients diagnosed with medulloblastoma. Germline mutations were identified in APC, BRCA2, PALB2, PTCH1, SUFU, and TP53 (cited Waszak et al. as reference 20).

Revised text to state that Turcot syndrome is exclusive to the WNT-activated subtype of medulloblastoma. Also added text to state that in one analysis, all germline TP53 mutations were restricted to the sonic hedgehog (SHH)–activated subtype of medulloblastoma.

The Consideration of genetic testing subsection was renamed from Additional diagnostic studies for patients with desmoplastic medulloblastoma and extensively revised.

Added Sabel et al. as reference 72.

Cellular and Molecular Classification of CNS Embryonal Tumors

Added text to state that a report that used DNA methylation arrays identified two subtypes of SHH medulloblastoma in young children. One of the subtypes contained all of the cases with SMO mutations, and it was associated with a favorable prognosis. The other subtype had most of the SUFU mutations, and it was associated with a much lower progression-free survival rate. PTCH1 mutations were present in both subtypes (cited Robinson et al. as reference 31).

Treatment of Newly Diagnosed Childhood Medulloblastoma

Added Sabel et al. as reference 28.

Treatment of Recurrent Childhood CNS Embryonal Tumors

Added Sabel et al. as reference 3.

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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