Unusual Cancers of Childhood Treatment (PDQ®)–Health Professional Version

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Changes to This Summary (01/26/2018)

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.

Abdominal Cancers

Added text about the outcome and prognostic factors for patients with adrenocortical carcinoma who do not have a germline TP53 mutation (cited Pinto et al. as reference 40).

The Nonappendiceal Neuroendocrine Tumors subsection was extensively revised.

Added text about the molecular subtypes of gastrointestinal stromal tumors (GIST), including the results of a study that genomically profiled wildtype GIST tumors (cited Boikos et al. as reference 152).

Other Rare Childhood Cancers

Added text to state that a retrospective analysis identified 262 patients with MEN2A syndrome. Median age of the cohort was 42 years and ranged from age 6 to 86 years. There was no correlation between the specific RET mutation identified and the risk of distant metastasis. Younger age at diagnosis did increase the risk of distant metastasis (cited Voss et al. as reference 26).

Revised text to update the melanoma annual incidence rates in the United States from 2010 to 2014 (cited Childhood cancer by the ICCC as reference 83).

Revised text to state that the incidence of pediatric melanoma increased by an average of 1.7% per year between 1975 and 1994, but then decreased by 0.6% per year from 1995 to 2014.

Revised text to state that neurocutaneous melanosis is an unusual condition that arises in the context of congenital melanocytic nevi and is associated with large or multiple congenital nevi of the skin in association with meningeal melanosis or melanoma; approximately 2.5% of patients with large congenital nevi develop this condition, and those with increased numbers of satellite nevi are at greatest risk.

Added text about the prognosis, molecular features, and treatment of central nervous system melanoma (cited Kinsler et al. as reference 92).

The Treatment subsection of the Melanoma section was extensively revised.

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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