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

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Changes to This Summary (03/31/2016)

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.

Head and Neck Cancers

Added text to state that a multi-institutional review of 24 patients younger than 21 years at diagnosis found a 5-year disease-free survival and overall survival of 73% to 74% (cited Venkatramani et al. as reference 38 and level of evidence 3iiiA).

Added text to state that for patients with papillary or follicular carcinoma, total thyroidectomy is the recommended treatment of choice. Central lymph node dissection should be done in the presence of clinical evidence of gross extrathyroidal invasion identified preoperatively or at the time of surgery.

Abdominal Cancers

Added text about a retrospective review of patients younger than 18 years in Germany that identified 31 patients with colorectal carcinoma (cited Weber et al. as reference 95).

Added text to state that overall, the prognosis for patients with appendiceal carcinoids is excellent.

Added Nonappendiceal neuroendocrine tumors as a new subsection.

Genital/Urinary Tumors

Added text to state that it is prudent to check alpha-fetoprotein (AFP) levels before surgery. Elevated AFP levels are usually indicative of a malignant germ cell tumor. However, AFP levels and decay in levels are often difficult to interpret in infants younger than 1 year (cited Blohm et al. as reference 22).

Other Rare Childhood Cancers

Added text to state that a series of 160 patients with MEN1 syndrome was the first large study of patients diagnosed before age 21 years. Four patients had a malignant tumor, and one of these patients died (cited Goudet et al. as reference 5).

Added text about how the most-recent literature suggests that familial medullary thyroid carcinoma should be recognized as a variant of MEN2A (cited Wells et al. as reference 18).

Added Stanelle et al. as reference 108.

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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