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

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Changes to This Summary (04/09/2018)

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.

Head and Neck Cancers

Added text to state that in the Surveillance, Epidemiology, and End Results (SEER) database, the papillary subtype of thyroid cancer was the most common, followed by the follicular variant subtype, the follicular subtype, and the medullary subtype. As pediatric patients reached the ages of 15 to 19 years, the incidences of the papillary and follicular variant subtypes increased. An analysis of medullary thyroid cancer data revealed that the incidence was the highest in the age group of 0 to 4 years and declined at older ages.

Added text to state that the AGK-BRAF gene fusion was identified in 3 of 30 patients younger than 18 years with sporadic papillary thyroid carcinoma (cited Cordioli et al. as reference 84).

Revised text to state that pediatric thyroid surgery is ideally completed by a surgeon who has experience performing endocrine procedures in children and in a hospital with the full spectrum of pediatric specialty care.

Added text to state that because of the increased morbidity associated with central lymph node dissection, it is important to carefully individualize each case on the basis of the risks and benefits of the extent of dissection (cited Machens et al. as reference 100).

Added text to state that investigators have concluded that prophylactic central node dissection should not be performed on patients with hereditary medullary thyroid cancer if their basal calcitonin serum levels are lower than 40 pg/mL.

Thoracic Cancers

Added Travis et al., McMullan et al., Qian et al., Jindal et al., Butrynski et al., Chavez et al., Finck et al., and Pernas et al. as references 43, 44, 45, 46, 47, 48, 49, and 50, respectively.

Abdominal Cancers

Revised text to state that in adults, it has been accepted practice to remove the entire right colon in patients with large carcinoid tumors of the appendix or with tumors that have spread to the lymph nodes; however, there are data that confirm that right hemicolectomy is unnecessary in children.

Added text to state that a single-institution retrospective review of 8,382 patients who underwent appendectomy between 2000 and 2015 identified 30 cases of appendiceal carcinoid tumor. Surgery was limited to appendectomy and there were no recurrences; however, none of the tumors exceeded 1.5 cm (cited Lobeck et al. as reference 131 and level of evidence 3iiDiii).

Added Elf et al., Brabander et al., Gajate et al., and Liu et al. as references 135, 138, 139, and 140, respectively.

Added text about the results of an observational study carried out at the National Cancer Institute of 116 patients with presumed wild-type gastrointestinal stromal tumors, including the three distinctive subgroups identified by molecular profiling.

Other Rare Childhood Cancers

Added text to state that a large retrospective review from tertiary medical centers identified 95 of 748 patients whose pheochromocytoma or paraganglioma tumor first presented in childhood. Tumors resulting from cluster 1 susceptibility gene mutations were more prevalent in children than in adults.

Added text to state that a retrospective analysis identified seven children with poorly differentiated chordomas; the median survival of these patients was 9 months. All poorly differentiated chordomas showed loss of SMARCB1 expression by immunohistochemistry. Copy number profiles were derived from intensity measures of the methylation probes and indicated 22q losses affecting the SMARCB1 region in all poorly differentiated chordomas (cited Hasselblatt et al. as reference 178 and level of evidence 3iiA).

Added text to state that patients with chordomas and SMARCB1 mutations may be offered treatment with tazemetostat on the APEC1621C treatment arm of the APEC1621 trial.

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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