Unusual Cancers of Childhood Treatment (PDQ®)–Health Professional Version
Changes to This Summary (08/16/2019)
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.
Added text to state that a Danish population-based study of thyroid cancer in patients younger than 24 years reported that the age-adjusted incidence rate increased significantly from 0.36 in 1980 to 0.97 in 2014, with an average annual percent change of 2.9%. No change in overall survival was observed, but a significant increase was seen in the incidence of thyroid cancer among young adults, mainly attributed to an increase among females and patients with papillary carcinoma (cited Schmidt et al. as reference 67).
Added targeted therapy as a treatment option for papillary and follicular (differentiated) thyroid carcinoma.
Added text to state that larotrectinib has been used to treat patients with TRK fusion–positive thyroid carcinoma. Five of five patients with TRK fusion–positive thyroid carcinomas who were treated with lartotrectinib therapy achieved partial or complete responses (cited Drilon et al. as reference 96).
Added targeted therapy as a treatment option for childhood salivary gland tumors.
Added text to state that ten of 11 adolescent or adult patients with TRK fusion–positive salivary gland tumors who were treated with larotrectinib experienced partial or complete responses.
Added text to state that an Australian survey of pediatric otorhinolaryngologists documented a decline in the incidence of laryngeal papillomatosis after the introduction of human papillomavirus vaccinations for adolescent girls and young women aged 12 to 26 years (cited Novakovic et al. as reference 173).
Added text to state that other benign lesions include tubular adenoma, benign phyllodes tumor, and benign fibroepithelial neoplasm (cited McLaughlin et al. as reference 5).
Added Potter et al. as reference 36 and level of evidence 3iiA.
Added Surveillance as a new subsection in the Pleuropulmonary Blastoma section.
Added text to state that in a report of 12 patients with Leydig cell tumors, precocious puberty was the presenting symptom in 7 of 12 patients (cited Luckie et al. as reference 19 and level of evidence 3iiA).
Added text to state that in one series of patients with Leydig cell tumors, 3 of 12 patients were treated with enucleation alone, and 9 patients were treated with orchiectomy. All patients were alive at the last follow-up.
Added text about a retrospective analysis that identified 167 children with RET mutations who underwent prophylactic thyroidectomy and were classified into risk groups by their specific type of RET mutation (cited Machens et al. as reference 18).
Added text about three susceptibility gene cluster groups associated with an increased risk of pheochromocytoma and paraganglioma, including the pseudohypoxia group, the WNT signaling group, and the kinase signaling group (cited Crona et al. as reference 56).
Added text to state that the pseudohypoxia cluster group tumors are characterized by the absence of epinephrine production, whereas tumors in the other two cluster groups produce epinephrine. These differences reflect the absence, versus the presence, of the enzyme phenylethanolamine N-methyltransferase, responsible for conversion of norepinephrine to epinephrine.
Added text about the results of an Australian study that compared the whole-genome sequencing of melanomas in adolescents and young adults with the sequencing of melanomas in older adults (cited Wilmott et al. as reference 132).
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.
About This PDQ Summary
Purpose of This Summary
This PDQ cancer information summary for health professionals provides comprehensive, peer-reviewed, evidence-based information about the treatment of unusual cancers of childhood. It is intended as a resource to inform and assist clinicians who care for cancer patients. It does not provide formal guidelines or recommendations for making health care decisions.
Reviewers and Updates
This summary is reviewed regularly and updated as necessary by the PDQ Pediatric Treatment Editorial Board, which is editorially independent of the National Cancer Institute (NCI). The summary reflects an independent review of the literature and does not represent a policy statement of NCI or the National Institutes of Health (NIH).
Board members review recently published articles each month to determine whether an article should:
- be discussed at a meeting,
- be cited with text, or
- replace or update an existing article that is already cited.
Changes to the summaries are made through a consensus process in which Board members evaluate the strength of the evidence in the published articles and determine how the article should be included in the summary.
The lead reviewers for Unusual Cancers of Childhood Treatment are:
- Denise Adams, MD (Children's Hospital Boston)
- Karen J. Marcus, MD (Dana-Farber Cancer Institute/Boston Children's Hospital)
- Paul A. Meyers, MD (Memorial Sloan-Kettering Cancer Center)
- Thomas A. Olson, MD (Aflac Cancer and Blood Disorders Center of Children's Healthcare of Atlanta - Egleston Campus)
- Alberto S. Pappo, MD (St. Jude Children's Research Hospital)
- Arthur Kim Ritchey, MD (Children's Hospital of Pittsburgh of UPMC)
- Carlos Rodriguez-Galindo, MD (St. Jude Children's Research Hospital)
- Stephen J. Shochat, MD (St. Jude Children's Research Hospital)
Any comments or questions about the summary content should be submitted to Cancer.gov through the NCI website's Email Us. Do not contact the individual Board Members with questions or comments about the summaries. Board members will not respond to individual inquiries.
Levels of Evidence
Some of the reference citations in this summary are accompanied by a level-of-evidence designation. These designations are intended to help readers assess the strength of the evidence supporting the use of specific interventions or approaches. The PDQ Pediatric Treatment Editorial Board uses a formal evidence ranking system in developing its level-of-evidence designations.
Permission to Use This Summary
PDQ is a registered trademark. Although the content of PDQ documents can be used freely as text, it cannot be identified as an NCI PDQ cancer information summary unless it is presented in its entirety and is regularly updated. However, an author would be permitted to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks succinctly: [include excerpt from the summary].”
The preferred citation for this PDQ summary is:
PDQ® Pediatric Treatment Editorial Board. PDQ Unusual Cancers of Childhood Treatment. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/childhood-cancers/hp/unusual-cancers-childhood-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389315]
Images in this summary are used with permission of the author(s), artist, and/or publisher for use within the PDQ summaries only. Permission to use images outside the context of PDQ information must be obtained from the owner(s) and cannot be granted by the National Cancer Institute. Information about using the illustrations in this summary, along with many other cancer-related images, is available in Visuals Online, a collection of over 2,000 scientific images.
Disclaimer
Based on the strength of the available evidence, treatment options may be described as either “standard” or “under clinical evaluation.” These classifications should not be used as a basis for insurance reimbursement determinations. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.
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More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s Email Us.
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