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Unusual Cancers of Childhood Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Head and Neck Cancers

Table 2. Thyroid Carcinomas in Children continued...

(Refer to the Multiple Endocrine Neoplasia (MEN) Syndromes and Carney Complex section of this summary for more information.)

Treatment options under clinical evaluation

The following is an example of a national and/or institutional clinical trial that is currently being conducted. Information about ongoing clinical trials is available from the NCI Web site.

  • NCI-07-C-0189 (NCT00514046) (Vandetanib to Treat Children and Adolescents With Medullary Thyroid Cancer): This phase I/II trial of children and adolescents (aged 5–18 years) with medullary thyroid cancer whose tumor cannot be surgically removed, has grown back after treatment, or has metastasized (spread beyond the thyroid gland) is evaluating the activity, safety, and tolerability of vandetanib given once daily.

Oral Cancers


The vast majority (>90%) of tumors and tumor-like lesions in the oral cavity are benign.[99,100,101,102] Cancer of the oral cavity is extremely rare in children and adolescents. According to the SEER Stat Fact Sheets, only 0.6% of all cases are diagnosed in patients younger than 20 years, and in 2008, the age-adjusted incidence for this population was 0.24 per 100,000.[103,104]

The incidence of cancer of the oral cavity has increased in adolescent and young adult females, and this pattern is consistent with the national increase in orogenital sexual intercourse in younger females and human papilloma virus (HPV) infection.[105] It is currently estimated that the prevalence of oral HPV infection in the United States is 6.9% in people aged 14 to 69 years and that HPV causes about 30,000 oropharyngeal cancers. Furthermore, the incidence rates for HPV-related oropharyngeal cancer from 1999 to 2008 have increased by 4.4% per year in white men and 1.9% in white women.[106,107,108] Current practices to increase HPV immunization rates in both boys and girls may reduce the burden of HPV-related noncervical cancers.[109]


Benign odontogenic neoplasms include odontoma and ameloblastoma. The most common nonodontogenic neoplasms are fibromas, hemangiomas, and papillomas. Tumor-like lesions include lymphangiomas, granulomas, and eosinophilic granuloma (Langerhans cell histiocytosis) (Refer to the Oral mucosa subsection in the PDQ summary on Langerhans Cell Histiocytosis Treatment for more information about Langerhans cell histiocytosis).

Malignant lesions were found in 0.1% to 2% of a series of oral biopsies performed in children [99,100] and 3% to 13% of oral tumor biopsies.[101,102] Malignant tumor types include lymphomas (especially Burkitt) and sarcomas (including rhabdomyosarcoma and fibrosarcoma). Mucoepidermoid carcinomas have rarely been reported in the pediatric and adolescent age group. Most are low grade and have a high cure rate with surgery alone.[110]; [111][Level of evidence: 3iiA]

The most common type of primary oral cancer in adults, squamous cell carcinoma (SCC), is extremely rare in children. Review of the SEER database identified 54 patients younger than 20 years with oral cavity SCC between 1973 and 2006. Pediatric patients with oral cavity SCC were more often female and had better survival than adult patients. When differences in patient, tumor, and treatment-related characteristics are adjusted for, the two groups experienced equivalent survival.[110][Level of evidence: 3iA] Diseases that can be associated with the development of oral SCC include Fanconi anemia, dyskeratosis congenita, connexin mutations, chronic graft-versus-host disease, epidermolysis bullosa, xeroderma pigmentosum, and HPV infection.[112,113,114,115,116,117,118,119]

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