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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...

    The most common type of primary oral cavity 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.[113][Level of evidence: 3iA] Diseases that can be associated with the development of oral cavity and/or head and neck SCC include Fanconi anemia, dyskeratosis congenita, connexin mutations, chronic graft-versus-host disease, epidermolysis bullosa, xeroderma pigmentosum, and HPV infection.[115,116,117,118,119,120,121,122]

    Treatment

    Treatment of benign oral cavity tumors is surgical.

    Management of malignant tumors of the oral cavity is dependent on histology and may include surgery, chemotherapy, and radiation.[123] Most reported cases of oral cavity SCC managed with surgery alone have done well without recurrence.[113,124] Langerhans cell histiocytosis of the oral cavity may require treatment in addition to surgery. (Refer to the PDQ summary on Langerhans Cell Histiocytosis Treatment for more information.)

    Salivary Gland Tumors

    Incidence

    Salivary gland tumors are rare and account for 0.5% of all malignancies in children and adolescents.[125] Most salivary gland neoplasms arise in the parotid gland.[126,127,128,129,130,131,132,133] About 15% of these tumors may arise in the submandibular glands or in the minor salivary glands under the tongue and jaw.[132] These tumors are most frequently benign but may be malignant, especially in young children.[134] Overall 5-year survival in the pediatric age group is approximately 95%.[135]

    Histology

    The most common malignant lesion is mucoepidermoid carcinoma.[125,132,136,137] Less common malignancies include acinic cell carcinoma, rhabdomyosarcoma, adenocarcinoma, adenoid cystic carcinoma, and undifferentiated carcinoma. These tumors may occur after radiation therapy and chemotherapy are given for treatment of primary leukemia or solid tumors.[138,139] Mucoepidermoid carcinoma is the most common type of treatment-related salivary gland tumor, and with standard therapy, the 5-year survival is about 95%.[140,141]

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