Cancer of the oral cavity is extremely rare in children and adolescents.[79,80] The incidence of cancer of the oral cavity has increased in adolescent and young adult females. The vast majority (>90%) of tumors and tumor-like lesions in the oral cavity are benign.[82,83,84,85] 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 histiocytoma [LCH]). Malignant tumors are found in 0.1% to 2% of a series of oral biopsies performed in children [82,83] and 3% to 13% of oral tumor biopsies.[84,85] 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.; [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 squamous cell carcinoma (OCSCC) between 1973 and 2006. Pediatric patients with OCSCC had better survival than adult patients. When differences in patient, tumor, and treatment-related characteristics are adjusted for, the two groups experienced equivalent survival.[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 bullosae, xeroderma pigmentosum, and human papillomavirus infection.[88,89,90,91,92,93,94,95] Most reported cases of SCC managed with surgery alone have done well without recurrence.[86,96]
Treatment of benign oral tumors is surgical. Management of malignant tumors is dependent on histology and may include surgery, chemotherapy, and radiation. LCH may require other treatment besides surgery. (Refer to the PDQ summaries on adult Oropharyngeal Cancer Treatment; Lip and Oral Cavity Cancer Treatment; and Langerhans Cell Histiocytosis Treatment for more information.)
Salivary Gland Tumors
Most salivary gland neoplasms arise in the parotid gland.[98,99,100,101,102,103] About 15% of these tumors may arise in the submandibular glands or in the minor salivary glands under the tongue and jaw. These tumors are most frequently benign but on very rare occasions may be malignant.
Sialoblastomas are usually benign tumors presenting in the neonatal period but can rarely metastasize. Chemotherapy regimens with carboplatin, epirubicin, vincristine, etoposide, dactinomycin, doxorubicin, and ifosfamide have produced responses in two children with sialoblastoma.; [Level of evidence: 3iiiDiv]
The most common malignant lesion is mucoepidermoid carcinoma.[108,109] 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.[110,111] Radical surgical removal is the treatment of choice, whenever possible, with additional use of radiation therapy and chemotherapy for high-grade tumors or tumors that have spread from their site of origin.[108,109,112,113] Prognosis for patients with these tumors is generally good.[101,109,114,115,116] (Refer to the PDQ summary on adult Salivary Gland Cancer Treatment for more information.)