Rare Pediatric Malignancies of the Head and Neck

Malignant head and neck neoplasms are rare occurrences in the pediatric population, and account for approximately 10% of all pediatric tumors. Less commonly encountered pediatric head and neck tumors include extraosseous Ewing's sarcoma (EOE), ameloblastoma odontogenic tumors, sinonasal undifferentiated carcinoma (SNUC), and esthesioneuroblastoma. Knowledge of the current management of these rare head and neck malignancies is essential in delivering optimal care and achieving favorable outcomes. Treatment strategies are similar to those applied to adults with the same neoplastic process, and include a multidisciplinary combination of surgery, radiation, and chemotherapy. However, it is important to be aware of the specific pathophysiology and tumor biology seen in pediatric malignancies. Close posttreatment surveillance is necessary as adverse effects may arise several years following cancer treatment. The following review will outline the biology and treatment of select rare pediatric head and neck malignancies.