Mucoepidermoid carcinoma of salivary glands in children and adolescents: assessment of proliferation markers.

Malignant neoplasms represent one-third of all pediatric salivary gland tumors. Mucoepidermoid carcinoma (MEC) composes 51% of malignant tumors and 16% of all salivary gland neoplasms in pediatrics. Prognostic factors in MEC in pediatric patients have not been well defined. Histopathologic features, clinical outcomes and proliferation markers in 26 pediatric patients (median age 11 years; 19F:7M) with salivary gland MECs were evaluated retrospectively. MEC histocytologic grading used a three-tiered system. Proliferation was assessed by determining the percentage of tumor cells immunoreactive for PCNA and Ki-67. Tumor site was 16 parotid, eight submandibular, one base of tongue and one maxillary lip. Median tumor size was 2.5 cm (range 1.5-5 cm). MEC grade was nine low grade (LG), 15 intermediate grade (IG) and two high grade (HG). Metastatic disease and capsular invasion occurred in five cases, while perineural invasion was noted in three cases. Mean percentage of tumor cells immunoreactive for proliferation markers is as follows: PCNA: LG 9%, IG 17%, HG 32%; and Ki-67: LG 7%, IG 12%, HG 26%. Treatment was surgical in 21 cases, and surgery with chemotherapy and radiotherapy in five cases. Two patients with high grade MECs died of disease (21, 44 months). Twenty-four patients had no evidence of disease at a median follow-up of 104 months (range 30-298 months). MECs were second malignancies in two children with prior radiotherapy and chemotherapy for leukemia and histiocytosis. Low and intermediate grade salivary gland MECS in a pediatric population may have a favorable outcome when compared with high grade MECs. Proliferation markers appear to be linked to histocytologic MEC grade and may provide information regarding biologic behavior of salivary gland MECs in children and adolescents.