Pediatric malignant salivary gland tumors: 60 year follow up.

Objective: To evaluate the presentation, treatments and outcomes in pediatric patients with salivary gland malignancies.

Methods: Retrospective chart review (1950-2012), Prospective phone interview. Methods: Patients ≤18 years old with a salivary gland malignancy treated at our institution were identified. Patients were also contacted by phone for a follow up survey.

Results: Fifty-six patients were identified. Tumor origin was 88% parotid (n = 49), 5% (n = 3) submandibular and 7% (n = 4) minor salivary glands. Time from onset of symptoms to diagnosis was over one year (mean = 14.4 years). Fifteen out of 52 patients with major gland malignancy had a locoregional recurrence and local recurrences were almost all after initial enucleation. Two of these patients died of disease (overall disease specific survival = 96%). Three out of 4 patients with minor gland malignancy had a local recurrence and two patients with high grade pathology developed metastases and died of their disease (overall survival = 50%). On long term follow up survey in 13 patients (25%), 100% reported normal facial movement and 54% described symptoms of Frey's syndrome, which is higher than other reported series in children. Recurrence was noted up to 45 years after initial treatment.

Conclusions: The majority of malignant pediatric salivary gland tumors are low grade and have excellent survival, especially if found at an early stage. Minor salivary gland malignancies, particularly high grade, have a worse prognosis. Long term mild Frey's syndrome can be expected in approximately half of patients. We advocate a need for long term follow up and increased awareness among providers to diagnose these patients earlier.