Salivary gland anlage tumor: Evaluation and management of a rare pediatric condition.

BACKGROUND: Neonates and infants are obligatory nasal breathers. Nasal obstruction can therefore be life-threatening and prompt recognition of its cause is critical. Salivary gland anlage tumor (SGAT) is a rare, benign condition that can present with nasal obstruction and respiratory distress within the first weeks of life. Our objective was to outline the clinical presentation, evaluation, and management of SGAT and provide a review of existing literature. METHODS: A retrospective chart review was performed at a tertiary children's hospital. A comprehensive literature search for SGAT was conducted using Medline, Embase, PubMed, and EBSCO. We present a case from our institution to describe the clinical approach to SGAT. RESULTS: Forty-one cases of previously reported SGAT, plus our new patient, were included. Patients presented with respiratory distress (30/42, 71.4%), feeding difficulty (14/42, 33.3%) and/or upper airway obstruction (13/42, 31.0%). The majority of tumors were in the nasopharynx (25/42, 59.5%). Surgical excision (36/42, 85.7%) was most commonly performed. Surgical approaches have evolved to include transoral, transpalatal, transnasal endoscopic or a combination of the above. Our patient had a suspected residual/recurrent tumor, later found to be negative on pathology. To date, there have been no reported recurrences. CONCLUSIONS: SGAT is a rare pediatric condition that is often excluded from the differential diagnosis of nasal obstruction. Clinical evaluation includes flexible nasopharyngoscopy and neuroimaging. Management involves surgical resection, except in cases of spontaneous expulsion. Tissue pathology is required for definitive diagnosis. Tissue biopsy should be performed on follow-up if there is a suspicion for recurrence.