Congenital salivary gland anlage tumor: a case series and review of the literature.

OBJECTIVE: To understand the clinical presentation and management of salivary gland anlage tumor (SGAT).

DESIGN AND METHODS: This case series includes a report of a newborn male who presented with acute airway obstruction secondary to a nasopharyngeal mass which was discovered in the course of the clinical evaluation. Six additional cases of SGAT from the pathology consultation files of one of the authors (LPD) presenting in similar fashion are also included. The relevant literature from 1966 to the present has been reviewed through a Medline keyword search utilizing terms "salivary gland anlage tumor", "neonatal", and "nasopharynx."

RESULTS: Endoscopic evaluation identified a nasopharyngeal mass tethered to the posterior septum. Although CT and MRI were helpful in identifying the mass and excluding involvement of the surrounding structures, the imaging characteristics of the mass itself were nonspecific. The patient was taken to the operating room and the polypoid mass was removed transorally after lysis of its septal attachment. Pathologic examination revealed a SGAT, a recently described entity in neonates and young infants, who present with early onset respiratory distress. Since the initial report of nine cases by one of the co-authors (LPD), seven additional cases including the present one have been seen in consultation.

CONCLUSIONS: Salivary gland anlage tumor of the nasopharynx is a rare cause of neonatal airway obstruction. Endoscopic evaluation and imaging studies are helpful in the exclusion of other etiologies, some of which may have intracranial extension. Simple excision has been curative to date. There have been no reported recurrences in any of the previously studied cases with clinical follow-up dating more than 5 years.