SUMMARY:We present a case of congenital salivary gland anlage tumor (SGAT) of the nasal septum in a 2-week-old infant who had difficulty breathing through her nose since birth. CT and MR imaging demonstrated a circumscribed mass within the nasal cavity that did not communicate with the intracranial compartment. Differential diagnosis and clinical significance of recognizing this rare lesion are reviewed. N asal airway obstruction in the neonatal period is a potentially life-threatening condition that can be caused by a wide range of entities including congenital malformations, inflammatory conditions, hamartomas, and tumors. If a nasal mass is identified, then CT and MR imaging is often required to further characterize the mass and assess for intracranial involvement before intervention. Congenital salivary gland anlage tumor (SGAT) is a rare, benign lesion of the nasopharynx that presents with respiratory distress in the first days or weeks of life. Although this rare entity has been reported in the surgical and pediatric literature, its imaging features have not been reported in detail.
Case ReportA 2-week-old infant had trouble breathing through her nose since birth. The patient's pediatrician noted some bleeding when passing a catheter through the left naris, and the patient was referred to a pediatric otolaryngologist. A nasal endoscopy revealed a friable mass filling the left nasal cavity at the level of the middle turbinate. The right nasal cavity was narrowed because of a severe right septal deviation.CT without contrast enhancement showed a soft tissue attenuation mass centered within the left nasal cavity with benign-appearing bony remodeling of the ethmoid labyrinth. A connection between the mass and the intracranial contents could not be excluded because of normal lack of ossification of the skull base at this age. An MR imaging examination was then performed and demonstrated a well-defined mass filling the posterior left nasal cavity that was T1 isointense and T2 iso-hyperintense to the brain parenchyma, with an intact skull base and no evidence of intracranial communication (Fig 1A-C).The patient underwent CT-guided endoscopic resection of the mass, which was found to be pedicled to an approximately 4-mm area of the nasal septum. The choana and nasopharynx were clear.The fragmented tissue measured 3.0 ϫ 2.3 ϫ 0.8 cm in aggregate. Microscopic examination revealed an overlying nonkeratinized squamous cell lining with foci of mucosal erosions. Nests of epithelial cells arranged as cords, and ductal and solid structures extended from the mucosal layer into an edematous submucosa. The ductlike structures contained foci of squamous differentiation surrounded by round and spindled stromal cells (Fig 2). Cellular pleomorphism and mitotic figures were absent. No neural, glial, or rhabdomyomatous differentiation was present. These findings were compatible with an SGAT.
DiscussionThe term SGAT was first used in the literature by Dehner et al 1 in 1994. They reported 9 cases of SGATs within the nasopharynx, al...