Year : 2016 | Volume
: 59 | Issue : 3 | Page : 273-
Salivary gland anlage tumor in a neonate
Sunil Vitthalrao Jagtap
Department of Pathology, Krishna Institute of Medical Sciences University, Karad, Maharashtra, India
Sunil Vitthalrao Jagtap
Department of Pathology, Krishna Institute of Medical Sciences University, Karad, Maharashtra
|How to cite this article:|
Jagtap SV. Salivary gland anlage tumor in a neonate.Indian J Pathol Microbiol 2016;59:273-273
|How to cite this URL:|
Jagtap SV. Salivary gland anlage tumor in a neonate. Indian J Pathol Microbiol [serial online] 2016 [cited 2020 Aug 6 ];59:273-273
Available from: http://www.ijpmonline.org/text.asp?2016/59/3/273/188116
Nasal airway obstruction in the neonatal period is a potentially life-threatening condition. The wide range of conditions is included to cause nasal and upper respiratory tract obstruction which includes congenital malformation, hamartoma, cystic lesion, mucosal edema, inflammatory conditions, and tumors.
Congenital salivary gland anlage tumor (SGAT) is a rare, benign lesion of the nasopharynx that clinically presents within 1st day or 1st week of life with respiratory distress. The term SGAT was first used by Dehner et al. in 1994. The report by Stillwater and Fee  in 1980 had described similar lesion as squamous cell proliferative lesion, and in 1985, Har-El et al. termed as congenial pleomorphic adenoma of the nasopharynx. It was proposed that SGAT is probably a hamartoma as it is mostly noted to the posterior pharyngeal wall in midline.
The most recent articles in PubMed search cite showed that approximately 25 cases of SGAT were reported in the literature. Most present in the neonatal period with a male predominance. Clinically, these cases were presented with nasal obstruction which worsened during feeding and improved on crying. However, in the absence of dysmorphic features or other abnormalities, cause of nasal obstruction is difficult to detect.
Symptoms of nasal obstruction may be severe and present with severe respiratory distress or in partial obstruction case with sleeping difficulty, feeding problems, episode of cyanosis and nasal discharge, etc., On anterior rhinoscopy/fibrotic endoscopy, SGAT appears as smooth nodular midline nasopharyngeal mass or to the mucosa of the posterior nasal septum. The size was reported up to 3 cm.
The common diagnostic approach is computed tomography (CT) and magnetic resonance imaging (MRI). On CT examination, the homogenous mass is noted but is important to evaluate on MRI study to define anatomy, size, and for assessment of intracranial extension before surgical intervention.
Histopathologically, SGAT is characterized by a mixture of benign but primitive epithelial and spindled cells forming proliferative nodules in a connective tissue stroma. Intermixed are more definitive ductal structures and nests of squamous epithelium. A hamartomatous rather than true neoplastic nature for this lesion is favored based on its midline location and a lack of recurrence following simple resection. It may show extensive necrosis, cyst formation, and squamous metaplasia mitosis is mild. Cellular atypia or pleomorphism is absent. Immunohistochemistry shows immunoreactive to AE1/AE3 keratin, EMA for epithelial component and vimentin, and SMA and focal S 100 protein for stromal tissue. Both epithelial and stromal spindle cells express salivary gland amylase. The common differential diagnoses for SGAT are: (1) Developmental mass lesions – meningomyelocele, nasal glioma, nasolacrimal mucocele, choanal stenosis, and pyriform aperture stenosis; (2) Benign lesions – hemangioma, hamartoma, dermoid, and teratoma; (3) Primary or secondary malignant neoplasms – rhabdomyosarcoma, neuroblastoma, chloroma, lymphoma, and langerhans cell histiocytosis; and (4) infectious causes.
SGAT is attached by a thin, delicate vascular pedicle which may be easily bleed or torn with gentle instrumentation so may result in dislodgement with resultant airway obstruction. It is necessary to careful approach and prompt intervention. Simple excision of lesion showed excellent result with no reported recurrence.
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