Sialoblastoma of salivary glands in children: chemotherapy should be discussed as an alternative to mutilating surgery.

Sialoblastoma is a very rare congenital salivary gland tumor. No consensus has been reached concerning the treatment of this tumor due to its rarity. The treatment of reference is surgery, which can be mutilating, in the case of a locally invasive tumor. The treatment of metastatic disease is also controversial. The authors report a new case of a 6-year-old girl with a progressively growing left parotid mass since birth. The first cytological diagnosis was that of pleomorphic adenoma. Due to local progression, superficial parotidectomy was performed at the age of 3.5 years and revealed a diagnosis of sialoblastoma. Six months later, local recurrence and lung metastasis were treated by neoadjuvant chemotherapy with a very good partial response on the local recurrence and the lung metastasis, allowing complete parotidectomy with sacrifice of the facial nerve. Bilateral lung biopsies after adjuvant chemotherapy showed total necrosis. No recurrence was observed with a follow-up of 1 year. This case and a review of the literature confirm the very good chemosensitivity of this tumor and argue in favor of neoadjuvant chemotherapy for locally invasive tumors rather than extensive mutilating surgery.

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.

Congenital salivary gland anlage tumor of the nasopharynx.

OBJECTIVE: Nasal and upper respiratory tract obstruction in the neonatal period can result from a variety of conditions, and may present with variable symptoms. In the absence of dysmorphic features or other abnormalities, causes of nasal obstruction may be difficult to differentiate on initial examination. We report an unexpected and potentially life-threatening condition arising during the work-up of this common neonatal complaint. DESIGN: Case report with literature review. RESULTS: A male neonate presented with complaints of nasal obstruction and feeding difficulties. A common diagnostic approach to neonatal nasal obstruction was performed, resulting in an unexpected and potentially life-threatening, albeit curative, result. Cannulation of the nasal cavity to rule out choanal atresia resulted in a burst of bleeding from the nose and mouth. A finger sweep of the oropharynx produced a dislodged mass lesion. Pathology revealed a salivary gland anlage tumor of the nasopharynx. CONCLUSIONS: The diagnosis of a nasopharyngeal mass lesion should be considered in neonates with nasal obstructive symptoms. It is wise to place an index finger in the oropharynx when passing catheters to rule out choanal atresia to feel a dislodged mass lesion before it can become an airway foreign body. Should passage of nasal catheters result in bleeding and/or respiratory distress, the possibility of a displaced mass lesion must be considered immediately to institute prompt intervention.

Salivary gland anlage tumor. A case with widespread necrosis and large cyst formation.

We describe a case of the salivary gland anlage tumor (congenital pleomorphic adenoma). The tumor arose in the nasopharynx as a pedunculated mass. Microscopically most of the tumor contained large necrotic areas which revealed squamous cell metaplasia resulting in the formation of large cysts. This feature has never been described previously in this tumor and might lead to an erroneous diagnosis.

Salivary gland anlage tumor ("congenital pleomorphic adenoma"). A clinicopathologic, immunohistochemical and ultrastructural study of nine cases.

Salivary gland anlage tumor (SGAT) is a polypoid lesion of the nasopharynx that presents with respiratory distress at birth or within the first few days or weeks of life. Among our nine cases, there was a male predilection (7M:2F). All tumors were in the midline and attached to the posterior pharyngeal wall by a delicate pedicle. The largest tumor measured 3 cm. A biphasic histologic pattern of squamous nests and duct-like structures at the periphery blended into solid, predominantly mesenchymal-appearing nodules centrally. The surrounding submucosal mantle of epithelial structures was consistently immunoreactive for cytokeratin and epithelial membrane antigen, whereas the stromal-like cells of the central nodules showed variable immunopositivity for cytokeratin, vimentin, and muscle-specific actin. Both components were equally reactive for salivary gland amylase. Ultrastructurally, some of the stromal-like cells had features of myoepithelial cells. The histologic and architectural features of SGAT are similar in some respects to the developing salivary gland. It is proposed that the SGAT is a probable hamartoma of minor salivary gland derivation whose origin in the nasopharynx is potentially life-threatening in an infant.

Congenital pleomorphic adenoma of the nasopharynx (report of a case).

A case of congenital salivary gland tumour occurring in the nasopharynx is reported. Congenital neoplasms of the head and neck (of any histological type) and congenital tumours of the nasopharynx are discussed and the literature is reviewed.