Congenital salivary gland anlage tumor - in utero and postnatal imaging.

We present a case of an infant with congenital salivary gland anlage tumor, with fetal and postnatal imaging. To the best of our knowledge, this is the first case describing the in utero imaging findings of salivary gland anlage tumor. A fetal MRI was performed secondary to the clinical finding of polyhydramnios, which identified a nasopharyngeal mass. Because findings were concerning for airway obstruction, the fetus was delivered by ex utero intrapartum treatment (EXIT) to airway procedure. A postnatal CT confirmed the findings of the fetal MRI. The lesion was resected when the baby was 4 days old and recovery was uneventful.

Congenital minor salivary gland sialoblastoma: a case report and review of the literature.

Sialoblastoma is the most common epithelial tumor of the salivary gland. We report a case of congenital sialoblastoma arising in a minor salivary gland of the buccal mucosa of a male infant. After radiologic evaluation, an incisional biopsy was performed and then the mass was excised en bloc. Histologic features were both favorable and unfavorable. However, there was no recurrence for 5 months. In spite of a reported histologic grading system, the clinical course of isolated sialoblastoma is considered unpredictable. More published case reports of this rare tumor may enable histologic and clinical correlation in order to accurately predict prognosis.

Congenital salivary gland anlage tumor of the nasopharynx.

Nasal and upper respiratory tract obstruction in the neonatal period can result from a variety of conditions, and may be present with variable symptoms. Salivary gland anlage tumor, also referred as congenital pleomorphic adenoma, is a very rare benign congenital tumor of the nasopharynx, which may produce nasal obstruction and other associated, nonspecific symptoms. We report a case of congenital salivary gland anlage tumor causing a severe neonatal respiratory distress with pulmonary hypertension. The tumor was removed and the outcome was favourable without recurrence at five years of the follow up.

How to manage an unresectable or recurrent sialoblastoma.

Only 2-5% of all salivary gland tumors occur in children. Sialoblastoma is an extremely rare salivary gland tumor diagnosed at birth or shortly thereafter with significant variability in histological range and clinical course, so that it may be difficult to predict the most appropriate therapy. In cases where surgical removal is not curative or technically feasible, chemotherapy may be attempted. We report herein a patient with progression of a huge partially resected sialoblastoma who was successfully treated with chemotherapy. Systemic chemotherapy with vincristine, actinomycin D, and cyclophosphamide (VAC) seems to be an effective adjuvant or neoadjuvant treatment option for unresectable or recurrent sialoblastoma.

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.

Sialoblastoma arising in ectopic salivary gland tissue.

Sialoblastoma is a rare, congenital or perinatal tumour predominately affecting the parotid or submandibular salivary glands. We report a case of sialoblastoma arising in ectopic salivary gland tissue in the anterior cheek of a female infant. This lesion clinically and on ultrasound mimicked a haemangioma. Following imaging and tissue diagnosis, the lesion was surgically excised with clear margins. The patient remained disease free at 6 months follow up.

Congenital sialoblastoma (embryoma) associated with premature centromere division and high level of alpha-fetoprotein.

Sialoblastoma is a rare, locally aggressive, and potentially malignant perinatal salivary tumor that predominantly affects the parotid glands. To date, 29 cases of sialoblastoma have been reported. We report a further case of sialoblastoma diagnosed at 37 weeks of gestation presenting with novel findings that are the premature centromere division and a high level of alpha-fetoprotein.

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.

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.

Embryoma (sialoblastoma) of salivary glands.

There are four clinicopathologic categories of the exclusively major salivary gland tumors that present in the perinatal period. The two with the smallest representation among the 20 cases reported to date are those with a hamartomalike appearance and those with benign adult equivalents--the pleomorphic and monomorphic adenomas. Five cases have been undifferentiated or basaloid salivary carcinomas. Embryomas (sialoblastomas) are the most numerous. These tumors manifest a histologic phenotype like that of the epithelial anlage of the salivary glands, albeit in an arrested state of differentiation.

Sialoblastoma: a case report and review of the literature on congenital epithelial tumors of salivary gland origin.

The histologic, immunohistochemical, and ultrastructural features of a congenital epithelial tumor of the parotid were studied. The tumor was characterized by solid nests of epithelial cells intermingled with proliferating ductal structures lined by a double layer of cells. Immunoperoxidase staining for cytokeratin, vimentin, actin, and S-100 protein showed the presence of cytokeratin in the ductal cells as well as the presence of vimentin, actin, and S-100 protein in the outermost layer of the ducts. The solid nests were focally reactive to S-100 and vimentin. Ultrastructural examination revealed myoepithelial cells with replication of basement membrane material. The tumor recurred 17 months after excision without lymph node involvement or metastasis. The term "sialoblastoma" is favored. Review of the literature on congenital, epithelial salivary gland tumors showed that a few cases recurred locally and only one case had regional lymph node involvement. No distant metastasis has been reported.

Pharyngeal cleft sinuses and cysts, and other benign neck lesions.

Few pediatricians can expect to acquire great personal experience in dealing with many of the aforementioned lesions. Nevertheless, an appreciation of the usual presentation and natural history of most of the entities will usually permit an accurate diagnosis and guide to management in all but the most obscure conditions.

Congenital hybrid basal cell adenoma--adenoid cystic carcinoma of the salivary gland.

A congenital parotid gland tumor involving the facial nerve was excised, revealing histologic and ultrastructural features of a basal cell adenoma and adenoid cystic carcinoma. An asynchronous regional metastasis was successfully eradicated with adriamycin. Seven other similar appearing tumors occurring in neonates are documented in the literature and serve to establish this tumor as a clinicopathologic entity. The surrounding parotid gland had "dysplastic" changes attributed to obstruction during morphogenesis.

Congenital basal cell adenoma of the submandibular gland.

Basal cell adenoma is a specific type of monomorphic tumor of the salivary glands that closely resembles basal cell lesions of the skin. Twenty-six of these tumors have been reported in the American literature, the majority occurring in patients 60 years or older. The most common sites of occurrence have been the minor salivary glands (especially in the upper lip) and the parotid. We report an exceptional case of basal cell adenoma in the submandibular gland of a neonate. Review of reported congenital epithelial tumors of the salivary glands indicates that at least two similar neoplasms have been described previously.

Neonatal cystic choristoma in submandibular salivary gland simulating cystic hygroma.

A congenital cystic malformation occurred in the submandibular salivary gland of a neonate. The embryogenesis and differential diagnosis are discussed. Since it seems to be a product of endodermal heterotopic differentiation or entrapment of foregut epithelial rests in the submandibular gland, it is classified as a cystic choristoma.