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Congenital salivary gland anlage tumor: a case series
and review of the literature.Int
J Pediatr Otorhinolaryngol. 2005 Feb;69(2):149-56.
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.Pediatrics.
2003 Jul;112(1 Pt 1):e66-9.
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 of the nasopharynx: a clinicopathologic and
immuno-histochemical study of three cases.
Pediatr Pathol Lab Med. 1996;16(6):973-83.
The histologic
and immunohistochemical features of three congenital pedunculated
nasopharyngeal midline masses are reported. The follow-up in all
cases was uneventful. The tumors were characterized by solid and
cystic squamous nests and ductlike structures focally continuous
with the surface squamous mucosa of the tumor. Most of epithelial
structures coalesced with densely cellular stroma-like nodules.
Immunoperoxidase staining showed the presence of epithelial markers
in both spindle cells and epithelial structures. Spindle cells were
also reactive to vimentin and smooth muscle actin, revealing their
myoepithelial phenotype. Based on these observations, a diagnosis of
salivary gland anlage tumor, also called congenital pleomorphic
adenoma of the nasopharynx, was made. The similarity of these
tumors' architecture and cellular composition to the normally
developing salivary gland has led to the hypothesis of a tumorlike,
hamartomatous lesion developing in a site in which minor salivary
gland tissue occurs. This report reviews 12 identified cases of this
tumor, of which all but one (in which the patient died of sepsis)
had a favorable outcome. In an infant with respiratory distress
and/or feeding difficulties, these tumors must be differentiated
from other midline masses such as encephaloceles and teratomas. They
appear curable by surgical exeresis only.
Salivary
gland anlage tumor. A case with widespread necrosis and large cyst
formation.Pathology.
1996 May;28(2):128-30.
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.Am
J Surg Pathol. 1994 Jan;18(1):25-36.
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.
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