Int. J. Oral Maxillofac. Surg.

2009; 38: 382–392

?available online at http://www.sciencedirect.com

Case Report
Congenital Craniofacial Anomalies

Heterotopic neuroglial tissue as M. C. Tambay1,3, I. Z. Rodriguez1,

Y. R. Gil2, A. R. Garcı́a1, R. S. Saez1,
J. J. M. Moreno1

a congenital laterocervical
1
Department of Oral and Maxillofacial
Surgery, Hospital Universitario ‘‘12 de
Octubre’’, Avenida de Cordoba s/n., Madrid,
28041, Spain; 2Histopathology Department,

mass: A case report Hospital Universitario ‘‘12 de Octubre’’,

Avenida de Cordoba s/n., Madrid, 28041,
Spain; 3Department of Oral and Maxillofacial
Surgery, Hospital General Ciudad Real,
C/Tomelloso s/n., Ciudad Rel, 13005, Spain
M. C. Tambay, I. Z. Rodriguez, Y. R. Gil, A. R. Garcı́a, R. S. Saez, J. J. M. Moreno:
Heterotopic neuroglial tissue as a congenital laterocervical mass: A case report. Int.
J. Oral Maxillofac. Surg. 38: 382–384. # 2009 International Association of Oral and
Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.
Keywords: heterotopic neuroglial tissue; neu-
Abstract. Heterotopic neuroglial tissue in the head and neck area is a rare clinical roglial mass; head and neck.
entity which can cause airway obstruction and feeding problems during the neonatal
period. The case is presented of heterotopic neuroglial tissue as a congenital Accepted for publication 7 May 2007
laterocervical and intraoral mass in a neonate. Available online 13 February 2009

Heterotopic neuroglial tissue in the head
and neck area is defined by the presence of
differentiated neuroectodermic tissue (neu-
rons are present in up to 10% of cases2)
anywhere in the head and neck area without
connection to the central nervous system
(CNS)1,3,4; it is not a true neoplasm5. This
condition usually involves the midline
structures3, but has been reported in other
areas such as the middle ear, parapharyn-
geal space, eye and orbit1,3,5–8. It may be
associated with other craniofacial abnorm-
alities, such as Pierre Robin syndrome8,
pectus excavatum and micrognathia.
Case report
A preterm baby girl was delivered, pre-
senting with shock symptoms and a
laterocervical, infratemporal, intranasal,
intraoral, parapharyngeal mass that
caused a respiratory and feeding disorder.
The initial diagnosis was made by fetal
ultrasound which indicated possible ter-
atoma. Immediate endotracheal intuba-
tion was required until tracheotomy at
the age of 2.5 months. Feeding was
assured parenterally and was then substi-
tuted by an orogastric tube for enteral
feeding. Ultrasound study showed the
presence of a 34  16 mm cystic mass;
computed tomography (CT) scan revea-
led an intact skull base and magnetic
resonance imaging (MRI) showed no
intracranial continuity. At the age of 21
days she underwent a first partial extirpa-
tion of the mass. The cystic specimen
measured 3.4  2.2  1.5 cm. Microsco-
pically, the lesion showed a background
of neuroglial fibers intermixed with a
fibrovascular connective tissue stroma
with maturing ganglion cells. Ependymal
cells were not present, but neurons were
frequently observed, raising the diagnosis
of ganglioneuroblastoma. Three weeks
later, a partial resection of the intraoral
component (Fig. 1) of the mass showed a
lesion composed of well differentiated
glial cells in a fibrovascular stroma,
with astrocytes and occasional oligoden-
drocytes. There was also ependyma-type
cystic degeneration and choroid plexus
tissue. Immunohistochemical staining
showed positivity for glial fibrillary
acidic protein and also S100 protein.
These histopathological findings were
consistent with heterotopic neuroglial tis-
sue (Fig. 2).
At the age of 12 months the entire
palatine tumor was excised (Fig. 3) allow-
ing closure of the tracheotomy 35 days
postoperatively; the gastrostomy tube was
retired when she was 15 months old. The
patient’s clinical evolution has been satis-
factory. There was no growth of the ori-
ginal lesion despite residual pathological
tissue. At the age of 3 years primary
closure of the palate was performed.
Velo-pharyngeal muscles were reanasto-

0901-5027/040382 + 011 $36.00/0 # 2009 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.
 Heterotopic neuroglial tissue as a congenital laterocervical mass: A case report 383

Fig. 2. Background of neuroglial fibers with frequent ganglion cells inter-

mingled (hematoxylin & eosin, original magnification 200). The inset shows
papillary excrescences of choroid plexus-like structures within the heterotopic
glial tissue (hematoxylin & eosin, original magnification 400).

Fig. 1. Intraoral mass before surgical extirpation. Extension

of the first resection was not completed due to the young age
and low weight of the patient; definitive ablative surgery was
delayed.

mosed and local flaps were used to achieve Histopathological differential diagnosis and astrocytes as well as more complex
complete closure of the mucosa. must be performed in the case of palatine CNS elements such as ependyma-lined
tumors which contain mature neural tis- structures or choroid plexus. The absence
sue, including teratoma7, encephalocele of these more complex structures may
Discussion
and glioma. Unlike glioma, heterotopic indicate that glioma and neuroglial hetero-
Heterotopic neuroglial tissue involving the neuroglial tissue might contain neurons topia are indeed separate entities2.
hard and soft palate is a rare cause of airway
obstruction in newborns. Many theories
have been put forward to explain the
appearance of neuroglial tissue in a hetero-
topic location1–3,5: an encephalocele that
has lost its connection to the CNS or has
become vestigial; the migration of totipo-
tent neuroectodermal cells during embry-
ogenesis which form isolated mature neural
tissue; an alteration in the fusion of the
condrocranium providing isolated nests
of primitive neural tissue.
A complete physical examination taking
special care around those areas where het-
erotopic neuroglial tissue may be present
and the presence of other congenital
abnormalities can help in the initial diag-
nosis of a laterofacial mass and/or airway
obstruction in a newborn or the first months
of life. A radiological assessment with CT
scan or MRI is always required (Fig. 4) to
provide information about the localization
of the tumor and its anatomic relations and
extent; an intracranial connection will be
better detected by a MRI study. A fine-
needle puncture of the mass or an incisional
biopsy will provide information about the Fig. 3. Residual cleft palate after complete resection of the palatine tumor component. Primary
type of tissue that forms the mass. closure of the palate was performed at the age of 3 years.
384 Tambay et al.
Fig. 4. (A and B) MRI shows a heterogeneous mass predominantly formed of multicystic components and solid nests. A (left): in the anterior
aspect we see destruction of the hard palate. B (right): more posteriorly the soft palate and oropharynx are compromised.
Heterotopic neuroglial tissue is a rare
entity and requires surgical removal as
definitive treatment1,5. Special attention
to vital structures and function must be
taken so that some residual mass can be
left in order to preserve these structures.
The timing of surgery is controversial; if
breathing and oral feeding are seriously
compromised, early surgical intervention
might prevent the necessity of a tracheot-
omy or gastrostomy. The need for three
operations is very infrequent, especially in
a case such as the present, because of its
size and location. First of all, the airway
and feeding must be secured. Initial extir-
pation of the oral mass allowed histo-
pathological study and was performed at
a very young age. Maybe a larger excision
could have been made in the second sur-
gical approach, but the diagnosis was still
not 100% sure and tumor growth was
under control. After definitive histopatho-
logical diagnosis and knowing the natural
outcome of this kind of tumor, a definitive
excision, leaving residual pathological tis-
sue in order to avoid additional damage
from the surgical procedures, was planned
and performed. Other surgical procedures,
such as the correction of the cleft palate,
can be delayed until an optimal age. In this
case, the cleft palate is a consequence of a
mass expansion effect rather than a clin-
ical association. The study of each indi-
vidual case will determine the optimal
moment and necessity for each surgical
procedure.
Acknowledgements. Esther Conde Gallego,
Jose Luis Rodrı́guez Peralto, Histopathol-
ogy Departament, ‘‘12 de Octubre Uni-
versitary Hospital’’, Madrid.
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Corresponding author. Address:
Manuel Castrillo Tambay
Department of Oral and
Maxillofacial Surgery
Hospital Universitario ‘‘12 de Octubre’’
Avenida de Córdoba s/n.
Madrid
28041
Spain
Tel: +34 913908245
Fax: +34 913908358
E-mail: mccmiguel@hotmail.com
doi:10.1016/j.ijom.2007.05.025