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Eterotopia nasale di tessuto nervoso centrale
Автор G. Altissimi, S. Ascani1, S. Falcetti, C. Cazzato, I. Bravi1   

The Authors present a case of heterotopic central nervous system tissue observed in an 81-year-old male in the form of an ethmoidal polyp.

A review of the literature indicates that this is a rare condition characterised by a connective tissue lesion with astrocytic and oligodendrocytic

glial cells, which may be located outside the nasal pyramid in some cases and inside the nasal cavity in others. The most important

diagnostic aspect involves differentiating these from meningoencephalocele, which maintains an anatomical connection with central nervous

system tissue. Contrast-enhanced imaging is essential for diagnosis, as in cases of heterotopic central nervous system tissue, it will

demonstrate that there are no connections with intra-cranial tissue. Endoscopic excision is the treatment of choice.

Heterotopic Central Nervous System Tissue (HCNST) refers

to non-cancerous neuroglial tissue at extracranial sites, usually

along the central region of the face, inside or near the

nasal cavity (nasopharynx, nasal pyramid, upper lip). Also

known as nasal gliomas or glial heterotopias, they are generally

present at birth or diagnosed in early childhood
1 2. The

most common clinical form is a subcutaneous extra-nasal

mass, whereas intra-nasal polypoid growths resembling

inflammatory (allergic) polyps are more rare
3 4. There are

also mixed forms – intra-nasal and extra-nasal – communicating

through a defect in the nasal bones. The exact

incidence is unknown. In 1998, Pasquini et al.
5 referred

to 164 cases reported worldwide and, in 1998, Shah et al. 6

quoted approximately 100 cases; at present, approximately

200 cases have been reported
7-10. At histological level,

the polypoid masses appear to be composed of astrocytes

and neuroglial fibres mixed with a stroma consisting of

fibro-vascular connective tissue. These malformations do

not usually have any anatomical continuity with central

nervous system tissue, a characteristic that distinguishes

them from cases of encephalocele
11 12.

Herewith, a recent case of HCNST is described, presenting

as an intra-nasal polypoid mass, in an 81-year-old patient.

Case report

For approximately two years, R.A., an 81-year-old male,

complained of progressive unilateral nasal obstruction on

the right side. He did not report rhinorrhoea or nosebleeds.

He was being treated for hypertension but presented no

other significant pathological conditions. The otolaryngological

examination revealed a translucent polypoid mass,

ACTA otorhinolaryngologica italica 2009;29:EPUB 12/05/09

soft and greyish pink, that completely occluded the right

nasal cavity, extending into the naso-pharynx through the

choana (Fig. 1).

Computed tomography (CT) of the facial bones showed a

right antro-choanal mass, resembling a polyp. It occupied

the middle and superior nasal turbinates and extended

to the floor of the nasal cavity, with erosion of the bony

walls of the ethmoidal cells and obstruction of the maxillary

ostium, obliterating the antrum due to secretion stasis

(Fig. 2). No connection was observed with the meninges

or cerebral tissue.

Intra-operative examination revealed that the mass was

in the middle nasal turbinate; it was removed endoscopi-

Fig. 2. CT shows unilateral growth that occupies right nasal-paranasal sinuses.

Intra-operative examination revealed that the mass was

in the middle nasal turbinate; it was removed endoscopi-

cally, under general anaesthesia, with Weil forceps and a

microdebrider until complete patency of the nasal cavity

and maxillary ostium was restored.

Histological examination revealed, under a normal layer

of nasal respiratory epithelium, vascularised dense fibrous

connective tissue with mature astrocytic and oligodentrocytic

glial cells (Fig. 3). Immuno-histochemical typing

was performed and the cells were positive for glial fibrillary

acid protein (GFAP) and S100 protein (Figs. 4, 5).

Several neurons and ependymal cells were also found.

There were no mitotic figures or necrotic areas.

At the time of this report (8 months after surgery), the

patient is in good health and has shown no signs of recurrence.

Discussion

Despite the fact that glial heterotopias, in or near the nasal

cavity, are not true cancers, they clearly show hyperplastic

tendencies, since they present as polypoid masses that

closely resemble common allergic polyps. Extra-nasal lesions

appear as bluish or reddish subcutaneous masses.

Mixed intra-nasal and extra-nasal lesions are possible,

connected by a kind of isthmus that extends through a dehiscence

of the nasal bones
1 2 13 14.

Histopathological diagnosis is based on the observation

of mature glial cells (astrocytes and oligodendrocytes),

in stromal connective tissue, covered by normal respiratory

epithelium; neurons and ependymal cells may also

be present. When immuno-histochemical typing is performed,

the glial cells are positive for glial fibrillary acid

protein (GFAP) and S100 protein
15-18.

Intra-nasal masses generally originate in the vault of the nasal

cavity, particularly the superior or middle nasal turbinate
2.

Although these can be found at all ages, they are generally

diagnosed at birth or in early childhood. The present case

is unique due to the advanced age of the patient (81 years),

who reported that obstructive symptoms had arisen only

two years earlier.

The symptoms reported in the literature, related to these

lesions, include not only unilateral nasal respiratory obstruction

but also nosebleeds, cerebrospinal fluid leak and

meningitis
19.

The most interesting aspect of nasal gliomas involves

differential diagnosis with respect to encephalocele. The

Furstenberg test, which is typically positive when encephalocele

is involved (the mass becomes enlarged and

pulsatile when the ipsilateral jugular vein is compressed),

is negative in the case of HCNST. Nevertheless, with diagnostic

imaging, particularly contrast-enhanced CT or

magnetic resonance (MR), it is possible to exclude the

suspicion of encephalocele by demonstrating the lack

of an anatomical connection between the mass and the

central nervous system (CNS). Therefore, these tests are

mandatory in the event of a unilateral intra-nasal mass,

since the mere suspicion of encephalocele will obviously

lead to surgical planning in a specific direction.

Cases of encephalocele associated with nasal glial heterotopia

in a same patient have also been reported
20 21. According

to several Authors, nasal gliomas represent cases

of encephalocele, the intracranial connection of which has

been lost
22 23.

Biopsy is not advisable, since, if there is an intra-cranial

connection, it can cause cerebro-spinal fluid leak
13 24 25.

Endoscopic excision of intra-nasal HCNST is the treatment

of choice
26 27. The rate of recurrences, which are

related to inadequate primary excision, range from 4 to

10%
14 21. No cases of malignant degeneration have been

reported.

Conclusions

The presence of glial heterotopia is a possibility to be

taken into consideration in the management of unilateral

intra-nasal polypoid masses. With reference to this possibility,

it seems highly advisable to perform scrupulous

pre-operative diagnostic imaging (contrast-enhanced CT

or MR) in order to rule out fronto-basal encephalocele.

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