10.14639/0392-100X-N1977
Nasal glial heterotopia (NGH) is a rare congenital, non-neoplastic displacement of cerebral tissue in extracranial sites. Together with a case report of NGH, we present the first systematic review of all published cases in order to summarise the relevant clinical findings and appropriate therapy, making the available evidence accessible to decision makers. A total of 72 original publications including 152 NGH cases were identified. The male:female ratio was 3:2. Most patients were children under 18 years (130 patients) and only 8% of cases were diagnosed in adults. The main clinical presentation forms were asymptomatic masses around the nasal root as well as nasal congestion. Magnetic resonance imaging was performed in 39% of patients, computed tomography in 22% of patients and a combination of both in 20% of patients. A diagnostic biopsy was performed in only 7 patients. All patients underwent surgical treatment and recurrence was reported in 14 patients within the first year of follow-up. In conclusion, NGH should be considered as a differential diagnosis of nasal masses in children. MRI is mandatory in order to exclude a connection to the central nervous system. Complete resection is curative treatment.
text/xml
1827-675X
1977
en
Simona Negoias
http://creativecommons.org/licenses/by-nc-nd/4.0
ACTA Otorhinolaryngologica Italica
nasal cerebral heterotopia
Nasal glial heterotopia: a systematic review of the literature and case report
Text.Serial.Journal
https://www.actaitalica.it/
42
Reviews
2022-09-30
2021-12-20
2022-10-12
2022-10-12
317-324
1
Simona Negoias
2022/09/30
10.14639/0392-100X-N1977
317
Department of Otorhinolaryngology, Head and Neck Surgery, University Hospital of Basel, Basel, Switzerland
1827-675X
nasal cerebral heterotopia
en
324
Nasal glial heterotopia: a systematic review of the literature and case report
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ACTA OTORHINOLARYNGOLOGICA ITALICA 2022;42:317-324; doi: 10.14639/0392-100X-N1977




Review


Nasal glial heterotopia:
a systematic review of the literature and case report
Eterotopia gliale nasale: una review sistematica della letteratura e case report
Montserrat Gallego Compte1, Thomas Menter2, Nicolas Guertler1,3, Simona Negoias1
1
  Department of Otorhinolaryngology, Head and Neck Surgery, University Hospital of Basel, Basel, Switzerland; 2 Department of
Pathology, Institute of Medical Genetics and Pathology, University Hospital Basel, University of Basel, Switzerland; 3 Department of
Otorhinolaryngology, University Children’s Hospital Basel, University of Basel, Switzerland


SUMMARY
Nasal glial heterotopia (NGH) is a rare congenital, non-neoplastic displacement of cerebral
tissue in extracranial sites. Together with a case report of NGH, we present the first system-
atic review of all published cases in order to summarise the relevant clinical findings and
appropriate therapy, making the available evidence accessible to decision makers. A total of
72 original publications including 152 NGH cases were identified. The male:female ratio
was 3:2. Most patients were children under 18 years (130 patients) and only 8% of cases
were diagnosed in adults. The main clinical presentation forms were asymptomatic masses
around the nasal root as well as nasal congestion. Magnetic resonance imaging was per-
formed in 39% of patients, computed tomography in 22% of patients and a combination of
both in 20% of patients. A diagnostic biopsy was performed in only 7 patients. All patients
underwent surgical treatment and recurrence was reported in 14 patients within the first
year of follow-up. In conclusion, NGH should be considered as a differential diagnosis of
nasal masses in children. MRI is mandatory in order to exclude a connection to the central
nervous system. Complete resection is curative treatment.
KEY WORDS: nasal glial heterotopia, nasal glioma, neuroglial heterotopia, nasal cerebral
heterotopia
                                                                                                   Received: December 20, 2021
                                                                                                   Accepted: May 3, 2022
RIASSUNTO
L’eterotopia gliale nasale (NGH) è una rara dislocazione congenita e non neoplastica del           Correspondence
tessuto cerebrale nei siti extracranici. Insieme a un case report di NGH, presentiamo la           Montserrat Gallego Compte
prima revisione sistematica di tutti i casi pubblicati al fine di riassumere i risultati clinici   Department of Otorhinolaryngology, Head and
rilevanti e la terapia appropriata, rendendo le prove disponibili accessibili ai decisori.         Neck Surgery, University Hospital of Basel, Peter-
Sono state identificate un totale di 72 pubblicazioni originali, che hanno analizzato 152          sgraben 4, CH-4031 Basel, Switzerland
                                                                                                   Tel. +41 77 9916092
casi di NGH. Il rapporto maschi:femmine era 3:2. La maggior parte dei pazienti erano
                                                                                                   E-mail: montserrat.gallego@usb.ch
bambini sotto i 18 anni (130 pazienti) e solo l’8% dei casi è stato diagnosticato negli adulti.
Le principali forme di presentazione clinica erano masse asintomatiche intorno alla radice
nasale e congestione nasale. La risonanza magnetica è stata eseguita nel 39% dei pazienti,           How to cite this article: Gallego Compte
la tomografia computerizzata nel 22% dei pazienti e una combinazione di entrambe le                  M, Menter T, Guertler N, et al. Nasal glial
tecniche è stata utilizzata nel 20% dei pazienti. Una biopsia diagnostica è stata eseguita in        heterotopia: a systematic review of the literature
                                                                                                     and case report. Acta Otorhinolaryngol Ital
soli 7 pazienti. Tutti i pazienti sono stati sottoposti a trattamento chirurgico ed è stata se-
                                                                                                     2022;42:317-324. https://doi.org/10.14639/0392-
gnalata una recidiva in 14 pazienti entro il primo anno di follow-up. In conclusione, l’NGH          100X-N1977
dovrebbe essere considerato fra le diagnosi differenziali delle masse nasali nei bambini. La
risonanza magnetica è indispensabile per escludere una connessione con il sistema nervoso          © Società Italiana di Otorinolaringoiatria
centrale. Una resezione completa è il trattamento curativo.                                        e Chirurgia Cervico-Facciale

PAROLE CHIAVE: eterotopia gliale nasale, glioma nasale, eterotopia neurogliale,
                                                                                                                  OPEN ACCESS
eterotopia cerebrale nasale
                                                                                                   This is an open access article distributed in accordance with
                                                                                                   the CC-BY-NC-ND (Creative Commons Attribution-Non-
Introduction                                                                                       Commercial-NoDerivatives 4.0 International) license. The
                                                                                                   article can be used by giving appropriate credit and mentio-
                                                                                                   ning the license, but only for non-commercial purposes and
Neuroglial heterotopias are rare congenital, non-neoplastic displacements of                       only in the original version. For further information: https://
cerebral tissue in extracranial sites. The incidence of congenital nasal masses                    creativecommons.org/licenses/by-nc-nd/4.0/deed.en




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is reportedly 1 in 20,000-40,000 live births 1,2, and nasal       positive in neurons. There were no signs of malignancy. An
glial heterotopia (NGH) accounts for approximately 5%             endoscopic excision was planned at the age of 1 year and
of them 3. Since the nose and nasopharynx are the most            2 months. The tumour appeared as a white doughy mass
commonly involved areas, NGH is also known as nasal               with no clear margins. Owing to its extension and difficulty
glioma 4. Other affected sites include ear, face, neck and        to identify the tumour margins, we decided to improve the
orbit. Nasal glioma was probably reported for the first time      exposure of the mass by a combined approach with a lateral
by Reid in 1852 5, but the term itself was introduced by          rhinotomy (Figs. 2B-D). During the operation, there were
Schmidt in 1900 6.                                                no defects of the anterior skull base and no signs of intra-
The development of nasal cerebral heterotopias is embry-          cranial connection. Histologically, the surgical margins on
ologically similar to that of nasal encephaloceles or der-        the lateral and anterior nasal wall as well as inferior tur-
moids. During retraction of the embryonic dural divertic-         binate were involved and revision surgery was performed
ulum, remnants of neural glial tissue become sequestered          one week later. These additional resections did not identify
when their connections to the subarachnoid space are              more remnants of the glioneuronal heterotopia. The post-
pinched off and obliterated 7,8. The lack of subarachnoid         operative recovery period was uncomplicated and the child
communication distinguishes NGH from anterior enceph-             was discharged.
aloceles 9. The distinction between glioma and enceph-            Postoperative endoscopy and control MRI confirmed that
alocele is not possible based on histopathologic findings         the mass was completely removed. At 1 year follow-up,
because glial tissue may be the predominant or exclusive          both MRI and nasal endoscopy including biopsy under
component in both types of lesions 10.                            general anaesthesia showed no signs of residual tissue or
Due to the rarity of this entity, its diagnosis can be delayed
                                                                  recurrence (Fig. 1B, Fig. 2E,F). The scar of the lateral rhi-
and the diagnostic tools, treatment options and follow-up
                                                                  notomy was barely visible.
may be unclear. Although some reviews exist, they are nei-
ther systematic or complete 4,11. We present a case report
and the first systematic review of all reported NGH.              Materials and methods
                                                                  A systematic literature search was done using PubMed and
Case report                                                       following a flow diagram based on the PRISMA Statement
                                                                  from 2015 12 (Fig. 3). PubMed was searched up to October
A 4-month-old boy was referred to our clinic due to nasal
                                                                  27th 2021 using the keywords (nasal OR nose OR endona-
obstruction with choking when breast-feeding since birth.
                                                                  sal OR intranasal OR extranasal) AND (glial OR neuroglial
No further symptoms were reported. The physical exam-
ination revealed an obstructive swelling of the inferior tur-     OR glioma) AND (heterotopia OR heterotopic). No further
binate in the right nasal cavity as well as a nasal septum        search restrictions were made. Additional articles were
deviation to the left side. Initial therapy with cortisone-con-   identified through hand searching from reference reviews.
taining nasal spray proved unsuccessful. The follow-up            In order to avoid duplication, there were no reviews includ-
showed that the findings were persistent and the symptoms         ed in the analysis, but only original case publications.
did not improve. Imaging was advised, which was refused           The search results were screened by titles and abstracts on
by the parents. Due to persistent symptoms, a magnetic            the basis of the exclusion criteria. After this selection, ar-
resonance imaging (MRI) was performed at the age of 1             ticles were retrieved or requested in full text and assessed
year, which showed a tumour in the right nasal cavity in          for eligibility. Some articles were excluded from further
contact with the inferior nasal turbinate with extension to       study for reasons of “full text not available” or insufficient
antero-cranial to the roof of the nose. An intracranial con-      clinical information. In one case of possible duplication,
nection as well as an encephalocele were ruled out (Fig. 1).      the author was contacted. A graphical representation of the
Subsequently, endoscopic examination with biopsy under            screening process is shown in Figure 3.
general anaesthesia was performed. During procedure, it           The following parameters were listed for analysis: gender,
was found that the basal part of the lesion was located in        age at the time of surgery, location of the tumour, laterality,
the anterior part of the inferior turbinate and extended cra-     symptoms, imaging, biopsy, type of surgery, bony defect,
nially to the roof of the nose (Fig. 2A). Histopathological       intracranial connection, length of follow-up and recurrence.
examination confirmed a diagnosis of neuroglial heteroto-
pia (Fig. 3). Immunohistochemical staining for glial fibril-      Statistics
lary acidic protein (GFAP) confirmed the presence of glial        The data of patients included in the review were analysed
tissue, S100 and synaptophysin and chromogranin A were            by descriptive statistics.



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Figure 1. (A) MRI T2-weighted image showing the mass (arrows) in the right nasal cavity, in contact with the inferior nasal turbinate, with a dimension of 1.7
x 2.6 x 2.1 cm and no connection between the mass and the dura or brain. (B) MRI T2-weighted image 1 year after surgery showing no recurrence of the NGH.



Results                                                                          in 69 cases, extra-nasal (protruding from the nasal root)
                                                                                 in 54 patients and mixed in 29 patients. All adult patients
Our search yielded 152 case reports resulting from 72 pub-
                                                                                 who were included in this study had an intra-nasal form of
lications and our case (Fig. 3).
                                                                                 NGH. There was no difference in the laterality of the lesion
An extensive review of literature undertaken by Lamesch 11
                                                                                 with 42% left-sided lesions and 45% right sided lesions. In
included 166 cases between 1900 and 1986 extracted from
                                                                                 only 12 patients (12% of the reported cases) did the tumour
reviews as well as original case reports. Through hand
searching, 24 of these 166 cases could be retrieved and                          appear in a purely midline location.
included in our publication. This means, that in addition                        The most common clinical presentation of a NGH is an as-
to our reviewed 152 cases, 142 additional non-retrievable                        ymptomatic tumour (49%) or with nasal congestion (41%).
cases of nasal glioma were documented in the review from                         The majority of patients underwent MRI (39%) prior to
Lamesch, making a total of at least 294 reported cases of                        surgery. Computed tomography (CT) scan was the pre-
this entity. These 142 cases were excluded for lack of trace-                    ferred imaging method in 22% of cases, and a combination
able documentation.                                                              of CT and MRI was performed in 25 patients (20%). An
Data regarding the analysed NGH cases is summarised in                           additional ultrasonography was used as a complementary
Table I. A male:female ratio of 3:2 was identified. Most                         diagnostic method only for extra-nasal tumours in 9 cases.
patients (84%) were diagnosed and treated before the age                         In isolated cases between 1950 and 1998, X-ray was used.
of 3. Only 11 patients were diagnosed during adulthood,                          A diagnostic biopsy was performed in only 7 cases (5%).
the remaining were children (92%). The location of the le-                       Surgery was the only therapy applied. An external ap-
sion was intra-nasal (located in the nasal cavity or sinuses)                    proach was used in 70 cases, while 49 patients underwent



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M. Gallego Compte et al.




                                                                                   Figure 3. (A) high magnification of the glioneuronal heterotopia showing both
                                                                                   neurons and glial cells (H&E, 400x); (B) staining for GFAP (glial fibrillary acidic pro-
                                                                                   tein) shows the extent of the glioneuronal heterotopia in the first resection.


                                                                                   bone, followed by the cribriform plate, ethmoid and sphe-
                                                                                   noid.
                                                                                   Recurrence after surgery was reported in 14 cases. They
                                                                                   appeared between 5 weeks to 11 months after surgery. All
                                                                                   patients with recurrence underwent revision surgery. Post-
                                                                                   operative follow-up investigations to rule out recurrence
                                                                                   were reported in only 5% of cases and included endoscopy
                                                                                   and MRI.

                                                                                   Discussion
                                                                                   Nasal glial heterotopia is a rare nonhereditary, benign con-
                                                                                   genital anomaly. Since first described by Reid in 1852, ap-
                                                                                   proximately 300 cases have been reported. In this study, the
                                                                                   first systematic review of cases of nasal glial heterotopia
                                                                                   or the so-called “nasal glioma” in children and adults pub-
Figure 2. (A) nasal endoscopy before tumour resection (IT: right nasal inferi-
or turbinate, NGH: nasal glial heterotopia, S: septum); (B) intraoperative nasal   lished so far is presented, including a total of 152 retriev-
endoscopy (MT: right nasal middle turbinate, S: Septum); (C) resected mass;        able cases described in original publications. The available
(D) result after endoscopic resection and lateral rhinotomy; (E) postoperative     reviews lack a systematic approach. For example, the latest
nasal endoscopy shows an unobstructed right nasal cavity (MT: right nasal
middle turbinate, S: septum); (F) scar of the lateral rhinotomy during postop-
                                                                                   review of the literature, performed in 2020 by Yan 4, de-
erative nasal endoscopy.                                                           scribes only 60 paediatric cases.
                                                                                   NGH occurred with a male to female ratio of 3:2, as de-
                                                                                   scribed in previous reports 4,13. The majority of patients
endoscopic or intranasal microscopic surgery. In 16% of                            were diagnosed and treated before the age of 1 year (66%).
cases the type of surgery was not specified. Other surgical                        Nevertheless, this entity can also be found in adulthood
approaches included explorative craniotomies in cases in                           (8%), always involving an intra-nasal lesion (inside the na-
which diagnostic imaging showed a possible intracranial                            sal cavity). The intra-nasal location of NGH is the most fre-
connection and an intra-oral approach in a case of simulta-                        quent (45%) and was generally diagnosed much later than
neous cleft palate and NGH.                                                        the extra-nasal type. The incidence rate of the mixed type of
Histological evaluation of surgical margins was reported in                        NGH is 19%. The leading symptom was nasal congestion
only 11 cases.                                                                     (41% of all NGH cases) although it can also appear in form
An intracranial connection appeared in 15 cases, all de-                           of rhinorrhoea, epistaxis, meningitis, chronic rhinosinusitis
scribed as a fibrous stalk. Out of these 15 cases with intra-                      and strabismus (8%). The extra-nasal type of NGH (36%)
cranial connection, 3 were located extra-nasal and the rest                        is normally detected after birth in form of a firm, non-com-
were intra-nasal or mixed. Bony defects were described in                          pressible mass often with a red or bluish appearance on the
13% of cases and were most frequently found in the nasal                           dorsum of the nose. The most common locations for NGH



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                                                                                                             Nasal glial heterotopia




Table I. Results of the systematic review on nasal glial heterotopia.
 Variable                                                                           N = 152     Ignoring not reported cases for
                                                                                                         each variable
 Gender                                                                                                    N = 123
    Female                                                                          49 (32%)               49 (40%)
    Male                                                                            74 (49%)               74 (60%)
    Not reported                                                                    29 (19%)
 Age at time of surgery                                                                                    N = 141
    ≤ 1 year                                                                         93 (61%)              93 (66%)
    1-3 years                                                                        26 (17%)              26 (18%)
    4-6 years                                                                         6 (4%)                6 (4%)
    6-18 years                                                                        5 (3%)                5 (4%)
    Total children                                                                  130 (86%)             130 (92%)
    > 18 years                                                                       11 (7%)               11 (8%)
    Not reported                                                                     11 (7%)
 Location
    Extra-nasal                                                                     54 (36%)
    Intra-nasal                                                                     69 (45%)
    Mixed                                                                           29 (19%)
 Laterality                                                                                                 N = 99
    Left                                                                            42 (28%)               42 (42%)
    Right                                                                           45 (30%)               45 (45%)
    Midline                                                                          12 (8%)               12 (12%)
    Not reported                                                                    53 (35%)
 Symptoms                                                                                                  N = 139
    Asymptomatic                                                                    68 (45%)               68 (49%)
    Nasal congestion                                                                57 (38%)               57 (41%)
    Cough, choking when feeding                                                      3 (2%)                 3 (2%)
    Other: rhinorrhoea, epistaxis, meningitis, strabismus, chronic rhinosinusitis   11 (7%)                11 (8%)
    Not reported
 Imaging                                                                                                   N = 124
    CT                                                                              27 (18%)               27 (22%)
    MRI                                                                             49 (32%)               49 (39%)
    CT + MRI                                                                        25 (16%)               25 (20%)
    Additional ultrasonography                                                       9 (6%)                 9 (7%)
    No imaging                                                                       7 (5%)                 7 (6%)
    Other: X-Ray, PET-CT                                                            17 (11%)               17 (14%)
    Not reported                                                                    27 (18%)
 Diagnostic biopsy                                                                   7 (5%)
 Surgical approach                                                                                         N = 148
    External / rhinotomy                                                            70 (46%)               70 (47%)
    Intranasal /endoscopic                                                          49 (32%)               49 (33%)
    Combined                                                                         2 (1%)                 2 (1%)
    Not defined                                                                     23 (15%)               23 (16%)
    Other (craniotomy, enoral)                                                       4 (3%)                 4 (3%)
    Not reported                                                                     4 (3%)
 Bony defect                                                                        19 (13%)
 Intracranial connection                                                                                   N = 134
    Fibrous stalk                                                                   15 (10%)              15 (11%)
    No connection                                                                   119 (78%)             119 (89%)
    Not reported                                                                    18 (12%)
 Follow-up (2 months to 26 years)                                                                          N = 101
    Recurrence                                                                      14 (9%)                14 (14%)
    No recurrence                                                                   90 (57%)               90 (86%)
    Not reported                                                                    51 (34%)
 Postoperative imaging/endoscopy                                                     8 (5%)




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M. Gallego Compte et al.




Figure 4. Flow diagram of the publication search, based on the PRISMA Statement 2015 12.


include the glabella and nasomaxillary suture and they tend                  most common diagnostic imaging was MRI alone (39%) or
to grow in proportion to the child 14.                                       with additional CT (20%), while CT alone was used in 22%
The evaluation of a congenital midline mass includes nasal                   of cases. In MRI, the lesion has signal intensity similar to
endoscopy as well as an imaging technique to evaluate the                    the brain on T1-weighted images (T1-WI) and high signal
extension of the tumour and exclude intracranial involve-                    intensity on T2-weighted images (T2-WI), with many cys-
ment or association with bone defect prior to surgery. The                   tic regions and does not enhance. No diffusion restriction



322
                                                                                                            Nasal glial heterotopia




appears in diffusion-weighted imaging (DWI). MRI is es-         and insufficient exposure through endoscopic approach, we
sential to rule out intracranial communication. Computed        decided to perform a combined technique by lateral rhi-
tomography demonstrated adjacent osseous structures and         notomy. While in the last years an endoscopic approach
bone defects preoperatively. In CT images, it appears as        has been proven to be the favourite for intra-nasal lesions,
a large, well-defined, hypodense soft-tissue mass 15. X-ray     we consider that each case should be individually evalu-
alone (14%) and no imaging diagnosis (6%) was reported          ated when planning surgery. Early resection is advised to
in a few cases. A timeline bias must be considered for im-      avoid the development of craniofacial deformities, particu-
aging diagnosis, with more recent case reports increasingly     larly involving the nasal architecture. Before any surgical
using MRI with or without CT. B-mode ultrasonography            intervention, imaging is mandatory. A connection to the
was used in 7% of cases to evaluate extension and bony          brain was found in 11% of cases, always in the form of a
defects of ex-nasal NGH. In summary, an MRI would be            fibrous stalk, which does not contain a direct fluid-filled
mandatory, while CT is highly recommended if bony de-           tract that communicates with the subarachnoid spaces. This
fects are suspected or as navigation support for endoscopic     incidence rate is similar to the previously described by Mo-
surgery.                                                        ron et al. 9. Bone defect appeared in 13% of cases and were
A diagnostic biopsy was performed in only 7 cases (5%).         most frequently found in the nasal bone.
According to the previous reviews, biopsies are not advised     A complete surgical excision showing no remains of NGH
in congenital midline masses because of CSF leak risk in        in the margins should guarantee no recurrence in the future.
case of intracranial connection 16. In our opinion, a biopsy    In our case, a revision had to be performed one week after
may be advisable especially in adults to exclude malignity,     primary surgery due to histologically positive surgical mar-
always after an appropriate diagnostic imaging (MRI) to         gins. Although most authors describe to have performed a
rule out an intracranial connection. MRI and biopsy could       complete surgical resection, histological margins were re-
be combined to avoid a second anaesthesia in paediatric pa-     ported in only 11 cases: 10 showed negative margins and
tients. Neurosurgical consultation may still be necessary in    never developed recurrence, while in our case margins
cases of reasonable doubts or intraoperatively discovered       were involved in the primary excision. Only 14% of cases
tract coursing to the skull base.                               presented recurrences after surgery and all appeared in a
Tetzlaff et al. published the only case of sinonasal undif-     short period of time (from 5 weeks to 11 months). These
ferentiated carcinoma (SNUC) arising in a background            incidence rates differ from those reported by Yan et al. who
of NGH in a 37-year-old patient 17. Numerous syndromes          described a 5% recurrence during a follow-up from 0.5 to 3
have been described in association with encephaloceles,         years 4. This difference may be attributable to the relatively
but NGH are typically isolated lesions, with syndromic as-      small sample size (60 patients) of the study by Yan et al. Pre-
sociation being exceedingly rare and only 1 case reported       vious reviews reported the lack of recurrence or tumour-in-
of NGH in association with metopia craniosynostosis 18.         vasion into adjacent tissues during long-term follow-up 19.
There is no association described between the presence of       Schroth et al. 20 described a regrowth of an intra-nasal NGH
heterotopic neuroglial tissue with an epithelial-derived ma-    mass just 5 weeks after primary excision, which was more
lignancy 17. This could be explained due to the young age       infiltrating than the initial tumour which required a much
of most patients diagnosed and treated for NGH. Although        more mutilating excision including nasal cartilages, upper
there is no data to suggest a causal connection between         nasal septum and parts of the nasal bone, as well as a poste-
SNUC and NGH, it would be interesting to follow cases of        rior reconstruction of the nose with autologous skin and ear
both entities in order to report any potential causal or syn-   cartilage transplants to correct the defects. Although dif-
dromic association between these two conditions.                ferentiation between recurrence and persistence is always
The treatment of choice is complete surgical excision,          difficult, we postulate that given the benign character of the
which is curative and allows pathohistological confirma-        entity, a persistence due to incomplete resection is more
tion of the diagnosis. The surgical approach will depend        probable than a true recurrence. Based on this data and our
on the extension and localisation of the NGH: external rhi-     experience, we would recommend performing a complete
notomy, endoscopic resection or combined approach are           excision with histologically free margins and a follow-up
the most common techniques. Since accurate intraopera-          period of at least 12 months after surgical excision includ-
tive histologic diagnosis may be difficult, the surgeon must    ing clinical examination. In uncertain cases, MRI should be
decide either to proceed with radical, potentially deforming    evaluated during the follow-up. In our case, we performed
surgery or to operate less radically with the possibility of    both MRI and nasal endoscopy with biopsy simultaneously
local recurrence. In our case, due to the location and exten-   to increase safety and avoid a possible future anaesthesia
sion of the mass towards the lateral and anterior nasal wall    for the patient.



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In terms of strengths and limitations of our study, we present        References
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                                                                           es: CT- and MR imaging features in 16 cases. AJNR Am J Neuroradiol
involvement is suspected or necessary for endoscopic surgery.              1991;12:105-116.
A complete resection is the curative treatment. Histological          9
                                                                           Moron FE, Morriss MC, Jones JJ, et al. Lumps and bumps on the head
evaluation of the surgical margins can reduce the risk of recur-           in children: use of CT and MR imaging in solving the clinical diagnos-
rence. A follow-up period of 1 year seems to be sufficient, as all         tic dilemma. RadioGraphics 2004;24:1655-1674. https://doi.org/10.1148/
                                                                           rg.246045034
recurrences appeared in the first 12 months and which might be        10
                                                                           Rahbar R, Resto VA, Robson CD, et al. Nasal glioma and encephalocele:
related to incomplete resection in the cases reported.                     diagnosis and management. Laryngoscope 2003;113:2069-2077. https://
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Conflict of interest statement
                                                                      11
                                                                           Lamesch P, Froment N, Lamesch AJ. Nasal glioma. Report of a case
                                                                           and review of the literature. Pediatr Surg Int 1988;3:176-180. https://doi.
The authors declare no conflict of interest.                               org/10.1007/BF00182777
                                                                      12
                                                                           Shamseer L, Moher D, Clarke M, et al. PRISMA-P Group. Preferred re-
Funding                                                                    porting items for systematic review and meta-analysis protocols (PRISMA-
                                                                           P) 2015: elaboration and explanation. BMJ 2015;349:g7647. https://doi.
This research did not receive any specific grant from funding              org/10.1136/bmj.g7647
agencies in the public, commercial, or not-for-profit sectors.        13
                                                                           Dimov P, Rouev P, Tenev K, et al. Endoscopic surgery for the removal of a
                                                                           nasal glioma: case report. Otolaryngol Head Neck Surg 2001;124:690-690.
Author contributions                                                       https://doi.org/10.1177/019459980112400619
MGC: investigation, methodology, writing – original draft
                                                                      14
                                                                           Saettele M, Alexander A, Markovich B, et al. Congenital midline nasofron-
                                                                           tal masses. Pediatr Radiol 2012;42:1119-11125. https://doi.org/10.1007/
and editing. TM: resources. NG: supervision, writing-                      s00247-012-2409-z
review. SN: conceptualisation, methodology, supervision,              15
                                                                           Abdel Razek AAK, Gamaleldin OA, Elsebaie NA. Peripheral nerve sheath
writing – reviewing and editing.                                           tumors of head and neck: imaging-based review of World Health Organiza-
                                                                           tion Classification. J Comput Assist Tomogr 2020;44:928-940. https://doi.
                                                                           org/10.1097/RCT.0000000000001109
Ethical consideration                                                 16
                                                                           Tahlan K, Tanveer N, Kumar H, et al. A rare case of nasal glial heterotopia
We consulted the Ethikkomission Nordwest - und Zentral-                    in an infant. Aesthet Surg 2020;13:233-236. https://doi.org/10.4103/JCAS.
schweiz (EKNZ). According HFG Art. 3 our Case Report                       JCAS_148_19
                                                                      17
                                                                           Tetzlaff MT, Liu P, O’Malley BW Jr, et al. Report of a case of sinonasal
does not require an ethical aproval as the patient’s personal              undifferentiated carcinoma arising in a background of extensive nasal glio-
details were kept anonymous. The authors certify. that they                matosis. Head Neck 2008;30:549-555. https://doi.org/10.1002/hed.20707
have obtained all appropriate patient consent forms.                  18
                                                                           Boyer AC, Krishnan A, Goncalves LF, et al. Prenatal diagnosis of nasal
The systematic review of this article does not contain un-                 glioma associated with metopic craniosynostosis: case report and review
                                                                           of the literature. J Radiol Case Rep 2015;9:1-8. https://doi.org/10.3941/jrcr.
published human or animal trials.                                          v9i4.2179
                                                                      19
                                                                           Amin A, Monabati A, Kumar PV, et al. Nasal glioma (neuroglial het-
Data availability                                                          erotopia) mimicking an astrocytoma: case report. Ear Nose Throat J
                                                                           2005;84:657-658. https://doi.org/10.1177/014556130508401014
The references of all included cases as well as datasets gen-         20
                                                                           Schroth M,Wolf S, Bentzien SC, et al. Relapsing nasal glioma in a three-week-
erated and analysed during the present study are available                 old infant. Med Pediatr Oncol 2000;34:375-376. https://doi.org/10.1002/
from the corresponding author on reasonable request.                       (SICI)1096-911X(200005)34:5<375::AID-MPO15>3.0.CO;2-D




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