Skip to main content

Advertisement

SpringerLink
Log in

Histopathologic features of nasal glial heterotopia (nasal glioma)

  • Original Article
  • Published:
Child's Nervous System Aims and scope Submit manuscript

Abstract

Purpose

Nasal glioneuronal heterotopia (NGH) is an uncommon developmental abnormality of the nasal cavity or paranasal soft tissue. Few detailed histologic studies of NGH exist, and molecular analyses have not been performed to date.

Methods

We describe six cases of pediatric NGH and two representative encephaloceles encountered in our practice over the past 20 years.

Results

Two clinically distinct patient groups were noted, those with 1) intranasal nasal cavity mass (n = 3), or 2) extranasal cutaneous mass on the nose (n = 3, 1 on nasal apex, 2 on nasal bridge). Intranasal cases presented within the first week of life, whereas the extranasal NGH presented at ages of 4, 7, and 8 months. Resection was curative in 5/6 cases, with a single case showing local recurrence. Histologic examination showed a predominantly glial cell composition, with nests of GFAP-immunoreactive neuropil containing large, often multinucleated astrocytes. Neurons, although difficult to identify on H&E-stains, were readily observed in all cases by NeuN-immunostain. At least focal leptomeninges were noted in 2/3 intranasal and 1/3 extranasal NGHs on routine histology, SSTR2A immunohistochemistry further confirmed leptomeninges/ arachnoid cells in 4/6 cases. 1 of 4 NGH (extranasal) cases showed copy number variations in chromosome 16, 17 and 19, which were also present in 1/2 encephalocele cases. The full significance of these alterations remains unknown.

Conclusion

We find evidence of histologic overlap between NGH and encephalocele, and, for the first time, report molecular alterations shared between the two entities, suggesting that these conditions may represent spectrum of the same histopathologic entity.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6

Similar content being viewed by others

Data availability

Data sharing is not applicable to this article as no new data were created or analyzed in this study.

Code availability

Not applicable.

References

  1. Cheung D, Woodruff GH, Brown L, Sampath RG (2005) Extranasal nasal glioma. Eye (Lond) 19:239–240.https://www.ncbi.nlm.nih.gov/pubmed/15286677

  2. Riffaud L, Ndikumana R, Azzis O, Cadre B (2008) Glial heterotopia of the face. J Pediatr Surg 43:e1–3.https://www.ncbi.nlm.nih.gov/pubmed/19040912

  3. Husein OF, Collins M, Kang DR (2008) Neuroglial heterotopia causing neonatal airway obstruction: presentation, management, and literature review. Eur J Pediatr 167:1351–1355.https://www.ncbi.nlm.nih.gov/pubmed/18758813

  4. Rahbar R, Resto VA, Robson CD, Perez-Atayde AR, Goumnerova LC, McGill TJ, Healy GB (2003) Nasal glioma and encephalocele: diagnosis and management. Laryngoscope 113:2069–2077.https://www.ncbi.nlm.nih.gov/pubmed/14660905

  5. Penner CR, Thompson L (2003) Nasal glial heterotopia: a clinicopathologic and immunophenotypic analysis of 10 cases with a review of the literature. Ann Diagn Pathol 7:354–359.https://www.ncbi.nlm.nih.gov/pubmed/15018118

  6. Amin A, Monabati A, Kumar PV, Hashemi SB (2005) Nasal glioma (neuroglial heterotopia) mimicking an astrocytoma: case report. Ear Nose Throat J 84:657–658.https://www.ncbi.nlm.nih.gov/pubmed/16382748

  7. Kobow K, Jabari S, Pieper T, Kudernatsch M, Polster T, Woermann FG, Kalbhenn T, Hamer H, Rossler K, Muhlebner A, Spliet WGM, Feucht M, Hou Y, Stichel D, Korshunov A, Sahm F, Coras R, Blumcke I, von Deimling A (2020) Mosaic trisomy of chromosome 1q in human brain tissue associates with unilateral polymicrogyria, very early-onset focal epilepsy, and severe developmental delay. Acta Neuropathol 140:881–891.https://www.ncbi.nlm.nih.gov/pubmed/32979071

  8. Lee JH, Huynh M, Silhavy JL, Kim S, Dixon-Salazar T, Heiberg A, Scott E, Bafna V, Hill KJ, Collazo A, Funari V, Russ C, Gabriel SB, Mathern GW, Gleeson JG (2012) De novo somatic mutations in components of the PI3K-AKT3-mTOR pathway cause hemimegalencephaly. Nat Genet 44:941–945.https://www.ncbi.nlm.nih.gov/pubmed/22729223

  9. (DKTK) GCfTCR. DNA methylation-based classification of central nervous system tumors. Reference set (classifier version: 11b2.https://www.molecularneuropathology.org/mnp/classifier/1

  10. Capper D, Jones DTW, Sill M, Hovestadt V, Schrimpf D, Sturm D, Koelsche C, Sahm F, Chavez L, Reuss DE, Kratz A, Wefers AK, Huang K, Pajtler KW, Schweizer L, Stichel D, Olar A, Engel NW, Lindenberg K, Harter PN, Braczynski AK, Plate KH, Dohmen H, Garvalov BK, Coras R, Holsken A, Hewer E, Bewerunge-Hudler M, Schick M, Fischer R, Beschorner R, Schittenhelm J, Staszewski O, Wani K, Varlet P, Pages M, Temming P, Lohmann D, Selt F, Witt H, Milde T, Witt O, Aronica E, Giangaspero F, Rushing E, Scheurlen W, Geisenberger C, Rodriguez FJ, Becker A, Preusser M, Haberler C, Bjerkvig R, Cryan J, Farrell M, Deckert M, Hench J, Frank S, Serrano J, Kannan K, Tsirigos A, Bruck W, Hofer S, Brehmer S, Seiz-Rosenhagen M, Hanggi D, Hans V, Rozsnoki S, Hansford JR, Kohlhof P, Kristensen BW, Lechner M, Lopes B, Mawrin C, Ketter R, Kulozik A, Khatib Z, Heppner F, Koch A, Jouvet A, Keohane C, Muhleisen H, Mueller W, Pohl U, Prinz M, Benner A, Zapatka M, Gottardo NG, Driever PH, Kramm CM, Muller HL, Rutkowski S, von Hoff K, Fruhwald MC, Gnekow A, Fleischhack G, Tippelt S, Calaminus G, Monoranu CM, Perry A, Jones C, Jacques TS, Radlwimmer B, Gessi M, Pietsch T, Schramm J, Schackert G, Westphal M, Reifenberger G, Wesseling P, Weller M, Collins VP, Blumcke I, Bendszus M, Debus J, Huang A, Jabado N, Northcott PA, Paulus W, Gajjar A, Robinson GW, Taylor MD, Jaunmuktane Z, Ryzhova M, Platten M, Unterberg A, Wick W, Karajannis MA, Mittelbronn M, Acker T, Hartmann C, Aldape K, Schuller U, Buslei R, Lichter P, Kool M, Herold-Mende C, Ellison DW, Hasselblatt M, Snuderl M, Brandner S, Korshunov A, von Deimling A, Pfister SM (2018) DNA methylation-based classification of central nervous system tumours. Nature 555:469–474.https://www.ncbi.nlm.nih.gov/pubmed/29539639

  11. Capper D, Stichel D, Sahm F, Jones DTW, Schrimpf D, Sill M, Schmid S, Hovestadt V, Reuss DE, Koelsche C, Reinhardt A, Wefers AK, Huang K, Sievers P, Ebrahimi A, Scholer A, Teichmann D, Koch A, Hanggi D, Unterberg A, Platten M, Wick W, Witt O, Milde T, Korshunov A, Pfister SM, von Deimling A (2018) Practical implementation of DNA methylation and copy-number-based CNS tumor diagnostics: the Heidelberg experience. Acta Neuropathol 136:181–210.https://www.ncbi.nlm.nih.gov/pubmed/29967940

  12. Anderson WJ, Doyle LA (2021) Updates from the 2020 World Health Organization Classification of Soft Tissue and Bone Tumours. Histopathology 78:644–657.https://www.ncbi.nlm.nih.gov/pubmed/33438273

  13. Kriho VK, Yang HY, Moskal JR, Skalli O (1997) Keratin expression in astrocytomas: an immunofluorescent and biochemical reassessment. Virchows Arch 431:139–147

    Article  CAS  Google Scholar 

  14. Pekmezci M, Villanueva-Meyer JE, Goode B, Van Ziffle J, Onodera C, Grenert JP, Bastian BC, Chamyan G, Maher OM, Khatib Z, Kleinschmidt-DeMasters BK, Samuel D, Mueller S, Banerjee A, Clarke JL, Cooney T, Torkildson J, Gupta N, Theodosopoulos P, Chang EF, Berger M, Bollen AW, Perry A, Tihan T, Solomon DA (2018) The genetic landscape of ganglioglioma. Acta Neuropathologica Communications 6:47.https://doi.org/10.1186/s40478-018-0551-z

  15. Penner CR, Thompson LD (2003) Nasal glial heterotopia: a clinicopathologic and immunophenotypic analysis of 10 cases with a review of the literature. Ann Diagn Pathol 7:354–359

    Article  Google Scholar 

  16. Machi H, Karata H, Yamane Y, Fukuoka J, Funakoshi Y, Moriuchi H (2017) A Neonatal Case of Glial Choristoma of the Tongue Causing Airway Obstruction. Case Rep Otolaryngol 2413035.https://www.ncbi.nlm.nih.gov/pubmed/29085690

  17. Ramadass T, Narayanan N, Rao P, Parameswaran A (2011) Glial Heterotopia in ENT-Two Case Reports and Review of Literature. Indian J Otolaryngol Head Neck Surg 63:407–410.https://www.ncbi.nlm.nih.gov/pubmed/23024956

  18. Peris-Celda M, Giannini C, Diehn FE, Eckel LJ, Neff BA, Van Gompel JJ (2018) Glioneuronal Heterotopia Presenting as Cerebellopontine Angle Tumor of Cranial Nerve VIII. World Neurosurg 114:289–292.https://www.ncbi.nlm.nih.gov/pubmed/29625310

  19. Ozolek JA, Losee JE, Lopes TC, Galambos C (2005) Temporal Soft Tissue Glioneuronal Heterotopia in a Child with a Seizure Disorder: Case Report and Review of the Literature. Pediatr Dev Pathol 8:673–679

    Article  Google Scholar 

  20. Schulz S, Pauli SU, Schulz S, Händel M, Dietzmann K, Firsching R, Höllt V (2000) Immunohistochemical determination of five somatostatin receptors in meningioma reveals frequent overexpression of somatostatin receptor subtype sst2A. Clin Cancer Res 6:1865–1874

    CAS  PubMed  Google Scholar 

  21. Fujioka M, Tasaki I, Nakayama R, Yakabe A, Baba H, Toda K, Itoh M, Hirano A (2006) Both Nasal Cerebral Heterotopia and Encephalocele in the Same Patient. Cleft Palate Craniofac J 43:112–116

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Pathology, University of Colorado, Children’s Hospital Colorado, Aurora, CO, USA

    A. Gilani

  2. Departments of Pathology, Neurology, Neurosurgery, University of Colorado, Aurora, CO, USA

    B. K. Kleinschmidt-DeMasters

Authors
  1. A. Gilani
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. B. K. Kleinschmidt-DeMasters
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

Ahmed Gilani, Acquisition, analysis, and interpretation of data for the work; drafting, revising, and critical review; B.K. Kleinschmidt-DeMasters, Acquisition, analysis, and interpretation of data for the work; revising and critical review.

Corresponding author

Correspondence to A. Gilani.

Ethics declarations

Conflict of interest

The authors have no conflicts of interest to declare that are relevant to the content of this article.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Presented as poster presentation at the annual meeting of the American association of neuropathologists, June 2021.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Gilani, A., Kleinschmidt-DeMasters, B.K. Histopathologic features of nasal glial heterotopia (nasal glioma). Childs Nerv Syst 38, 63–75 (2022). https://doi.org/10.1007/s00381-021-05369-4

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00381-021-05369-4

Keywords

Search

Navigation