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Transsphenoidal extension of heterotopic glioneuronal tissue: pathoanatomic considerations in symptomatic neonates

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Abstract

Purpose

In this clinical investigation, we aimed (1) to re-evaluate the nature of glioneuronal tissue with transsphenoidal extension and how it fits into the nomenclature of midline malformations and mass lesions; (2) to find out if our imaging findings support current pathoanatomic concepts of clefts and canals in the sphenoid body of newborns.

Methods

In two neonates with respiratory distress due to nasopharyngeal masses, 3T MRI was performed, and CT in one of them. Imaging features were analyzed in consensus by two pediatric neuroradiologists with histological reports being available. An interdisciplinary panel compared the findings to those of case publications and differential entities from our institutional case collection.

Results

Referring to our rare case of transsphenoidal cerebral heterotopia and unique case of hypothalamic hamartoma with transsphenoidal herniation, glioneuronal heterotopia may definitely extend through the sphenoid bone. Consequently, there is reason for brain heterotopias to be labeled as such also in case of an intracranial component. Connection between heterotopic glioneuronal tissue in the nasopharynx and a hypothalamic hamartoma may go along with indistinct margins to normal brain. Neither extension through a transsphenoidal cleft nor association with a cleft palate are specific for cerebral heterotopia. Our findings support the hypothesis that transsphenoidal cerebral heterotopias do not or at least not invariably follow the route of Rathke's pouch, known as the craniopharyngeal canal.

Conclusion

Transsphenoidal glioneuronal heterotopia should be the top differential diagnosis in MR imaging if a non-enhancing nasopharyngeal mass of an infant extends through a craniopharyngeal cleft within the intersphenoid synchondrosis.

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References

  1. Huisman TA, Schneider JF, Kellenberger CJ, Martin-Fiori E, Willi UV, Holzmann D (2004) Developmental nasal midline masses in children: neuroradiological evaluation. Eur Radiol 14:243–249

    Article  PubMed  Google Scholar 

  2. Laine FJ, Nadel L, Braun IF (1990) CT and MR imaging of the central skull base. Part 1: techniques, embryologic development, and anatomy. Radiographics 10:591–602

    PubMed  CAS  Google Scholar 

  3. Nemzek WR, Brodie HA, Hecht ST, Chong BW, Babcook CJ, Seibert JA (2000) MR, CT, and plain film imaging of the developing skull base in fetal specimens. AJNR Am J Neuroradiol 21:1699–1706

    PubMed  CAS  Google Scholar 

  4. Kjaer I, Russell BG (1995) The craniopharyngeal canal indicating the presence of pharyngeal adenopituitary tissue. Eur J Radiol 20:212–214

    Article  PubMed  CAS  Google Scholar 

  5. Sadler TW (2004) Langman's essential medical embryology. Lippincott Williams and Wilkins, Philadelphia

    Google Scholar 

  6. Currarino G, Maravilla KR, Salyer KE (1985) Transsphenoidal canal (large craniopharyngeal canal) and its pathologic implications. AJNR Am J Neuroradiol 6:39–43

    PubMed  CAS  Google Scholar 

  7. Arey LB (1950) The craniopharyngeal canal reviewed and reinterpreted. Anat Rec 106:1–16

    Article  PubMed  CAS  Google Scholar 

  8. Madeline LA, Elster AD (1995) Postnatal development of the central skull base: normal variants. Radiology 196:757–763

    PubMed  CAS  Google Scholar 

  9. Hughes ML, Carty AT, White FE (1999) Persistent hypophyseal (craniopharyngeal) canal. Br J Radiol 72:204–206

    PubMed  CAS  Google Scholar 

  10. Chen CJ (2001) Suprasellar and infrasellar craniopharyngioma with a persistent craniopharyngeal canal: case report and review of the literature. Neuroradiology 43:760–762

    Article  PubMed  CAS  Google Scholar 

  11. Ekinci G, Kiliç T, Baltacioğlu F, Elmaci I, Altun E, Pamir MN, Erzen C (2003) Transsphenoidal (large craniopharyngeal) canal associated with a normally functioning pituitary gland and nasopharyngeal extension, hyperprolactinemia, and hypothalamic hamartoma. AJR Am J Roentgenol 180:76–77

    PubMed  Google Scholar 

  12. Severino M, Schwartz ES, Thurnher MM, Rydland J, Nikas I, Rossi A (2010) Congenital tumors of the central nervous system. Neuroradiology 52:531–548

    Article  PubMed  Google Scholar 

  13. 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  PubMed  Google Scholar 

  14. Newman NJ, Miller NR, Green WR (1986) Ectopic brain in the orbit. Ophthalmology 93:268–272

    PubMed  CAS  Google Scholar 

  15. Taege C, Musil A, Klohs G, Rath FW (2001) Cerebral heterotopia appearing as an extranasal polyp. Eur J Pediatr Surg 11:268–270

    Article  PubMed  CAS  Google Scholar 

  16. Ruff T, Diaz JA (1986) Heterotopic brain in the nasopharynx. Otolaryngol Head Neck Surg 94:254–256

    PubMed  CAS  Google Scholar 

  17. Cohen AH, Abt AB (1970) An unusual cause of neonatal respiratory obstruction: heterotopic pharyngeal brain tissue. J Pediatr 76:119–122

    Article  PubMed  CAS  Google Scholar 

  18. Ferrer I, Galofré E, Soler T (1986) Structure of an isolated cerebellum and related nuclei developed within the matrix of a mature ovarian teratoma. Childs Nerv Syst 2:266–269

    PubMed  CAS  Google Scholar 

  19. Takhtani D, Melhem ER, Carson BS (2000) A heterotopic cerebellum presenting as a suprasellar mass with associated nasopharyngeal teratoma. AJNR Am J Neuroradiol 21:1119–1121

    PubMed  CAS  Google Scholar 

  20. Patterson K, Kapur S, Chandra RS (1986) “Nasal gliomas” and related brain heterotopias: a pathologist's perspective. Pediatr Pathol 5:353–362

    Article  PubMed  CAS  Google Scholar 

  21. Yeoh GP, Bale PM, de Silva M (1989) Nasal cerebral heterotopia: the so-called nasal glioma or sequestered encephalocele and its variants. Pediatr Pathol 9:531–549

    Article  PubMed  CAS  Google Scholar 

  22. Lammens M (2000) Neuronal migration disorders in man. Eur J Morphol 38:327–333

    Article  PubMed  CAS  Google Scholar 

  23. Nakamura T, Kakinuma H, Imaku M, Takahashi H, Nojima T, Kumano K, Iizuka H (1998) A large lateral parapharyngeal heterotopic brain tissue extending into the intracranial area. J Child Neurol 13:518–520

    Article  PubMed  CAS  Google Scholar 

  24. Sidebottom AJ, Begley A, May P, Richardson D (2000) Aetiology of nasopharyngeal glioma. Int J Oral Maxillofac Surg 29:210–211

    Article  PubMed  CAS  Google Scholar 

  25. Celedin S, Kau T, Gasser J, Kraschl R, Sinzig M (2010) Fetal MRI of a hypothalamic hamartoma in Pallister-Hall syndrome. Pediatr Neurol 42:59–60

    Article  PubMed  Google Scholar 

  26. Prabhu VC, Brown HG (2005) The pathogenesis of craniopharyngiomas. Childs Nerv Syst 21:622–627

    Article  PubMed  Google Scholar 

  27. Wen L, Hu LB, Feng XY, Desai G, Zou LG, Wang WX, Zhang D (2010) Rathke's cleft cyst: clinicopathological and MRI findings in 22 patients. Clin Radiol 65:47–55

    Article  PubMed  CAS  Google Scholar 

  28. Toyoshima Y, Kakita A, Yamada M, Sato G, Mori H, Okamoto K, Tanaka R, Takahashi H (2003) Extracerebral ectopic mass with huge cysts in the anterior cranial cavity. Neuropathology 23:301–306

    Article  PubMed  Google Scholar 

  29. Harris CP, Townsend JJ, Klatt EC (1994) Accessory brains (extracerebral heterotopias): unusual prenatal intracranial mass lesions. J Child Neurol 9:386–389

    Article  PubMed  CAS  Google Scholar 

  30. Muzumdar D, Michaud J, Ventureyra EC (2006) Anterior cranial base glioneuronal heterotopia. Childs Nerv Syst 22:227–233

    Article  PubMed  Google Scholar 

  31. Uemura T, Yoshikawa A, Onizuka T, Hayashi T (1999) Heterotopic nasopharyngeal brain tissue associated with cleft palate. Cleft Palate Craniofac J 36:248–251

    Article  PubMed  CAS  Google Scholar 

  32. Franco D, Alonso N, Ruas R, da Silva FR, Franco T (2009) Transsphenoidal meningoencephalocele associated with cleft lip and palate: challenges for diagnosis and surgical treatment. Childs Nerv Syst 25:1455–1458

    Article  PubMed  Google Scholar 

  33. Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, Burger PC, Jouvet A, Scheithauer BW, Kleihues P (2007) The 2007 WHO classification of tumours of the central nervous system. Acta Neuropathol 114:97–109

    Article  PubMed  Google Scholar 

  34. Lee YH, Park EK, Park YS, Shim KW, Choi JU, Kim DS (2009) Treatment and outcomes of primary intracranial teratoma. Childs Nerv Syst 25:1581–1587

    Article  PubMed  Google Scholar 

  35. Régis J, Scavarda D, Tamura M, Nagayi M, Villeneuve N, Bartolomei F, Brue T, Dafonseca D, Chauvel P (2006) Epilepsy related to hypothalamic hamartomas: surgical management with special reference to gamma knife surgery. Childs Nerv Syst 22:881–895

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Pediatric Radiology, Section of Neuroradiology, University Children’s Hospital Zurich, Steinwiesstrasse 75, 8032, Zurich, Switzerland

    Thomas Kau, Heinrich Schiegl, Christian J. Kellenberger & Ianina Scheer

  2. Department of Surgery, Section of Otorhinolaryngology, University Children’s Hospital Zurich, Steinwiesstrasse 75, 8032, Zurich, Switzerland

    Claudine Gysin

  3. Department of Neuropathology, University Hospital Zurich, Ramistrasse 100, 8091, Zurich, Switzerland

    Hildegard Dohmen-Scheufler

  4. Department of Intensive Care and Neonatology, University Children’s Hospital Zurich, Steinwiesstrasse 75, 8032, Zurich, Switzerland

    Barbara Brotschi

  5. Department of Pediatric Neurology, University Children’s Hospital Zurich, Steinwiesstrasse 75, 8032, Zurich, Switzerland

    Eugen Boltshauser

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  1. Thomas Kau
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  2. Claudine Gysin
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  3. Hildegard Dohmen-Scheufler
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  8. Ianina Scheer
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Correspondence to Thomas Kau.

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Kau, T., Gysin, C., Dohmen-Scheufler, H. et al. Transsphenoidal extension of heterotopic glioneuronal tissue: pathoanatomic considerations in symptomatic neonates. Childs Nerv Syst 27, 771–778 (2011). https://doi.org/10.1007/s00381-010-1326-4

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  • DOI: https://doi.org/10.1007/s00381-010-1326-4

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