Ectopic glioneuronal tissue in the middle cranial fossa region

Report of 4 cases

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The growth of ectopic glioneuronal tissue in the middle cranial fossa region is an uncommon event, with very few cases reported in the literature. In this paper the authors document 4 cases of ectopic glioneuronal tissue in the middle cranial fossa in children and briefly describe the clinical course and pathology. All of the children presented within the first 6 months of life. Two children presented with facial masses, 1 with airway obstruction, and another with proptosis of the right eye. Each child underwent a customized surgery dependent on the location and characteristics of the harbored lesion. Ectopic glioneuronal masses in the middle cranial fossa are rare and benign congenital tumors, and affected newborns can present with airway obstruction, feeding difficulties, and facial deformity depending on the lesion location. Determining an appropriate surgical approach and strategy is a significant challenge and may involve a multidisciplinary team of craniofacial plastic surgeons, otolaryngologists, and neurosurgeons. Although these lesions share clinical and anatomical similarities, because of their histopathological heterogeneity, it is unlikely that they represent a single pathological entity. The long-term outcome in these children is still unknown and is an area for future study. The pathogenesis of these lesions also remains unknown and may be revealed in future research.

Abbreviation used in this paper: GTR = gross-total resection.
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Contributor Notes

Address correspondence to: Anthony M. Avellino, M.D., M.B.A., Department of Neurological Surgery, Seattle Children's Hospital, 4800 Sand Point Way NE, Mailstop W-7729, Seattle, Washington 98105. email: anthony.avellino@seattlechildrens.org.
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