Glial Choristoma in the Oral and Maxillofacial Region: A Clinicopathologic Study of 6 Cases

Abstract

Abstract Context.—Glial choristoma is an uncommon developmental abnormality typically presenting at birth or in early childhood. The nasal region is most frequently affected. Palate, tongue, cheek, scalp, and orbit can also be affected but these occurrences are relatively rare. Objective.—To report 6 cases of glial choristoma arising in the oral and maxillofacial region and to document the clinical and pathologic features of these lesions. Design.—Histologic and immunocytochemical examinations were performed on 6 cases of glial choristoma. Biologic behavior, prognosis, and pathogenesis were discussed together with a review of the literature. Result.—The patients included 5 boys and 1 girl. They all presented with the lesions at birth or soon after birth. Four lesions occurred on the dorsal side of the tongue, near the foramen caecum. One lesion was present in the infratemporal fossa and parapharyngeal space, and the other one was in the submandibular region. All patients received surgical excision, and follow-up data revealed no recurrence for a period of 10 months to 5 years after surgery. Histologically, the lesions showed mature glial cells intermixed with connective tissue. The glial tissue was strongly positive for glial fibrillary acidic protein and S100 but negative for neurofilament. Conclusion.—Glial choristoma should be classified as a developmental malformation that occurs in many sites of the head and neck. In oral cavity, the tongue is the most frequently affected site. Although these lesions are rare, they should be included in the differential diagnosis of congenital masses in the oral and maxillofacial region.