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

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.

Nasopharyngeal neuroglial heterotopy ‐ Choristoma: Case report and review of the literature

“…Other theory is the displacement of neuroectodermal cells during an early stage of embryogenesis, which may subsequently differentiate into a variety of neuroglial tissue. 2,4,11 The third theory is that the lesion is a neoplasm, 4 but this one is not supported by most authors.…”

“…It is a very rare entity, but the most common location in the oral and maxillofacial region is the nasal area, followed by the palate, tongue, cheek, scalp and orbit. 2,3 All type of choristomas have been described, more commonly in females, including heterotopic central nervous system tissue choristomas. 4,5 However, some cases have been described in males.…”

“…4,5 However, some cases have been described in males. 2,3,6 There are different types of choristomas, including glial, gastric, hairy polyps, among others depending on the type of mature cells that make up the tumor. 7e10 Choristomas are masses with growth rates similar to that of the surrounding tissue with no tumor like behavior, therefore they should be considered a malformation and not a neoplasm.…”

“…These cystic lesions encapsulate fluid similar to cerebrospinal fluid that might result from the production of functioning choroid plexus. 2 There are three theories of the pathogenesis of glial choristomas. One is the protrusion of the neural glial tissue from the developing cerebral tissue, Neuroglial heterotopy -Choristoma which becomes isolated from the brain.…”

“…If this defect is present, an MRI is helpful to determine an intracranial connection. 2 Despite the usual relation to a bony skull base bony defect, choristomas by definition do not communicate with the subarachnoid space. 6 On MRI the lesion shows isointense signal in T1WI and slight hyperintense signal on T2WI, as the normal brain.…”