"Nasal gliomas" and related brain heterotopias: a pathologist's perspective

Abstract

Brain heterotopias are rare congenital malformations embryologically related to encephaloceles. They present as a mass in or about the nose (nasal glioma) or in the nasopharynx. We present the clinical and pathological features of 5 cases of heterotopic brain tissue. Four nasal gliomas consisted of mature neuroglial tissue, including neurons in 2 cases, embedded in a fibrovascular stroma. A nasopharyngeal brain heterotopia showed histologic features of mature neuroglial tissue including neurons and ependymal-lined cystic structures. The finding of mature neuroglial tissue in a mass from the head and neck region raises three differential diagnostic possibilities: teratoma, encephalocele, or heterotopic tissue. A teratoma can be ruled out by examination of the entire specimen. Encephaloceles and brain heterotopias can be distinguished only after correlation with the patient's clinical and radiologic findings.