Heterotopic neuroglial tissue of the face: Report of six cases and review of the literature

Abstract

We report six cases of heterotopic neuroglial tissue of the face. All cases appeared at birth with a laterofacial mass and, in most cases, a respiratory disorder. The initial diagnosis has frequently been lymphangioma. But the rapidly growing character of the mass with resistance to high dose corticoid therapy, the fluid aspect at computerized tomography imaging with no connection to the central nervous system, and the presence of cerebrospinal fluid at the needle puncture were the most helpful features in narrowing the differential diagnoses. Patients were treated by surgical removal of the mass. The pathologic study showed the presence of neuroglial tissue. The management of the patients is discussed. The thirteen previous cases reported in the literature and our six cases lead to a discussion on the embryologic origin of this rare entity—the cephalic neural crest.