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.
Subject(s)
Choristoma/congenital , Facial Neoplasms/congenital , Neoplasms, Germ Cell and Embryonal/congenital , Neoplasms, Nerve Tissue/congenital , Neuroglia , Child , Choristoma/complications , Choristoma/diagnosis , Diagnosis, Differential , Facial Neoplasms/complications , Facial Neoplasms/diagnosis , Female , Head and Neck Neoplasms/complications , Head and Neck Neoplasms/congenital , Head and Neck Neoplasms/diagnosis , Humans , Infant , Infant, Newborn , Lymphangioma/diagnosis , Male , Neoplasms, Germ Cell and Embryonal/complications , Neoplasms, Germ Cell and Embryonal/diagnosis , Neoplasms, Nerve Tissue/complications , Neoplasms, Nerve Tissue/diagnosis , Neural Crest , Respiration Disorders/etiologySubject(s)
Choristoma/pathology , Neoplasms, Nerve Tissue/congenital , Palatal Neoplasms/congenital , Palate, Soft/pathology , Tongue Neoplasms/congenital , Female , Humans , Infant , Infant, Newborn , Neoplasms, Nerve Tissue/pathology , Neuroglia , Palatal Neoplasms/pathology , Tongue Neoplasms/pathologyABSTRACT
Two well differentiated pure Paciniomas, bearing no resemblance to neurofibromas, were resected from infants with spinal deformities. Each was in the form of a thick cord running from a sacral dimple through a low occult spina bifida to the spinal dura. The lesions are considered to be a malformation or hamartomatous overgrowth. Control sacrococcygeal regions from necropsies on 15 infants were examined histologically, and Pacinian corpuscles were found in 7, usually near the tip of the coccyx, and never numerous.
