Multiple hairy pacinian neurofibromas (nerve-sheath myxomas)

Pacinian neurofibromas are unusual tumors with components that resemble Vater-Pacini corpuscles and are probably a variant of nerve-sheath myxoma. Lesions composed predominantly of these structures have occurred on or near the buttocks in three previously reported cases; however, all were solitary and congenital. Two of these patients, with lesions that closely resembled mature Vater-Pacini corpuscles, had underlying skeletal anomalies. Our patient had multiple hairy pacinian neurofibromas (nerve-sheath myxomas) on the buttocks that were not associated with radiographic evidence of and underlying skeletal anomaly. Skeletal anomalies may be associated with "sacrococcygeal paciniomas" but probably not with true pacinian neurofibromas.