Cutaneous neural heterotopias and related tumors relevant for the dermatopathologist

Heterotopic neural tumors are rare in the skin; however, when encountered, they often pose a diagnostic problem. While the clinicopathologic features of most neuroaxis abnormalities are well-documented in the neuropathologic literature, their significance in cutaneous pathology as well as the associated nonneural dermatopathologic findings have not received enough attention. This report offers a comprehensive review of the most common cutaneous neural heterotopic abnormalities and their related tumors. The clinicopathologic features of the following entities along with their histogenetic considerations are discussed: classical and rudimentary meningocele, primary cutaneous meningioma, heterotopic brain tissue (nasal glioma), peripheral primitive neuroectodermal tumor, metastatic neuroblastoma, and ganglioneuroma. Familiarity with the associated dermatopathologic changes and with the differential diagnosis should assist in arriving at the correct diagnosis even without special training in dermatopathology or neuropathology.