Embryology of Neurocutaneous Syndromes

摘要: The neurocutaneous diseases and syndromes are a broad group of congenital disorders with diverse genetic, clinical pathological features that share in common developmental lesions the skin central peripheral nervous systems. Subcutaneous systemic involvement is common. In many these conditions another feature tendency to develop tumors multiple sites body. Many hamartomatous nature, produce benign tumors, but patients also may malignant tumors. etiology has been identified several conditions: such cases properly referred as diseases. case neurofibromatosis tuberous sclerosis complex, neoplasias can be explained because genes responsible for disease tumor-suppressor genes. manifest overgrowth one area, region or side body, usually progressive (Cohen et al. 2001). Asymmetry hemimegalencephaly (Flores-Sarnat 2002). present at birth become later.