Neurofibromatosis and neural crest neoplasms: primary acquired melanosis and malignant melanoma of the conjunctiva.

With an occurrence of approximately 1 in 3000 births, von Recklinghausen neurofibromatosis (NF) is one of the most common inherited human disorders. NF is considered a neurocristopathy, a disorder of neural crest derived cells. One of the complications of NF is the development of neural crest derived malignancies such as malignant schwannoma, pheochromocytoma, and malignant melanoma of the skin and choroid. The case history of a patient with NF and conjunctival malignant melanoma which developed in an eye with primary acquired melanosis is yet another example of a neural crest malignancy developing in a NF patient.