Neurocutaneous Melanosis in Infancy: Always a Dismal Prognosis?

Özet

AIM: To analyze the current literature on neurocutaneous melanosis (NCM), as well as, our cases, in order to better define theprognosis and the presence of risk factors affecting it, thus, offering better information to the parents.MATERIAL and METHODS: Two cases observed at the Pediatric Neurosurgery Unit of the Catholic University Medical School inRome are described. Both of them had cutaneous stigmata and cerebral MR evidence of intracranial melanin deposits. These twochildren showed a very different clinical course.RESULTS: The present study enlighten the differences among the two cases and review the literature on the subject, with theattempt to understand which are clinical and disease related factors that might influence the prognosis.CONCLUSION: Beside malignant features of cutaneous melanotic lesions, the presence of hydrocephalus at diagnosis and theearly appearance of clinical symptoms, when appearing contemporarily, are predicting the rapid progression of the disease and aworse prognosis.