نتایج جستجو برای: neurocutaneous melanosis
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Neurocutaneous melanosis (NCM) is a rare nonfamilial syndrome and characterized by large or numerous congenital melanocytic nevi and excessive proliferation of melanin-containing cells in the leptomeninges. It is believed to be an embryonic neuroectodermal dysplasia. Patients with NCM may develop severe hydrocephalus and other neurological symptoms with extremely poor prognosis. We report an in...
Giant congenital melanocytic nevi (GCMN) are a rare occurrence. Gain-of-function mutation in the NRAS gene is found to be associated with GCMN, causing abnormal proliferation of embryonic melanoblasts. The two major complications associated with GCMN are malignant melanoma and neurocutaneous melanosis. Treatment of GCMN has conventionally been surgical. However, the role of NRAS inhibitors and ...
Background: This case report presents a very rare Dandy-Walker malformation (DWM) in association with a sporadic condition characterized by congenital melanocytic nevi and melanocytic thickening of the leptomeninges called Neurocutaneous melanosis (NCM). The DWM is a rare congenital disorder characterized by enlarged posterior fossa and a cystic enlargement of the four...
made in our patient because of the presence of highly characteristic melanin deposits in the MRI study. This is the only method to diagnose CNS melanosis in a living patient. Secondly, in our patient there were no clinical indicators suggestive of a developing cutaneous melanoma, viz. changes in colour, size, shape, rapid growth rate, proliferative nodules or ulceration, in any of the GCMN. Hen...
Introduction Meningeal melanocytomas are rare tumors of the CNS that develop from melanocytes that are present in leptomeninges, with differing pigmented appearance. They generally occur in the posterior fossa and the spinal cord. This lesion may manifest at any age, but most patients are in the fifth decade of life. Occasionally, these tumors appear in a complex neurocutaneous grouping with ot...
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