Once a patient with<unk>  becomes symptomatic, little can be done to improve prognosis as there is no effective treatment for the disorder. Most therapies are designed to treat the symptoms associated with the disorder, mainly those related to hydrocephalus. A ventriculoperitoneal shunt to relieve intracranial pressure is the preferred method. Chemotherapy and radiotherapy have been shown to be ineffective in cases of<unk>  where malignancy is present. Additionally, due to the total infiltration of the central nervous system by these lesions, surgical resection is not a viable treatment option. It has been demonstrated that early embryonic, post-zygotic somatic mutations in the NRAS gene are implicated in the pathogenesis of<unk> . Recently, experimental treatment with MEK162, a MEK inhibitor, has been tried in a patient with<unk>  and progressive symptomatic leptomeningeal melanocytosis. Pathological studies with immunohistochemical and Western Blot analyses using Ki67 and pERK antibodies showed a potential effect of MEK inhibiting therapy. Further studies are needed to determine whether MEK inhibitors can effectively target NRAS-mutated symptomatic<unk> .
Neurocutaneous melanosis
Nevus anemicus
Phakomatosis pigmentokeratotica
Granulomatous slack skin
Blue nevus
Erythema dyschromicum perstans
Sparganosis
Hypertrichosis cubiti