Neuro-cutaneous Melanosis

Abstract

Combined congenital abnormalities of the skin and central nervous system are well recognized-such conditions as Sturge-Weber syndrome, tuberous sclerosis, and neurofibromatosis. A very much rarer and less well-known member of this group is neurocutaneous melanosis. posterior fossa decompression was performed, and it was noted at operation that the meninges overlying the cerebellum were thickened and pigmented. The underlying cerebellar cortex also appeared to be pigmented. After operation the child renained well for eight weeks, but it was then noted that he was ataxic and though he was talking well he was emitting a shrill high-pitched cry at frequent intervals. The posterior fossa decompression was tense and bulging, and a Pudenz valve was therefore inserted into the right lateral ventricle draining into the right internal jugular vein. The child then remained fairly well, apart from some degree of ataxia, for a further period of four months, when he was readmitted with increasing restlessness, ataxia, and rigidity. The posterior fossa decompression was again tense and bulging, and at operation the Pudenz valve was found to be blocked by a protein coagulum, and it was replaced. Three days after the operation the child's conscious level began to decline:he developed left-sided Jacksonian epileptiform attacks and, despite repeated ventricular taps, died at 25 months.NECROPSY. There were no abnormal pathological findings outside of the skin and the central nervous system. In particular, no evidence of tumour was found in any other organ. The skin showed numerous large pigmented hairy naevi on the skin of the limbs, chest, trunk, back, and scalp. These varied in size, but ranged up to a maximum diameter of 6 cm.The dura appeared normal and showed no evidence of pigmentation, but the pia-arachnoid showed a diffusely. grey coloration most marked over the right cerebral hemisphere. In addition there were numerous scattered FiG.

Dural Melanoma Associated with Ocular Melanosis and Multiple Blue Nevi

“…36,[39][40][41]43,45 Intracranial meningeal melanomas have also been associated with neurocutaneous melanosis. 7 In this rare disorder, a giant hairy congenital nevus is associated with increased leptomeningeal melanosis, primarily of the pia arachnoid but with sparing of the dura. 7,46,47 It is remarkable that our patient remains disease-free seven years after her initial diagnosis.…”

“…7 In this rare disorder, a giant hairy congenital nevus is associated with increased leptomeningeal melanosis, primarily of the pia arachnoid but with sparing of the dura. 7,46,47 It is remarkable that our patient remains disease-free seven years after her initial diagnosis. It appears that primary dural melanoma follows a less aggressive course with patients surviving longer than those with leptomeningeal melanoma.…”

“…4 In the craniospinal axis, melanocytes are most frequent in the leptomeninges that occur predominantly around the ventral and lateral aspects of the spinal cord, the ventral aspect of the brain, cerebral peduncles, and sylvian fissure. [5][6][7][8] While some degree of leptomeningeal melanosis occurs in up to 85% of the population, heterotrophic melanin detected in the dura is rare. 9 Melanoma can arise anywhere melanoblasts are found, and it has been suggested dural melanoma originates from heterotrophic dural melanocytes.…”

Melanose neurocutânea. Relato de caso com melanoma maligno do sistema nervoso central

“…Na histopatologia, 2/3 das lesões são nevos melanocíticos intradérmicos e 1/3 nevos compostos 2,3 . As células névicas estendem-se à derme reticular e, ocasionalmente, até a hipoderme.…”

Giant congenital pigmented nevi of the face: Operative management and risk of malignancy

“…Al though it is well docu mented that gi ant pig mented nevi un dergo ma lig nant trans for ma tion, the in ci dence var ies in the lit era ture and has not been de ter mined pre cisely. The rela tion be tween gi ant con geni tal nevi of the face and lep tomen in geal mela noma is of par ticu lar clini cal im por tance (13)(14)(15)(16). We re viewed gi ant con geni tal pig mented nevi of the face to as sess the dis tri bu tion of the le sions and evalu ate our ex pe ri ence in their sur gi cal man age ment.…”