Extensive Pigmented Nevus Associated With Primary Melanoblastosis of Leptomeninges of Brain and Spinal Cord

Netherton, E. W.

doi:10.1001/archderm.1936.01470080034003

Cited by 21 publications

(3 citation statements)

References 15 publications

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“…Of the 18 previously reported, the authors had considered that malignant change had occurred in all but 6 cases (Rokitansky. 1861;Binsack, 1912;Van Bogaert, 1948;Netherton, 1936;Maclachlan, 1914;Jiitte, 1939). The malignant change may be diffuse or localized, and the criteria by which malignancy has been assessed vary.…”

Section: Discausionmentioning

confidence: 99%

Neuro-cutaneous Melanosis

Fox¹,

Emery²,

Goodbody³

et al. 1964

Archives of Disease in Childhood

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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.

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Section: Discausionmentioning

confidence: 99%

Neuro-cutaneous Melanosis

Fox¹,

Emery²,

Goodbody³

et al. 1964

Archives of Disease in Childhood

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“…NCM was first described by Rokitansky in 1861 (8), and at least 82 additional cases associated with CMN have been reported in the English language literature (1,5,9–63). The criteria for the diagnosis of NCM were redefined by Kadonga and Frieden in 1991 (5), and include large (greater than 20 cm in an adult; greater than 9 cm on the head or greater than 6 cm on the body in an infant) and/or multiple (more than two) CMN in association with the proliferation of melanocytes within the central nervous system (CNS).…”

Section: Discussionmentioning

confidence: 99%

Cerebral Mass Due to Neurocutaneous Melanosis: Eight Years Later

Schaffer

McNiff

Bolognia

2001

Pediatric Dermatology

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Neurocutaneous melanosis (NCM) is associated most commonly with giant congenital melanocytic nevi (CMN), in particular those on the scalp or in a posterior axial location that are accompanied by satellite congenital nevi. It also can occur in patients with multiple medium-sized CMN. In general, the prognosis of those with symptomatic NCM is poor, even in the absence of malignancy, while the prognosis of those with asymptomatic NCM detected via screening varies and is more difficult to predict. Herein we report an asymptomatic patient with a giant CMN and multiple satellite nevi who had a screening magnetic resonance imaging (MRI) study at age 5 months that showed a rounded area of increased signal in the right temporal lobe on T1-weighted images, suggestive of parenchymal melanosis. This melanotic mass was resected at age 10 months, and histologic examination of the surgical specimen showed prominent perivascular collections of benign, pigment-containing melanocytes within cerebral tissue. The patient remains healthy 8 years later. His excellent long-term outcome and other reports of NCM with localized central nervous system (CNS) involvement apparent on MRI may have implications for management, including early imaging of patients with high-risk CMN and potential surgical intervention for NCM.

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“…Hydrocephalus was the cause of death in five cases before their first year of life. Netherton (1936), Wilcox (1939) and Fanconi (1956) considered cerebral melanocytosis to be nevoid in character; others, on the basis of the cytology and infiltrative ability, are inclined to regard it as neoplastic. We have found no case of diffuse meningeal melanosis reported with melanotic nodules and involvement of both temporal bones, spheno-ethmoidal complex, pharynx and the viscera.…”

Section: Introductionmentioning

confidence: 99%