Neurocutaneous Melanosis

Yu, Hsin‐Su; Tsaur, Keh-Chang; Chien, Chun-Hsien; Perng, Jyi-Jiang; Lu, Cheng‐Chang

doi:10.1111/j.1346-8138.1985.tb01573.x

Cited by 20 publications

(4 citation statements)

References 4 publications

(2 reference statements)

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“…The helpful histological and radiological criteria for differentiation of benign meningeal lesions from malignant melanocytoma are: the existence of cellular anaplasia, necrosis and hemorrhage, cytoarchitecture (increased tendency of malignant cells to accumulate in nests or nodules) and the presence of associated edema [1, 30, 31, 32]. The characteristic MRI finding is the T 1 shortening of the involved structures (hyperintensity on T 1 -weighted images), which is due to the presence of melanin pigment [4, 5, 30, 33].…”

Section: Discussionmentioning

confidence: 99%

Neurocutaneous Melanosis Associated with Dandy-Walker Malformation

et al. 2000

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Neurocutaneous melanosis is a rare dysmorphogenesis associated with single or multiple giant pigmented cutaneous nevi and diffuse involvement of the leptomeninges anywhere in the central nervous system (CNS). It is interesting that almost 8–10% of patients had associated Dandy-Walker malformation in the literature, suggesting a common origin of the developmental abnormalities. In this article, we present a 2-year-old patient with neurocutaneous melanosis associated with Dandy-Walker malformation. We reviewed the literature and discuss the pathogenesis based on the preferred hypotheses so far.

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Section: Discussionmentioning

confidence: 99%

Neurocutaneous Melanosis Associated with Dandy-Walker Malformation

et al. 2000

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“…The pathogenesis of NCM is not clear. Melanocytes normally exist in human epidermis, hair bulb, leptomeninges, uveal tract and retina 26 , 30) , and are thought to be derived from multipotential precursor cells of the neural crest 19) . And these neural crest cells normally migrate to their final position in embryonic skin by day 50 of development 26) , and have been detected in fetal epidermis at 8-10 weeks and 5.5 months in the fetal meninges 14) .…”

Section: Discussionmentioning

confidence: 99%

“…The association of NCM with DWC are very rare and fewer than 30 cases have been reported. We reviewed the 26 literatures 2 , 4 , 6 , 7 , 8 , 9 , 10 , 12 , 13 , 14 , 16 , 17 , 19 , 20 , 21 , 22 , 23 , 24 , 25 , 26 , 27 , 28 , 29 , 30) , and our case. The definite mechanism of the concurrent NCM and DWC is still unknown.…”

Section: Discussionmentioning

confidence: 99%

“…Nine of 11 patients of pNCM with DWC were alive when they were reported. On the other hand, the prognosis of definite NCM (dNCM) with DWC was very poor 7 , 9 , 12 , 16 , 17 , 20 , 21 , 22 , 23 , 24 , 25 , 27 , 29 , 30) . The clinical symptoms of dNCM were more severe and the spinal involvement was more frequent 9 , 12 , 16 , 23 , 24) .…”

Section: Discussionmentioning

confidence: 99%

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Neurocutaneous Melanosis in Association with Dandy-Walker Complex with Extensive Intracerebral and Spinal Cord Involvement

Sung

Song

2014

J Korean Neurosurg Soc

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Neurocutaneous melanosis (NCM) is a rare congenital syndrome consisting of benign or malignant melanotic tumors of the central nervous system with large or numerous cutaneous melanocytic nevi. The Dandy-Walker complex (DWC) is characterized by an enlarged posterior fossa with high insertion of the tentorium, hypoplasia or aplasia of the cerebellar vermis, and cystic dilatation of the fourth ventricle. These each two conditions are rare, but NCM associated with DWC is even more rare. Most patients of NCM with DWC present neurological symptoms early in life such as intracranial hemorrhage, hydrocephalus, and malignant transformation of the melanocytes. We report a 14-year-old male patient who was finally diagnosed as NCM in association with DWC with extensive intracerebral and spinal cord involvement.

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Nevomelanocytic proliferations in the central nervous system of children

Reyes‐Múgica

Chou

Byrd

et al. 1993

Cancer

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Background. Melanocytic proliferations affecting the central nervous system (CNS) of children may be classified as meningeal melanocytosis, primary melanoma, or metastatic melanoma. Meningeal melanocytosis often is associated with giant congenital pigmented nevi (preferentially involving the midline, the head and neck) representing the lethal condition neurocutaneous melanocytosis. Primary or metastatic melanomas, although extremely rare in children, can occur in the brain and its coverings and are associated with a poor prognosis. Methods. A retrospective study of five patients with nevomelanocytic proliferations of the CNS was performed. Results. There was characteristic enhancement in the magnetic resonance imaging (MRI) with gadolinium contrast, abnormal cerebral spinal fluid (CSF) cytology with neoplastic cells showing cytoplasmic prolongations, and nevomelanocytic proliferation in the meninges expressing HMB45 positivity and exhibiting ultrastructural features of melanocytes. Conclusions. Because of the rarity of these lesions, awareness of their existence is crucial for their recognition. Clinical, radiologic, and cytologic, correlation may allow an opportune diagnosis, which would allow avoidance of brain biopsy. Melanin production is not restricted to melanocytic neoplasms, and other CNS tumoral lesions occasionally may feature melanin as part of their histologic findings.

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Neurocutaneous Melanosis

Cited by 20 publications

References 4 publications

Neurocutaneous Melanosis Associated with Dandy-Walker Malformation

Neurocutaneous Melanosis Associated with Dandy-Walker Malformation

Neurocutaneous Melanosis in Association with Dandy-Walker Complex with Extensive Intracerebral and Spinal Cord Involvement

Nevomelanocytic proliferations in the central nervous system of children

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