Adult-onset neurocutaneous melanosis with Dandy–Walker malformation
Neurocutaneous melanosis (NCM) is characterized by diffuse or focal proliferation of melanin-producing cells over the skin or leptomeninges. Even without malignant transformation, the prognosis is poor after symptomatic progression of NCM, resulting from either mass effect in central nervous system...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
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Wolters Kluwer Health/LWW
2018-01-01
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| Series: | Formosan Journal of Surgery |
| Subjects: | |
| Online Access: | http://www.e-fjs.org/article.asp?issn=1682-606X;year=2018;volume=51;issue=3;spage=118;epage=121;aulast=Chang |
| _version_ | 1827843077421662208 |
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| author | Ting-Wei Chang Po-Chuan Hsieh Zhuo-Hao Liu Po-Hsun Tu |
| author_facet | Ting-Wei Chang Po-Chuan Hsieh Zhuo-Hao Liu Po-Hsun Tu |
| author_sort | Ting-Wei Chang |
| collection | DOAJ |
| description | Neurocutaneous melanosis (NCM) is characterized by diffuse or focal proliferation of melanin-producing cells over the skin or leptomeninges. Even without malignant transformation, the prognosis is poor after symptomatic progression of NCM, resulting from either mass effect in central nervous system or hydrocephalus. We reported a 26-year-old female patient with rapid deterioration after the onset of NCM. Despite no solid tumor formation, elevated cerebrospinal fluid protein content, which complicated shunting procedure, associated with hydrocephalus played the key role in clinical deterioration. Adult onset of NCM with Dandy–Walker malformation was never reported in our literature review. Diagnosis and treatment for such disease entity are discussed. |
| first_indexed | 2024-03-12T08:20:33Z |
| format | Article |
| id | doaj.art-376a7bf9705a469992a1cb134bc680d5 |
| institution | Directory Open Access Journal |
| issn | 1682-606X |
| language | English |
| last_indexed | 2024-03-12T08:20:33Z |
| publishDate | 2018-01-01 |
| publisher | Wolters Kluwer Health/LWW |
| record_format | Article |
| series | Formosan Journal of Surgery |
| spelling | doaj.art-376a7bf9705a469992a1cb134bc680d52023-09-02T18:32:41ZengWolters Kluwer Health/LWWFormosan Journal of Surgery1682-606X2018-01-0151311812110.4103/fjs.fjs_108_17Adult-onset neurocutaneous melanosis with Dandy–Walker malformationTing-Wei ChangPo-Chuan HsiehZhuo-Hao LiuPo-Hsun TuNeurocutaneous melanosis (NCM) is characterized by diffuse or focal proliferation of melanin-producing cells over the skin or leptomeninges. Even without malignant transformation, the prognosis is poor after symptomatic progression of NCM, resulting from either mass effect in central nervous system or hydrocephalus. We reported a 26-year-old female patient with rapid deterioration after the onset of NCM. Despite no solid tumor formation, elevated cerebrospinal fluid protein content, which complicated shunting procedure, associated with hydrocephalus played the key role in clinical deterioration. Adult onset of NCM with Dandy–Walker malformation was never reported in our literature review. Diagnosis and treatment for such disease entity are discussed.http://www.e-fjs.org/article.asp?issn=1682-606X;year=2018;volume=51;issue=3;spage=118;epage=121;aulast=ChangDandy–Walker malformationneurocutaneous melanosisprognosisshunt |
| spellingShingle | Ting-Wei Chang Po-Chuan Hsieh Zhuo-Hao Liu Po-Hsun Tu Adult-onset neurocutaneous melanosis with Dandy–Walker malformation Formosan Journal of Surgery Dandy–Walker malformation neurocutaneous melanosis prognosis shunt |
| title | Adult-onset neurocutaneous melanosis with Dandy–Walker malformation |
| title_full | Adult-onset neurocutaneous melanosis with Dandy–Walker malformation |
| title_fullStr | Adult-onset neurocutaneous melanosis with Dandy–Walker malformation |
| title_full_unstemmed | Adult-onset neurocutaneous melanosis with Dandy–Walker malformation |
| title_short | Adult-onset neurocutaneous melanosis with Dandy–Walker malformation |
| title_sort | adult onset neurocutaneous melanosis with dandy walker malformation |
| topic | Dandy–Walker malformation neurocutaneous melanosis prognosis shunt |
| url | http://www.e-fjs.org/article.asp?issn=1682-606X;year=2018;volume=51;issue=3;spage=118;epage=121;aulast=Chang |
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