Abstract:
BACKGROUND:Primary leptomeningeal melanomatosis (PLM) is a rare and aggressive form of nonmetastatic invasion of leptomeninges by malignant melanocytic cells. Clinical presentation includes nonspecific meningism with various forms of cerebrospinal fluid circulation or absorption disorders leading to hydrocephalus. CASE DESCRIPTION:A 5-year-old child with PLM without neurocutaneous melanosis presented with cystic enlargement of the brainstem surrounding cisterns and hydrocephalus requiring occipitoaxial decompression and endoscopic cystostomy. Initial cerebrospinal fluid cytology screening and frontal meningeal and brain biopsy specimens showed negative results. The diagnosis of unpigmented (amelanotic) malignant melanocytic cells was made after a biopsy specimen of the bulbar leptomeninges was obtained. Despite continuous management of hydrocephalus and chemotherapy, the disease progressed, and the patient died 11 months after diagnosis. CONCLUSIONS:To the best of our knowledge, this is the first report of an amelanotic form of PLM without association of neurocutaneous melanosis in a child. This case report illustrates the difficulty of diagnosis in the absence of cutaneous lesions and lack of melanin.
journal_name
World Neurosurgjournal_title
World neurosurgeryauthors
Szathmari A,Perbet R,Hermier M,Di Rocco F,Frappaz D,Mottolese Cdoi
10.1016/j.wneu.2016.06.039subject
Has Abstractpub_date
2016-08-01 00:00:00pages
581.e15-581.e20eissn
1878-8750issn
1878-8769pii
S1878-8750(16)30424-7journal_volume
92pub_type
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doi:10.1016/j.wneu.2020.11.108
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pub_type: 杂志文章
doi:10.1016/j.wneu.2018.06.027
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journal_title:World neurosurgery
pub_type: 杂志文章
doi:10.1016/j.wneu.2017.09.079
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journal_title:World neurosurgery
pub_type: 杂志文章,meta分析
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abstract:OBJECTIVE:Previous studies have suggested that postoperative hypopituitarism in patients with nonsellar intracranial tumors is caused by traumatic surgery. However, with development of minimally invasive and precise neurosurgical techniques, the degree of injury to brain tissue has been reduced significantly, especiall...
journal_title:World neurosurgery
pub_type: 杂志文章
doi:10.1016/j.wneu.2017.03.116
更新日期:2017-09-01 00:00:00
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pub_type: 杂志文章
doi:10.1016/j.wneu.2018.11.071
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pub_type: 杂志文章
doi:10.1016/j.wneu.2018.11.110
更新日期:2019-02-01 00:00:00
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journal_title:World neurosurgery
pub_type: 杂志文章,评审
doi:10.1016/j.wneu.2017.03.030
更新日期:2017-05-01 00:00:00
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pub_type: 杂志文章
doi:10.1016/j.wneu.2017.08.062
更新日期:2017-11-01 00:00:00
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pub_type: 杂志文章
doi:10.1016/j.wneu.2017.06.031
更新日期:2017-09-01 00:00:00
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journal_title:World neurosurgery
pub_type: 杂志文章,meta分析,评审
doi:10.1016/j.wneu.2014.07.041
更新日期:2014-12-01 00:00:00
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pub_type: 杂志文章
doi:10.1016/j.wneu.2016.04.020
更新日期:2016-07-01 00:00:00
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pub_type: 杂志文章
doi:10.1016/j.wneu.2016.03.104
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pub_type: 杂志文章
doi:10.1016/j.wneu.2017.06.013
更新日期:2017-09-01 00:00:00
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pub_type: 杂志文章,评审
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更新日期:2019-02-01 00:00:00
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更新日期:2018-01-01 00:00:00
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更新日期:2010-02-01 00:00:00
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更新日期:2017-06-01 00:00:00
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pub_type: 杂志文章
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更新日期:2018-05-01 00:00:00
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pub_type: 杂志文章
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更新日期:2018-04-01 00:00:00
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journal_title:World neurosurgery
pub_type: 杂志文章
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更新日期:2020-07-01 00:00:00
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pub_type: 杂志文章
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更新日期:2021-01-11 00:00:00