Neurocutaneous Melanocytosis-Associated Hydrocephalus: The Memorial Sloan Kettering Experience from 2001 to 2022.

IF 2.4 3区 医学 Q2 HEMATOLOGY Pediatric Blood & Cancer Pub Date : 2024-11-06 DOI:10.1002/pbc.31434
Scarlett Rodriguez, Francesca DeIeso-Frechette, Chidobe Umeasor, Christianah Alli, Caline Azzi, Sofia Haque, John A Pugh, Nathan Meeker, Yasmin Khakoo
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Abstract

We report two neurocutaneous melanocytosis (NCM) patients who required ventriculoperitoneal shunt placement and subsequently developed intraperitoneal melanoma. These patients with NCM are at an increased risk for developing NRAS-associated melanomas in the central nervous system, which in turn may lead to symptomatic hydrocephalus requiring cerebrospinal fluid diversion. Due to the rarity of NCM, current knowledge on disease progression and appropriate management is limited. Ongoing studies aiming to better understand this condition and inform its clinical management may help to identify risk factors for developing more severe complications.

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神经皮肤黑色素细胞增多症相关性脑积水:2001 年至 2022 年纪念斯隆凯特琳医院的经验。
我们报告了两名神经皮肤黑色素细胞增多症(NCM)患者,他们需要进行脑室腹腔分流术,随后患上了腹膜内黑色素瘤。这些 NCM 患者在中枢神经系统中罹患 NRAS 相关黑色素瘤的风险增加,进而可能导致症状性脑积水,需要进行脑脊液分流。由于 NCM 的罕见性,目前有关疾病进展和适当治疗的知识非常有限。目前正在进行的研究旨在更好地了解这种疾病并为临床治疗提供依据,这些研究可能有助于确定出现更严重并发症的风险因素。
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来源期刊
Pediatric Blood & Cancer
Pediatric Blood & Cancer 医学-小儿科
CiteScore
4.90
自引率
9.40%
发文量
546
审稿时长
1.5 months
期刊介绍: Pediatric Blood & Cancer publishes the highest quality manuscripts describing basic and clinical investigations of blood disorders and malignant diseases of childhood including diagnosis, treatment, epidemiology, etiology, biology, and molecular and clinical genetics of these diseases as they affect children, adolescents, and young adults. Pediatric Blood & Cancer will also include studies on such treatment options as hematopoietic stem cell transplantation, immunology, and gene therapy.
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