关键词: Leptomeningeal melanoma Neural crest Neurocutaneous melanosis Nevus Primary melanoma Seizures

来  源:   DOI:10.25259/SNI_856_2022   PDF(Pubmed)

Abstract:
UNASSIGNED: Primary melanocytic tumors of the central nervous system accounts for approximately 1% of all melanoma with a peak incidence in the fourth decade. The tumor originates from leptomeningeal melanocytes with a variable degree of belligerence. The proliferation of these melanocytes in large amounts in the dermis and nervous system can raise suspicion of neurocutaneous melanosis (NCM), which is an association between malignant melanoma and the presence of a giant intradermal nevus.
UNASSIGNED: We present a case of a 62-year-old South Asian male with a large congenital melanocytic nevus (>20 cm in size) in the left hemifacial, and head region who presented with complaints of a single episode of grand-mal seizure followed by neuropsychiatric symptoms. The patient was thoroughly evaluated both clinically and surgically leading to a rare diagnosis of primary leptomeningeal melanoma of the left temporal lobe. The patient subsequently underwent a neuronavigation guided left temporal craniotomy with gross total resection of the lesion.
UNASSIGNED: Primary leptomeningeal melanoma with a clinical association with NCM is rarely ever reported within the literature. To date, our case is one of the very few instances where such an association is being reported in this age group along with rare neuropsychiatric symptoms.
摘要:
未经证实:中枢神经系统的原发性黑色素细胞肿瘤约占所有黑色素瘤的1%,在第四个十年中发病率最高。肿瘤起源于软脑膜黑素细胞,具有不同程度的好战性。这些黑素细胞在真皮和神经系统中大量增殖可以引起神经皮肤黑变病(NCM)的怀疑,这是恶性黑色素瘤和巨大皮内痣之间的关联。
UNASSIGNED:我们介绍了一例62岁的南亚男性,左半面有一个巨大的先天性黑素细胞痣(>20厘米),和头部区域,主诉一次癫痫发作,随后出现神经精神症状。对患者进行了全面的临床和手术评估,导致罕见的诊断为左颞叶原发性软脑膜黑色素瘤。患者随后接受了神经导航引导的左颞开颅手术,并完全切除了病变。
未经证实:与NCM临床相关的原发性软脑膜黑色素瘤在文献中很少报道。迄今为止,我们的病例是在该年龄组中报道这种关联并伴有罕见神经精神症状的极少数病例之一.
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