PDF(Pubmed)
Abstract：
Introduction: Primary melanocytic tumors originating from CNS melanocytes are rare, with a low incidence of 0.7 cases per 10 million annually. This study focuses on primary leptomeningeal melanocytomas, emphasizing their epidemiology, clinical characteristics, and diagnostic challenges. Despite their infrequency, these tumors warrant attention due to their unique features and potential for local recurrence. Case Report: A 32-year-old female presented with syncope and seizures, leading to the discovery of two left-sided supratentorial lesions initially misidentified as convexity meningiomas. Detailed imaging suggested meningioma-like features, but intraoperative findings revealed unexpected hyperpigmented lesions. Histopathological examination, supported by immunohistochemistry, confirmed primary leptomeningeal melanocytoma. The surgical approach and subsequent management are discussed. Discussion: The discussion emphasizes challenges in diagnosing primary leptomeningeal melanocytomas. Treatment debates, especially regarding adjuvant radiotherapy, are explored. Recurrence risks stress the importance of vigilant follow-up, advocating for complete surgical resection as the primary approach. The rarity of supratentorial cases adds complexity to diagnosis, necessitating a multidisciplinary approach. Insights from this case contribute to understanding and managing primary leptomeningeal melanocytomas, addressing challenges in differentiation from more common tumors and prompting ongoing research for refined diagnostics and optimized treatments. Conclusion: This study contributes insights into primary leptomeningeal melanocytomas, highlighting their rarity in supratentorial regions. The case underscores the importance of a multidisciplinary approach, incorporating clinical, radiological, and histopathological expertise for accurate diagnosis and tailored management. Ongoing research is crucial to refine treatment strategies, enhance prognostic precision, and improve outcomes for individuals with this uncommon CNS neoplasm.
摘要：
简介：源自中枢神经系统黑素细胞的原发性黑素细胞肿瘤很少见，发病率低，每年每1000万有0.7例。本研究的重点是原发性软脑膜黑素细胞瘤，强调他们的流行病学，临床特征,和诊断挑战。尽管他们的频率不高，这些肿瘤因其独特的特征和可能的局部复发而值得关注.病例报告：一名32岁女性出现晕厥和癫痫发作，导致发现两个最初被误认为是凸面脑膜瘤的左侧幕上病变。详细的影像显示脑膜瘤样特征，但是术中发现了意想不到的色素沉着过度病变。组织病理学检查，由免疫组织化学支持，证实原发性软脑膜黑色素细胞瘤。讨论了手术方法和后续处理。讨论：讨论强调了诊断原发性软脑膜黑素细胞瘤的挑战。治疗辩论，特别是关于辅助放疗，正在探索。复发风险强调了警惕后续行动的重要性，提倡以完全手术切除为主要方法。罕见的幕上病例增加了诊断的复杂性，需要多学科方法。这种情况下的见解有助于理解和管理原发性软脑膜黑色素细胞瘤，解决从更常见的肿瘤中区分的挑战，并促进正在进行的研究，以完善的诊断和优化的治疗。结论：本研究有助于了解原发性软脑膜黑素细胞瘤，突出了它们在幕上区域的稀有性。该案例强调了多学科方法的重要性，结合临床，放射学,和组织病理学专业知识的准确诊断和量身定制的管理。持续的研究对于完善治疗策略至关重要，提高预后精度，并改善患有这种罕见中枢神经系统肿瘤的个体的结局。
