Supratentorial meningeal melanocytoma mimicking meningioma: case report and literature review

Mayle Gomes Ferreira de Araújo, Luiz Euripedes Almondes Santana Lemos, Pedro Lucas Negromonte Guerra, Fernanda Marcia dos Santos Lima Didjurgeit, Auricelio Batista Cezar, I. Faquini, Hildo Rocha Cirne de Azevedo Filho
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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.
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幕上脑膜黑色素细胞瘤模仿脑膜瘤:病例报告和文献综述
导言:源于中枢神经系统黑色素细胞的原发性黑色素细胞瘤非常罕见,发病率很低,每年每1000万人中仅有0.7例。本研究重点关注原发性脑膜黑色素细胞瘤,强调其流行病学、临床特征和诊断难题。尽管这些肿瘤并不常见,但由于其独特的特征和局部复发的可能性,仍值得关注:一名 32 岁的女性因晕厥和癫痫发作就诊,结果发现了两个左侧脑室上病变,最初被误诊为凸面脑膜瘤。详细的影像学检查提示其具有脑膜瘤样特征,但术中发现了意想不到的色素沉着病变。组织病理学检查和免疫组化证实了原发性脑膜黑色素细胞瘤。本文讨论了手术方法和后续处理:讨论强调了诊断原发性脑膜黑色素细胞瘤所面临的挑战。探讨了治疗方面的争论,尤其是关于辅助放射治疗的争论。复发风险强调了警惕随访的重要性,主张以完全手术切除为主要方法。幕上病例的罕见性增加了诊断的复杂性,因此有必要采用多学科方法。本病例的见解有助于理解和处理原发性脑膜外黑色素细胞瘤,解决了与更常见肿瘤鉴别的难题,并促进了对精细诊断和优化治疗的持续研究:本研究有助于深入了解原发性脑膜外黑色素细胞瘤,突出了其在幕上区域的罕见性。该病例强调了结合临床、放射学和组织病理学专业知识的多学科方法对于准确诊断和针对性治疗的重要性。持续的研究对于完善治疗策略、提高预后精确度以及改善这种不常见的中枢神经系统肿瘤患者的预后至关重要。
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