Department of Neurosurgery, Hospital da Restauração, Recife, Brazil.
Center for Medical Sciences, Universidade Federal de Pernambuco, Recife, Brazil.
Pathol Oncol Res. 2024 Jan 4;29:1611482. doi: 10.3389/pore.2023.1611482. eCollection 2023.
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. 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. 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. 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.
原发于 CNS 黑色素细胞的黑色素细胞肿瘤很少见,每年每 100 万人的发病率为 0.7 例。本研究侧重于原发脑膜黑色素细胞瘤,强调其流行病学、临床特征和诊断挑战。尽管这些肿瘤罕见,但由于其独特的特征和局部复发的可能性,值得关注。
一名 32 岁女性因晕厥和癫痫发作就诊,最初发现两个左侧幕上病变,最初误诊为凸面脑膜瘤。详细的影像学检查提示脑膜瘤样特征,但术中发现出乎意料的色素沉着病变。组织病理学检查,辅以免疫组织化学,证实了原发脑膜黑色素细胞瘤。讨论了手术方法和随后的管理。
讨论强调了诊断原发脑膜黑色素细胞瘤的挑战。治疗争议,特别是关于辅助放疗的争议,也进行了探讨。复发风险强调了密切随访的重要性,主张完全手术切除作为主要方法。幕上病例的罕见性增加了诊断的复杂性,需要多学科方法。该病例的见解有助于理解和管理原发脑膜黑色素细胞瘤,解决与更常见肿瘤区分的挑战,并促使进行更精细的诊断和优化治疗的研究。
本研究深入了解了原发脑膜黑色素细胞瘤,强调了其在幕上区域的罕见性。该病例强调了多学科方法的重要性,即整合临床、放射学和组织病理学专业知识,以进行准确的诊断和个体化的管理。持续的研究对于完善治疗策略、提高预后准确性以及改善此类罕见 CNS 肿瘤患者的结局至关重要。