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梅克尔腔原发性中枢神经系统黑色素瘤:1例罕见病例并文献复习

Primary CNS Melanoma of Meckel's Cave: A Rare Case with Literature Review.

作者信息

Makkina Charan, Joseph Jeena, Raju Krishna Prabhu, Jayachandran Ranjani, Chacko Geeta

机构信息

Department of Neurological Sciences, Christian Medical College, Vellore, Tamil Nadu, India.

Department of Neuropathology, Christian Medical College, Vellore, Tamil Nadu, India.

出版信息

Asian J Neurosurg. 2024 Jun 13;19(3):563-566. doi: 10.1055/s-0044-1787822. eCollection 2024 Sep.

Abstract

Meckel's cave is a dural recess in the posteromedial portion of the middle cranial fossa, serving as a conduit for the trigeminal nerve and hosting various pathologies. The radiological diagnosis of Meckel's cave pathologies is often challenging, especially when they are atypical and rarely encountered. Here, we discuss the case of a 41-year-old woman who presented with right hemifacial pain, numbness, and binocular diplopia. Imaging features suggested a T2 hypointense, T1 hyperintense, and nonenhancing mass in the right Meckel's cave. Intraoperatively, an extra-axial black mass was observed, suggestive of melanoma, which was radically excised. Further postoperative workup and biopsy revealed it was a primary central nervous system (CNS) malignant melanoma, an exceedingly rare condition. Due to the rarity of the disease, a consensus regarding treatment regimens is lacking. This case report underscores the significance of considering uncommon diagnoses when faced with unusual radiological findings and emphasizes the importance of aggressive surgical resection and the evolving landscape of adjuvant treatments for primary CNS melanomas.

摘要

梅克尔腔是中颅窝后内侧部分的硬脑膜隐窝,是三叉神经的通道,并可发生多种病变。梅克尔腔病变的影像学诊断往往具有挑战性,尤其是当病变不典型且罕见时。在此,我们讨论一例41岁女性患者,她表现为右侧面部疼痛、麻木和双眼复视。影像学特征显示右侧梅克尔腔内有一个T2低信号、T1高信号且无强化的肿块。术中观察到一个轴外黑色肿块,提示为黑色素瘤,并进行了根治性切除。术后进一步检查和活检显示这是一例原发性中枢神经系统(CNS)恶性黑色素瘤,极为罕见。由于该病罕见,缺乏关于治疗方案的共识。本病例报告强调了面对不寻常的影像学表现时考虑罕见诊断的重要性,并强调了积极手术切除以及原发性CNS黑色素瘤辅助治疗不断发展的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ab3c/11349392/0c407607c467/10-1055-s-0044-1787822-i23120016-1.jpg

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