Qazi Saba Saleem, Shah Syed Muhammad Ismail, Baqai Muhammad Waqas Saeed, Enam Syed Ather
Department of Neurosurgery, Ziauddin Medical University, Karachi, Pakistan.
Department of Neurosurgery, The Aga Khan University Hospital, Karachi, Pakistan.
Surg Neurol Int. 2022 Nov 25;13:547. doi: 10.25259/SNI_856_2022. eCollection 2022.
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.
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.
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.
中枢神经系统原发性黑素细胞肿瘤约占所有黑色素瘤的1%，发病高峰在第四个十年。该肿瘤起源于软脑膜黑素细胞，侵袭性程度不一。这些黑素细胞在真皮和神经系统中大量增殖可能引发对神经皮肤黑素沉着症（NCM）的怀疑，NCM是恶性黑色素瘤与巨大真皮内痣并存的一种关联。
我们报告一例62岁的南亚男性患者，其左半面部和头部有一个巨大的先天性黑素细胞痣（尺寸>20 cm），患者主诉有一次全身性强直阵挛发作，随后出现神经精神症状。对该患者进行了全面的临床和手术评估，最终罕见地诊断为左颞叶原发性软脑膜黑色素瘤。该患者随后接受了神经导航引导下的左颞开颅手术，病变被全切。
原发性软脑膜黑色素瘤与NCM存在临床关联的情况在文献中鲜有报道。迄今为止，我们的病例是极少数在这个年龄组中报道这种关联并伴有罕见神经精神症状的病例之一。
