一名青少年女性的原发性颅内恶性黑色素瘤:病例报告

IF 0.7 Q4 CLINICAL NEUROLOGY Egyptian journal of neurosurgery Pub Date : 2024-04-09 DOI:10.1186/s41984-024-00287-4
Vikarn Vishwajeet, Pallavi Saraf, Poonam Abhay Elhence, Sarbesh Tiwari, Jaskaran Singh Gosal, Deepak Kumar Jha
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摘要

原发性中枢神经系统黑色素瘤是一种极为罕见的疾病,与成人相比,儿童和青少年中的原发性中枢神经系统黑色素瘤更为罕见。如果没有任何神经皮肤黑色素沉着的临床证据,通常很难在术前考虑颅内黑色素瘤的诊断。本文报告了一例脑部原发性黑色素瘤病例,患者是一名 17 岁女性,表现为头痛、呕吐以及左侧面瘫和四肢无力的局灶性神经功能缺损。头部对比增强计算机断层扫描显示,左侧神经节囊区有一个异质性高增强肿块病变,显示周围增强,内部为非增强囊性成分。患者接受了左额颞部开颅手术。组织病理学检查显示,该肿瘤为浸润性肿瘤,由上皮样至纺锤形细胞组成,呈片状、巢状和单个散在排列。特殊染色和免疫组化标记对确诊非常有帮助。该病例突显了原发性颅内黑色素瘤在儿童年龄组中的罕见性、令人困惑的组织学特征以及迅速而致命的病程。
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Primary intracranial malignant melanoma in an adolescent female: a case report
Primary central nervous system melanoma is an extremely rare entity and even rarer in children and adolescents as compared to adults. It is often difficult to consider a diagnosis of intracranial melanoma pre-operatively without any clinical evidence of neurocutaneous melanosis. Herein, a case of primary melanoma of the brain is reported in a 17-year-old female who presented with headache, vomiting, and focal neurological deficit in the form of left-sided facial palsy and limb weakness. A contrast-enhanced computed tomography of head was performed which revealed a heterogeneously hyperattenuating mass lesion at left gangliocapsular region showing peripheral enhancement with internal non-enhancing cystic component. The patient underwent left frontotemporal craniotomy. The diagnosis was made on histopathological examination, which showed an invasive tumor comprising of epithelioid to spindled cells arranged in sheets, nests, and singly scattered. The special stains and immunohistochemical markers proved very helpful in establishing the diagnosis. The case highlights the uncommon occurrence of primary intracranial melanoma in the pediatric age group, the perplexing histological features, and the rapid and fatal course.
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