Background: Intramedullary melanocytomas are exceedingly rare, with only twenty-four cases reported up to now. They present as local invasive tumors despite their benign biological behavior. Attempting a complete safe resection often results in severe post-operative neurological deficits, as in our case presented here. Methods: A systematic review was conducted across the PubMed and Scopus databases including studies published till February 2024. Results: A total of 19 studies were included, encompassing 24 cases. A similar distribution between sexes was noted (M:F 13:11), with ages ranging from 19 to 79 years. The thoracic segment was most affected, and intermediate-grade melanocytoma (19 cases) was the most common histotype. Radiographically, intramedullary melanocytomas usually appear as hyperintense hemorrhagic lesions peripheral to the central canal with focal nodular enhancement. Intraoperatively, they are black–reddish to tan and are tenaciously adherent lesions. In the sampled studies, IONM employment was uncommon, and post-operative new-onset neurological deficits were described in 16 cases. Adjuvant RT was used in four cases and its value is debatable. Recurrence is common (10 cases), and adjuvant therapies (RT or repeated surgery) seem to play a palliative role. Case presentation: A 68-year-old woman presented with a three-year history of worsening spastic paraparesis and loss of independence in daily activities (McCormick grade 4). An MRI revealed an intramedullary tumor from Th5 to Th7, characterized by T1-weighted hyperintensity and signs of recent intralesional hemorrhage. Multimodal neuromonitoring, comprising the D-Wave, guided the resection of a black–tan-colored tumor with hyper-vascularization and strong adherence to the white matter. During final dissection of the lesion to obtain gross total resection (GTR), a steep decline in MEPs and D-Wave signals was recorded. Post-operatively, the patient had severe hypoesthesia with Th9 level and segmental motor deficits, with some improvement during neurorehabilitation. Histopathology revealed an intermediate-grade melanocytoma (CNS WHO 2021 classification). A four-month follow-up documented the absence of relapse. Conclusions: This literature review highlights that intramedullary T1 hyperintense hemorrhagic thoracic lesions in an adult patient should raise the suspicion of intramedullary melanocytoma. They present as locally aggressive tumors, due to local invasiveness, which often lead to post-operative neurological deficits, and frequent relapses, which overwhelm therapeutic strategies leading to palliative care after several years.
背景：髓内黑色素细胞瘤极为罕见，迄今仅报道24例。尽管其生物学行为良性，却表现为局部侵袭性肿瘤。如本文病例所示，试图进行完全安全切除常导致严重的术后神经功能缺损。 方法：对PubMed和Scopus数据库截至2024年2月发表的研究进行系统性综述。 结果：共纳入19项研究，涵盖24例病例。性别分布相近（男:女=13:11），年龄范围19至79岁。胸段最常受累，组织学类型以中级别黑色素细胞瘤最常见（19例）。影像学上，髓内黑色素细胞瘤通常表现为中央管周围的高信号出血性病灶，伴局灶性结节样强化。术中可见肿瘤呈黑红色至棕褐色，质地坚韧且粘连紧密。在纳入研究中，术中神经监测使用并不普遍，16例病例报告了术后新发神经功能缺损。4例采用辅助放疗，但其疗效存在争议。复发常见（10例），辅助治疗（放疗或再次手术）似乎仅起姑息作用。 病例报告：一位68岁女性因进行性加重的痉挛性截瘫三年就诊，日常生活能力丧失（McCormick分级4级）。MRI显示Th5至Th7水平髓内肿瘤，T1加权像呈高信号，伴近期瘤内出血征象。在多模态神经监测（包括D波监测）引导下，切除了呈黑棕色、血供丰富且与白质紧密粘连的肿瘤。在最终分离病灶以实现大体全切时，监测到运动诱发电位和D波信号急剧下降。术后患者出现Th9水平以下严重感觉减退及节段性运动功能障碍，经神经康复治疗后部分改善。组织病理学诊断为中级别黑色素细胞瘤（2021年CNS WHO分类）。四个月随访未见复发。 结论：本综述提示，成年患者胸段髓内T1高信号出血性病变需警惕髓内黑色素细胞瘤可能。这类肿瘤因局部侵袭性呈现局部侵袭特征，常导致术后神经功能缺损，且易复发，使得治疗策略往往在数年后转为姑息治疗。
肿瘤(癌症)患者之家