PDF(Pubmed)
Abstract：
BACKGROUND: Neurocutaneous melanosis (NCM) is a rare congenital, nonhereditary neurocutaneous syndrome that mainly occurs in children; adult NCM is very rare. Due to its rarity, the clinical features and treatment strategies for NCM remain unclear. The purpose of this study was to explore the clinical features, diagnosis, treatment and prognosis of NCM in adults. Most intracranial meningeal melanomas are solid masses, and cystic-solid malignant melanomas are very rare. Due to the lack of data, the cause of cystic changes and the effect on prognosis are unknown.
METHODS: A 41-year-old woman was admitted to the hospital with intermittent headache for 1 mo. Magnetic resonance imaging (MRI) showed a 4.7 cm × 3.6 cm cystic-solid mass in the left temporal lobe with peritumoral edema. The entire mass was removed, and postoperative pathology indicated malignant melanoma.
CONCLUSIONS: MRI is the first-choice imaging approach for diagnosing central nervous system diseases in NCM patients, although cerebrospinal fluid may also be used. At present, there is no optimal treatment plan; gross total resection combined with BRAF inhibitors and MEK inhibitors might be the most beneficial treatment.
METHODS: A 41-year-old woman was admitted to the hospital with intermittent headache for 1 mo. Magnetic resonance imaging (MRI) showed a 4.7 cm × 3.6 cm cystic-solid mass in the left temporal lobe with peritumoral edema. The entire mass was removed, and postoperative pathology indicated malignant melanoma.
CONCLUSIONS: MRI is the first-choice imaging approach for diagnosing central nervous system diseases in NCM patients, although cerebrospinal fluid may also be used. At present, there is no optimal treatment plan; gross total resection combined with BRAF inhibitors and MEK inhibitors might be the most beneficial treatment.
摘要：
背景：神经皮肤黑变病（NCM）是一种罕见的先天性，非遗传性神经皮肤综合征，主要发生在儿童；成人NCM非常罕见。由于它的稀有性,NCM的临床特征和治疗策略尚不清楚.这项研究的目的是探讨临床特征，诊断,成人NCM的治疗和预后。大多数颅内脑膜黑素瘤是实性肿块，囊实性恶性黑色素瘤非常罕见。由于缺乏数据,囊性改变的原因和对预后的影响尚不清楚.
方法：一名41岁女性因间歇性头痛入院1个月。磁共振成像（MRI）显示左侧颞叶有4.7cm×3.6cm的囊实性肿块，并伴有瘤周水肿。整个肿块都被切除了,术后病理提示恶性黑色素瘤。
结论：MRI是诊断NCM患者中枢神经系统疾病的首选影像学检查方法，尽管也可以使用脑脊液。目前,目前尚无最佳治疗方案;大体全切除联合BRAF抑制剂和MEK抑制剂可能是最有益的治疗方案.
方法：一名41岁女性因间歇性头痛入院1个月。磁共振成像（MRI）显示左侧颞叶有4.7cm×3.6cm的囊实性肿块，并伴有瘤周水肿。整个肿块都被切除了,术后病理提示恶性黑色素瘤。
结论：MRI是诊断NCM患者中枢神经系统疾病的首选影像学检查方法，尽管也可以使用脑脊液。目前,目前尚无最佳治疗方案;大体全切除联合BRAF抑制剂和MEK抑制剂可能是最有益的治疗方案.
