Objective To investigate the clinical features, imaging characteristics, treatment measures, and prognosis of melanocytic tumors of the central nervous system (CNS). Methods The clinical data of 14 patients with melanocytic tumors of CNS confirmed by surgery and pathology were analyzed retrospectively, and the disease was analyzed according to the literature. Results The clinical manifestations of melanocytic tumors of CNS were diverse; the main symptoms were headache, increased intracranial pressure, seizures, and focal neurological deficits.The imaging features were diversified, with 7 of short T1 and short T2 signal, 3 of identical T1 and long T2 signal, and 3 of heterogenous signal among the 13 patients who had the MRI examination.Immunohistochemistry showed HMB45 (+ ) in 14 cases, S-100 (+ ) in 13 cases, and VIM (+ ) in 8 cases.In the initial operations, the total, subtotal and partial removals of the tumors were achieved in 7, 5, and 2 cases, respectively. Four patients were operated again for recurrence of tumors after procedure.The postoperative follow-up time was 2 to 60 months.Eleven patients were followed up, and 7 of them received adjuvant therapy. Nine cases died and 2 cases survived; the median survival time of the died cases was 14 months. Conclusions The clinical manifestation of melanocytic tumors of CNS do not have any specificity, and short T1 and short T2 signal on the MRI is the relatively characteristic imaging display. The immunohistochemistry test is help to get the definite diagnosis. The overall malignant degree is high and the prognosis is poor. Operation should be the preferred therapeutic measure. Postoperative adjuvant treatment measures can be taken, such as radiotherapy and chemotherapy. Key words: Central nervous system; Melanoma; Neurosurgical procedures; Radiotherapy; Chemotherapy
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