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以下肢、排便及勃起功能障碍为表现的原发性髓内黑素细胞瘤:一例病例报告及文献复习

Primary intramedullary melanocytoma presenting with lower limbs, defecation, and erectile dysfunction: A case report and review of the literature.

作者信息

Liu Zhi-Qiang, Liu Chao, Fu Jia-Xin, He Yan-Qing, Wang Ying, Huang Tian-Xiang

机构信息

Department of Neuroscience, Erasmus Medical Center, Erasmus University Rotterdam, Rotterdam 3015 AA, Netherlands.

Department of Oncology, Xiangya Hospital of Central South University, Changsha 410008, Hunan Province, China.

出版信息

World J Clin Cases. 2021 Oct 6;9(28):8616-8626. doi: 10.12998/wjcc.v9.i28.8616.

Abstract

BACKGROUND

Primary intramedullary melanocytoma is an exceedingly rare type of primary melanocytic tumor in the central nervous system. Unfortunately, primary intramedullary melanocytoma lacks specificity in clinical symptoms and imaging features and there is currently no standard strategy for diagnosis or treatment.

CASE SUMMARY

A 52-year-old male patient suffered from weakness and numbness involving the bilateral lower limbs for 18 mo, and defecation and erectile dysfunction for 6 mo. Furthermore, these symptoms started to worsen for the last 3 mo. Preoperative magnetic resonance imaging (MRI) revealed an intramedullary tumor located at the T9-T10 level. In subsequently surgery, the maximal safe resection extent approached to 98%. The lesion was confirmed to be melanocytoma by pathological examination. In addition, the possibility of original melanocytoma outside the spinal cord was excluded after the examination of the whole body. Therefore, a diagnosis of primary intramedullary melanocytoma was established. The patient refused to accept radiotherapy or Gamma Knife, but MRI examination on July 28, 2020 showed no sign of development. In addition, on April 10, 2021, the recent review showed that the disorder of defecation and lower limbs improved further but erectile dysfunction benefited a little from the surgery.

CONCLUSION

After diagnosing intramedullary melanocytoma by postoperative pathology, the inspection of the whole body contributed to excluding the possibility of metastasis from other regions and further suggested a diagnosis of primary intramedullary melanocytoma. Complete resection, adjuvant radiation, and regular review are critical. In addition, maximal safe resection also benefits prognosis while the tumor is difficult to be resected totally.

摘要

背景

原发性髓内黑素细胞瘤是中枢神经系统中一种极其罕见的原发性黑素细胞肿瘤。不幸的是,原发性髓内黑素细胞瘤在临床症状和影像学特征上缺乏特异性,目前尚无标准的诊断或治疗策略。

病例摘要

一名52岁男性患者双侧下肢无力和麻木18个月,排便和勃起功能障碍6个月。此外,在过去3个月中这些症状开始加重。术前磁共振成像(MRI)显示T9 - T10水平有髓内肿瘤。在随后的手术中,最大安全切除范围接近98%。病理检查证实病变为黑素细胞瘤。此外,全身检查排除了脊髓外原发性黑素细胞瘤的可能性。因此,确诊为原发性髓内黑素细胞瘤。患者拒绝接受放疗或伽马刀治疗,但2020年7月28日的MRI检查显示无进展迹象。此外,2021年4月10日的近期复查显示,排便和下肢功能障碍进一步改善,但勃起功能障碍术后改善不大。

结论

术后病理诊断为髓内黑素细胞瘤后,全身检查有助于排除其他部位转移的可能性,进一步提示原发性髓内黑素细胞瘤的诊断。完整切除、辅助放疗和定期复查至关重要。此外,在肿瘤难以完全切除时,最大安全切除也有利于预后。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1056/8554421/d7c1e39ce063/WJCC-9-8616-g001.jpg

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