Liu ZQ, Liu C, Fu JX, He YQ, Wang Y, Huang TX. Primary intramedullary melanocytoma presenting with lower limbs, defecation, and erectile dysfunction: A case report and review of the literature. World J Clin Cases 2021; 9(28): 8616-8626 [PMID: 34754876 DOI: 10.12998/wjcc.v9.i28.8616]
Corresponding Author of This Article
Tian-Xiang Huang, MD, PhD, Research Associate, Department of Neurosurgery, Xiangya Hospital of Central South University, No. 87 Xiangya Road, Changsha 410008, Hunan Province, China. firstname.lastname@example.org
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This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Zhi-Qiang Liu, Department of Neuroscience, Erasmus Medical Center, Erasmus University Rotterdam, Rotterdam 3015 AA, Netherlands
Chao Liu, Department of Oncology, Xiangya Hospital of Central South University, Changsha 410008, Hunan Province, China
Jia-Xin Fu, Grade of 2015 in Oral Medicine, Medical College of Jinzhou Medical University, Jinzhou 121001, Liaoning Province, China
Yan-Qing He, Ying Wang, Tian-Xiang Huang, Department of Neurosurgery, Xiangya Hospital of Central South University, Changsha 410008, Hunan Province, China
Author contributions: Liu ZQ drafted the manuscript; Huang TX reviewed the literature and contributed to manuscript drafting; Liu C and Fu JX collected and analysed the data; He YQ and Wang Y were responsible for the follow-up; all authors issued final approval for the version to be submitted.
Supported byNatural Science Foundation of Hunan Province, China, No. 2019JJ50964.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: The authors declare that they have no conflict of interest to report.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Tian-Xiang Huang, MD, PhD, Research Associate, Department of Neurosurgery, Xiangya Hospital of Central South University, No. 87 Xiangya Road, Changsha 410008, Hunan Province, China. email@example.com
Received: May 29, 2021 Peer-review started: May 29, 2021 First decision: June 14, 2021 Revised: June 16, 2021 Accepted: August 6, 2021 Article in press: August 6, 2021 Published online: October 6, 2021
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.
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.
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.
Core Tip: Primary intramedullary melanocytoma is an extremely rare kind of primary melanocytic tumor within the spinal cord. The features in imaging are nonspecific depending on the degree of melanization, intra-tumoral hemorrhage, and duration of bleeding. Therefore, diagnosis confirmation consists of two key points: (1) Pathological test; and (2) excluding the possibility of metastases from other melanocytomas outside the spinal cord using the positron emission tomography–computed tomography scanning and physical examination of the whole body. The recommended therapy strategy includes complete resection and subsequent adjuvant radiation or Gamma Knife radiosurgery. Besides, maximal safe resection is also beneficial for cases with difficulty to be resected totally. More case studies are needed to determine the optimal management strategy due to its rarity.