title: Clinical analysis of primary melanotic ependymoma in the central nervous system: case series and literature review.
abstract: BACKGROUND
  Melanotic ependymoma (ME) is an extremely rare subset of ependymoma characterized by cytoplasmic and extracellular deposition of melanin, with no case series having been reported to date. The aim of this study was to analyze the clinical manifestations, pathological and radiological features, treatment, and follow-up data, and to discuss the diagnosis, management and prognosis of ME.
  
  
  METHODS
  We retrospectively reviewed the clinical and imaging data of six patients with primary ME. The diagnosis was based on radiological and pathological criteria. All the patients were treated surgically with gross total resection. The preoperative and postoperative neurological states of the patients were analyzed. All the follow-up data were obtained from office visits, and the long-term outcomes are presented and discussed.
  
  
  RESULTS
  Patients presented with nonspecific symptoms. On magnetic resonance imaging (MRI), although a characteristic signal variation could be found reflecting the melanin component, individual appearances varied widely and were related to the content and distribution of melanin in the ME lesions. The characteristic ependymoma-related pathological appearance and specific immunostaining for glial fibrillary acidic protein and antimelanoma monoclonal antibody were revealed. During a mean follow-up period of 46.8 months, no local recurrences or distant metastases were observed.
  
  
  CONCLUSIONS
  The accurate diagnosis of ME depends on pathology. The histological grading used for ependymoma can be applied. Most MEs have a relatively benign course. Complete surgical resection alone is the treatment of choice for low-grade MEs, and the outcome may be favorable. Postoperative adjuvant radiotherapy can be considered for lesions with histologically anaplastic features.
authors.affiliation: Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, No.6, Tiantan Xili, Chongwen District, Beijing, 100050, China, 165208@qq.com.
authors.forename: Chenlong
authors.initials: C
authors.lastname: Yang
authors.forename: Guang
authors.initials: G
authors.lastname: Li
authors.forename: Jingyi
authors.initials: J
authors.lastname: Fang
authors.forename: Liang
authors.initials: L
authors.lastname: Wu
authors.forename: Xiaofeng
authors.initials: X
authors.lastname: Deng
authors.forename: Yulun
authors.initials: Y
authors.lastname: Xu
mesh_terms: D000328:Adult; D000368:Aged; D001932:Brain Neoplasms / Q000473:pathology / Q000532:radiotherapy / Q000601:surgery*; D002490:Central Nervous System / Q000473:pathology / Q000601:surgery*; D004806:Ependymoma / Q000532:radiotherapy / Q000601:surgery*; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D008875:Middle Aged; D060787:Neoplasm Grading; D009364:Neoplasm Recurrence, Local / Q000532:radiotherapy / Q000601:surgery*; D011379:Prognosis; D012189:Retrospective Studies; D012196:Review Literature as Topic; D013120:Spinal Cord Neoplasms / Q000532:radiotherapy / Q000601:surgery*