Clinical characteristics and treatment outcomes of pigmented tumors in central nervous system: Focusing on melanocytic tumors

J Clin Neurosci. 2020 Nov:81:83-89. doi: 10.1016/j.jocn.2020.09.009. Epub 2020 Sep 29.


Pigmented tumors are rare neoplasm of central nervous system. Melanocytic tumor, including primary and metastatic lesions, is the most common type. Owing to the rarity, the differential diagnosis of pigmented tumors and clinical management of melanocytic tumor remain challenge. Therefore, focusing on melanocytic tumors, the clinical, radiological, histopathological features and treatment outcomes were presented and analyzed in this study. We identified 22 melanocytic tumors, 2 melanotic medulloblastomas, 2 melanotic ependymomas and 1 melanotic schwannoma. Compared with metastatic melanocytic tumors (MMTs), primary melanocytic tumors (PMTs) were characterized by younger age (36.11 ± 17.96 vs. 51.69 ± 12.58 years, p = 0.0262), lower possibility to be multiple lesions (11.1%vs. 61.5%, p = 0.0306), higher proportion of hypointensity on T2-weighted images (66.7% vs. 15.4%, p = 0.0260) and higher frequency in black appearance (77.8% vs. 23.1%, p = 0.0247). During the follow-up, 4 PMTs and 11 MMTs (71.4%) experienced tumor progression. PMTs had better prognosis than MMTs that progression-free survival (PFS) rate of PMT was 50.0% but decreased to 23.1% for MMTs at 12 months (p = 0.0123). Cox proportional hazards regression revealed that multiplicity of tumor was an independent predictor for PFS. None of patient with multiple tumors was in PFS after 12 months' follow-up whereas PFS rate was 40.5% for single tumor (p = 0.0002). In conclusion, radiological appearances, especially hypointensity on T2-weighted images, might be an indication for PMT. MMTs are more likely to be multiple intraparenchymal masses in elder patients located in supratentorial region. Current treatments included operation, radiotherapy and chemotherapy are not competent to control tumor progression and other therapeutic modalities are urgently needed.

Keywords: Central nervous system; Differential diagnosis; Metastatic melanocytic tumor; Pigmented tumors; Primary melanocytic tumor.

MeSH terms

  • Adult
  • Aged
  • Brain Neoplasms / diagnostic imaging*
  • Brain Neoplasms / surgery
  • Cohort Studies
  • Diagnosis, Differential
  • Female
  • Follow-Up Studies
  • Humans
  • Magnetic Resonance Imaging / methods
  • Male
  • Medulloblastoma / diagnostic imaging*
  • Medulloblastoma / surgery
  • Middle Aged
  • Prognosis
  • Retrospective Studies
  • Skull Base Neoplasms / diagnostic imaging*
  • Skull Base Neoplasms / surgery
  • Spinal Neoplasms / diagnostic imaging*
  • Spinal Neoplasms / surgery
  • Treatment Outcome
  • Young Adult