Intracranial Mesenchymal Chondrosarcoma: Report of 16 Cases

World Neurosurg. 2018 Aug:116:e691-e698. doi: 10.1016/j.wneu.2018.05.069. Epub 2018 May 18.

Abstract

Objectives: Limited data regarding intracranial mesenchymal chondrosarcoma (MCS) are available. The goal of this study was to report the clinical characteristics, challenges in management, and poor outcomes of intracranial MCS.

Methods: Clinical data for 16 patients with MCS were reviewed retrospectively to evaluate their clinical characteristics, management, and outcomes.

Results: This study included 11 male and 5 female patients with a mean age of 22.9 ± 14.4 years. The most common presentations were headache (n = 10; 62.5%), followed by cranial deficits (n = 7; 43.6%). The radiologic spectrum for MCS was broad, and only 18.8% (3/16) of MCSs were correctly diagnosed preoperatively. Aggressive resection (including subtotal resection and gross total resection) and partial resection was performed in 62.5% (10/16) and 37.50% (6/16) of patients. With a median follow-up of 34 months (range, 10-78 months), 5 patients (31.3%) died and 8 patients (50%) developed tumor recurrence. The 1-, 3-, and 5-year rates of progression-free survival and overall survival were 86%, 53%, and 42% and 93%, 70%, and 56%, respectively. Although the differences were not significantly different, aggressive resection and the use of radiotherapy tended to improve the prognosis of the patients.

Conclusions: Clinical characteristics of MCS are variable. The current management of intracranial MCS referring to conventional chondrosarcoma could not yield satisfactory outcomes. Further study is needed to identify the optimal treatments.

Keywords: Chemotherapy; Clinical characteristics; Intracranial mesenchymal chondrosarcoma; Prognosis; Radiotherapy; Skull base.

MeSH terms

  • Adolescent
  • Adult
  • Bone Neoplasms / diagnostic imaging*
  • Bone Neoplasms / mortality
  • Bone Neoplasms / therapy*
  • Child
  • Child, Preschool
  • Chondrosarcoma, Mesenchymal / diagnostic imaging*
  • Chondrosarcoma, Mesenchymal / mortality
  • Chondrosarcoma, Mesenchymal / surgery
  • Chondrosarcoma, Mesenchymal / therapy*
  • Female
  • Humans
  • Kaplan-Meier Estimate
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Severity of Illness Index
  • Tomography Scanners, X-Ray Computed
  • Young Adult