Intracranial Extraskeletal Mesenchymal Chondrosarcoma

World Neurosurg. 2016 Nov:95:618.e1-618.e6. doi: 10.1016/j.wneu.2016.08.049. Epub 2016 Aug 23.

Abstract

Background: Intracranial extraskeletal mesenchymal chondrosarcomas (MCSs) are rare tumors accounting for <0.16% of intracranial tumors. They are usually described as occurring in the age group of 20-30 years and are commonly found in the frontoparietal region arising from the falx and surrounding dura. We describe 3 cases at varying ages, locations, and outcomes.

Case description: A 42-year-old woman with anterior one third falx-based lesion; a 7-year-old boy with mid one third falcine lesion with hyperostosis of bone, presenting in comatose stage; and a 52-year-old woman with left posterior lateral frontal dural-based lesion are presented. Histopathology of all cases was confirmed with immunohistochemistry. All patients underwent complete excision but had varying outcomes.

Conclusions: Intracranial MCSs are rare malignant tumors with poor prognosis. Because of their rarity, it may not be considered as a differential diagnosis and it is often misdiagnosed radiologically, but the importance lies in the need of radical excision. The potential effect of postoperative radiotherapy and chemotherapy is controversial. This article sheds some light on variable prognosis of this tumor.

Keywords: Hemangiopericytoma; Meningeal tumor; Meningioma; Mesenchymal chondrosarcoma; Mesenchymal tumor.

Publication types

  • Case Reports

MeSH terms

  • 12E7 Antigen / metabolism
  • Adult
  • Brain Neoplasms / diagnostic imaging*
  • Brain Neoplasms / metabolism
  • Brain Neoplasms / pathology
  • Brain Neoplasms / surgery
  • Child
  • Chondrosarcoma, Mesenchymal / diagnostic imaging*
  • Chondrosarcoma, Mesenchymal / metabolism
  • Chondrosarcoma, Mesenchymal / pathology
  • Chondrosarcoma, Mesenchymal / surgery
  • Dura Mater / diagnostic imaging*
  • Dura Mater / metabolism
  • Dura Mater / pathology
  • Dura Mater / surgery
  • Female
  • Humans
  • Immunohistochemistry
  • Magnetic Resonance Imaging
  • Male
  • Meningeal Neoplasms / diagnostic imaging*
  • Meningeal Neoplasms / metabolism
  • Meningeal Neoplasms / pathology
  • Meningeal Neoplasms / surgery
  • Middle Aged
  • Mucin-1 / metabolism
  • S100 Proteins / metabolism
  • Synaptophysin / metabolism
  • Tomography, X-Ray Computed
  • Vimentin / metabolism

Substances

  • 12E7 Antigen
  • Mucin-1
  • S100 Proteins
  • Synaptophysin
  • Vimentin