Some CNS sarcomas seen: A 22-year series

Clin Neuropathol. 2023 Mar-Apr;42(2):54-65. doi: 10.5414/NP301512.


Aims: Central nervous system (CNS) and spine are seldom impacted by primary or metastatic sarcomas. We reviewed our 22-year experience with metastatic versus primary mesenchymal sarcomas in adults versus pediatric patients, additionally asking how many might today undergo nomenclature changes using CNS World Health Organization, 5th edition criteria.

Materials and methods: Case identification via text word search of pathology databases from our adult and pediatric referral hospitals, 2000 to August 2022, with exclusion of peripheral nervous system and primary chondro-osseous and notochordal tumors. Demographic, immunohistochemical, fluorescence in situ hybridization (FISH), and fusion results performed at the time of original diagnosis were acquired from reports.

Results: 57 cases were identified, with a 16 : 15 primary and 19 : 7 metastatic ratio in adult versus pediatric patients. Ewing sarcoma was the most frequent type (n = 18, 7 adult, 11 pediatric), with a rare primary PEComa, 2 alveolar soft part sarcomas, and metastatic angiosarcoma in the cohort. Only 3 cases, an intracranial sarcoma, DICER-1 mutant formerly diagnosed as rhabdomyosarcoma, an intracranial mesenchymal tumor, FET::CREB fusion-positive formerly diagnosed as angiomatoid fibrous histiocytoma, and a CIC-rearranged sarcoma required nomenclature updating by CNS WHO5 criteria.

Conclusions: Few primary or metastatic, adult or pediatric, CNS/spinal sarcomas required nomenclature updates; almost all had been satisfactorily classified at the time of diagnosis, using immunohistochemistry, FISH, or fusion results.

Publication types

  • Review

MeSH terms

  • Biomarkers, Tumor
  • Bone Neoplasms* / pathology
  • Brain Neoplasms* / genetics
  • Humans
  • In Situ Hybridization, Fluorescence
  • Rhabdomyosarcoma*
  • Sarcoma* / pathology
  • Sarcoma, Ewing* / diagnosis
  • Sarcoma, Ewing* / pathology


  • Biomarkers, Tumor