Primary central nervous system sarcomas in adults: A systematic review

Clin Neurol Neurosurg. 2022 Mar:214:107127. doi: 10.1016/j.clineuro.2022.107127. Epub 2022 Jan 12.


Background: Primary central nervous system (CNS) sarcomas represent a heterogeneous group of rare neoplasms with unclear etiology. Available data on clinical characteristics, treatment strategies, and survival are scarce. We comprehensively reviewed management strategies and outcomes of primary CNS sarcomas in adults.

Methods: PubMed, Scopus, and Cochrane were search following the PRISMA guidelines to include studies on primary CNS sarcomas in adults. Clinical features, management strategies, and survival were analyzed.

Results: We included 9 studies comprising 78 patients. Primary CNS sarcomas were mostly intracranial (87.2%), frequently located in the parietal (17.9%), frontal (14.1%), and temporal (14.1%) lobes. Spinal CNS sarcomas were found in 10 patients (12.8%). The most common tumor histology were fibrosarcoma (16.7%), intracranial synovial sarcoma (12.8%), extraosseous mesenchymal chondrosarcoma (11.5%), perivascular sarcoma (11.5%), reticulum cell sarcoma (11.5%), and myeloid sarcoma (9%). Partial resection (57.7%) was preferred over complete resection (42.3%), and 43 patients (55.1%) received adjuvant treatments: radiotherapy (51.3%) and/or systemic chemotherapy (20.5%). 21 patients experienced CNS sarcomas recurrences, with a median progression-free survival of 9 months (range, 4-48). At last follow-up, 60 patients (76.9%) were dead, with a median overall survival of 9 months (0.1-396). Overall survival was significantly longer in patients with fibrosarcoma (p = 0.001).

Conclusion: Surgical resection coupled with adjuvant chemotherapy or radiation has historically been the cornerstone treatment for CNS sarcoma but showed poor local control and dismal survival. A better understanding of the CNS sarcoma microenvironment may favor the development of tailored strategies aimed at improving survival.

Keywords: Brain tumor; Primary CNS sarcoma; Systematic review.

Publication types

  • Review
  • Systematic Review

MeSH terms

  • Adult
  • Central Nervous System / pathology
  • Central Nervous System Neoplasms* / therapy
  • Chemotherapy, Adjuvant
  • Fibrosarcoma* / drug therapy
  • Humans
  • Retrospective Studies
  • Sarcoma* / therapy
  • Tumor Microenvironment