Non-meningothelial mesenchymal tumours of the CNS in the diagnostic practice of the pathologist

Introduction

Primary mesenchymal tumours of the central nervous system with uncertain differentiation are rare. The 5th edition of the WHO classification groups three of these entities according to their molecular profiles into: intracranial mesenchymal tumours with FET-CREB fusion, sarcomas with CIC rearrangement, and primary intracranial sarcomas with DICER1 mutations. The objectives of this study were to carry out a systematic review of the literature and to determine whether a specific morphology predominates in relation to the proposed molecular types.

Materials and methods

A systematic review was performed using the PubMed database, with the search terms: “(sarcomas) and (central nervous system)”. Studies were included if they reported cases of patients with a diagnosis of primary sarcoma in the central nervous system (CNS) and provided information on both the molecular profile and histopathological characteristics.

Results

Of the 173 articles identified, eight were ultimately selected for analysis.

Conclusions

Histopathological differences were observed that may assist in diagnosis in the absence of molecular testing; however, follow-up data are required to evaluate the benefits of this classification in clinical practice.