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Central nervous system tumors of uncertain differentiation
1Neuro-Oncology Unit, Center for the Treatment and Investigation About Cancer (CTIC), Bogotá, Colombia.
Background:
The 2021 World Health Organization classification for brain tumors introduced several new entities and categories.Tumors of uncertain differentiation are a new subcategory that includes the intracranial mesenchymal tumor, FET-CREB fusion-positive; the CIC-rearranged sarcoma; and the Primary intracranial sarcoma, DICER1-mutant.
Methods:
A search was made in Pubmed and Google Scholar to include all articles with the term "uncertain differentiation", "Mesenchymal, non-meningothelial", "FET-CREB fusion positive", "DICER1-mutant sarcoma" and "CIC-Rearranged sarcoma" in their title. These articles were reviewed to draft a concise review on this subject.
Results:
This review on CNS non-meningothelial mesenchymal tumors is meant to provide an update with diagnostic, prognostic, and therapeutic implications.
Conclusion:
Tumors of uncertain differentiation include a variety of mesenchymal, non-meningothelial tumors that have distinct molecular characteristics and consequently behave in a very particular matter.Given that these tumors have been described only recently, there is still an important lack of information regarding the most appropriate treatment and prognosis.
