A Systematic Review of the Outcomes of Mucosa‐Associated Lymphoid Tissue Intracranial Lymphomas

Background Mucosa‐associated lymphoid tissue (MALT) lymphoma in the central nervous system (CNS) is rare. As part of the non‐Hodgkin B cell lymphoma group, it is much more indolent in presentation and progression than its aggressive cousin diffuse B‐cell lymphoma (DBCL) of the CNS. Methods This systematic review collates all the published outcome and treatment data on CNS MALT to shed more light on this rare entity. Results With 3‐year overall survival rates of over 94%, females represent two thirds of cases. Dural involvement is much more common and carries a positive prognostic advantage, as does younger age. Treatment strategies still appear to be widely varied, but surgery and adjuvant therapy appear to provide the longest lasting progression‐free survival. Conclusion The rare entity of CNS MALT lymphoma can present in a range of ways. While this means a variety of symptoms, its general prognostic course is usually more indolent than other B cell lymphomas. It frequently affects a younger and more female predominant population, yet no demographic, presentation, or histological characteristics are associated with poorer prognosis. Its rare nature means collaborative databases will need to be considered to better collect robust data for quantitative analysis on ideal treatment and prognosis.