Purpose Primary central nervous system lymphoma (PCNSL) of the cerebellopontine angle (CPA) is rare, thus the preoperative diagnosis is still a challenge.MethodsWe searched the database at our institution and performed a search of English literature in PubMed and Google Scholar. Keywords used were as follows: "primary central nervous system lymphoma"; "cerebellopontine angle"; "lymphoma". Only cases in the English language that were located in the CPA and contained adequate clinical information

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... pertinent to the analysis were included.Results297 cases of pathologically confirmed PCNSLs were recorded between January 2009 and October 2020 at our institution. 6 cases were located in the CPA, accounting for 2.0% of all PCNSLs. 26 cases meeting the above criteria were found in the literature. Including ours, a total of 32 patients were analyzed. Females were more frequently affected (F/M ratio, 2.2:1). There was a preponderance of left-sided lesions in the PCNSLs of the CPA (L/R ratio, 1.5:1). On CT, 80.0% of them presented as hyperdense lesions. On T1-weighted image, 66.7% appeared isointense. While on T2-weighted image (T2WI), 68.4% appeared isointense/hypointense. After contrast administration, 86.2% presented intense homogeneous enhancement.ConclusionPCNSL of the CPA is extremely rare, accounting for 2.0% of all PCNSLs in our study. There is a preponderance of females and left-sided lesions in this disease. Contrast-enhanced magnetic resonance imaging with T2WI is very helpful in the preoperative diagnosis of the CPA PCNSL. Although rare, lymphoma should be included in the differential diagnosis of CPA lesions.