Background: Primary central nervous system (CNS) small lymphocytic lymphoma (SLL), as a type of low-grade\nlymphoma, is extremely rare. The diagnosis of CNS SLL is challenging due to its variable clinical and radiological\nfeatures, which may overlap with those of diffuse large B-cell lymphoma (DLBCL). Primary CNS SLL differs from\nDLBCL in that it has an indolent clinical course and a good prognosis. Thus, it is important to distinguish SLL from\nDLBCL. By reviewing the literature, only two cases of low-grade SLL, primarily located in the CNS and involving the\nbrain parenchyma and dura, have been reported. To our knowledge, primary CNS SLL in the bilateral ventricles has\nnever been reported. Interestingly, the two cases in our report are identical in terms of the clinical presentations,\nmagnetic resonance imaging (MRI) features, pathological results and prognoses.\nCase presentation: Both patients presented with headaches. MRI suggested solid lesions located in the bilateral\nventricles that were isointense on T1-weighted images and hypointense on T2-weighted images. After the injection\nof contrast agent (gadolinium, Gd), the intraventricular lesions were homogeneously enhanced and hyperperfused.\nCSF cytology revealed malignant cells. Brain biopsy revealed diffuse proliferation of small lymphocytes with positive\nlabelling of B-cell immunomarkers. The primary origin in the CNS was confirmed with no evidence of systemic\nlymphoma. Two patients were given high doses of methotrexate-based chemotherapy and were free from\nsymptoms and progression for more than 1-year of follow-up.\nConclusions: The presence of homogeneously enhanced intraventricular MRI lesions should raise the suspicion of\nprimary CNS SLL.
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