Primary central nervous system small lymphocytic lymphoma in the bilateral ventricles: two case reports

Abstract

Background Primary central nervous system (CNS) small lymphocytic lymphoma (SLL), as a type of low-grade lymphoma, is extremely rare. The diagnosis of CNS SLL is challenging due to its variable clinical and radiological features, which may overlap with those of diffuse large B-cell lymphoma (DLBCL). Primary CNS SLL differs from DLBCL in that it has an indolent clinical course and a good prognosis. Thus, it is important to distinguish SLL from DLBCL. By reviewing the literature, only two cases of low-grade SLL, primarily located in the CNS and involving the brain parenchyma and dura, have been reported. To our knowledge, primary CNS SLL in the bilateral ventricles has never been reported. Interestingly, the two cases in our report are identical in terms of the clinical presentations, magnetic resonance imaging (MRI) features, pathological results and prognoses. Case presentation Both patients presented with headaches. MRI suggested solid lesions located in the bilateral ventricles that were isointense on T1-weighted images and hypointense on T2-weighted images. After the injection of contrast agent (gadolinium, Gd), the intraventricular lesions were homogeneously enhanced and hyperperfused. CSF cytology revealed malignant cells. Brain biopsy revealed diffuse proliferation of small lymphocytes with positive labelling of B-cell immunomarkers. The primary origin in the CNS was confirmed with no evidence of systemic lymphoma. Two patients were given high doses of methotrexate-based chemotherapy and were free from symptoms and progression for more than 1-year of follow-up. Conclusions The presence of homogeneously enhanced intraventricular MRI lesions should raise the suspicion of primary CNS SLL.

Refractory Chronic Lymphocytic Leukemia with Central Nervous System Involvement: A Case Report with Literature Review

“…200. 22 49 M Headache and dizziness for past 5 years NA NA NA NA Biopsy CSF + Rtx, HD Mtx, Dex, and vincristine with IT Mtx CR Neuropathology. 2019.39.54.…”

Bilateral lesions of the basal ganglia and thalami (central grey matter)—pictorial review

“…The lesions characteristically show dense homogeneous enhancement and very low diffusion with dark appearance on ADC maps (primarily due to high cellularity). Although the incidence of PCNSL had decreased slightly in the mid-1990s, concordantly with the decreasing rates of AIDS, it has been increasing in the elderly immunocompetent population [65][66][67]. When hyperdense homogeneous mass lesions with prominent surrounding oedema are seen along the ventricular surfaces on non-enhanced CT scan, the possibility of PCNSL should be raised.…”

Clinical Features, Diagnosis, and Treatment of Primary Intraventricular Lymphoma: Insights From a Monocentric Case Series

“…For cluster-like lesions that can be completely resected, surgical resection may also be considered. Given the established and wide application of endoscopic technology, endoscopic resection can be considered, as reported in the literature ( 37 ). A biopsy is recommended as the first choice for patients with diffuse growth and multinodular growth.…”