Central nervous system involvement in mantle cell lymphoma

Abstract

Background: Extranodal involvement, including central nervous system (CNS), is a frequent event in patients with mantle cell lymphoma (MCL). However, the incidence, risk factors, and impact on outcome remain controversial. Patients and methods:Main clinical, biological, and evolutive features of 82 patients (60 males/22 females; median age: 61 years) diagnosed with MCL (blastoid, 26%) in a single institution were analyzed for risk of CNS involvement and prognosis. Results: Most patients had advanced stage and intermediate or high-risk International Prognostic Index (IPI). Elevenpatients eventually developed CNS involvement with an actuarial 5-year risk of 26% (95% confidence interval 10% to 42%). In one asymptomatic patient, cerebrospinal fluid infiltration was detected at staging maneuvers (1/62; 1.6%). The remaining 10 patients developed neurological symptoms during the course of the disease (median time from diagnosis, 25 months). Initial variables predicting CNS involvement were blastoid histology, high proliferative index measured by Ki-67 staining, high lactate dehydrogenase (LDH) and intermediate-or high-risk IPI. Histological subtype and serum LDH maintained significance in multivariate analysis. Treatment of CNS infiltration consisted of intrathecal chemotherapy (two cases), and intrathecal chemotherapy plus systemic treatment (seven cases). Median survival after CNS involvement was 4.8 months, patients with this complication having shorter survival than those with no CNS disease.Conclusion: This study confirms the high incidence of CNS involvement in MCL patients. Treatments aimed at preventing this complication are warranted.

Translocations targeting CCND2, CCND3, and MYCN do occur in t(11;14)-negative mantle cell lymphomas

“…The reported CNS involvement in MCL varied between 4% and 22% in retrospective analyses. 19,21,22 In a recent study of Ferrer et al, 23 CNS involvement was detected in 13% of patients (11 of 83) with MCL; that is, 6 (8%) of 62 typical MCLs and 5 (23%) of 21 blastoid MCLs. Our findings indicate that CNS infiltration in t(11;14)-negative MCL heralds a poor prognosis, similar to that in t(11;14)-positive MCL.…”

Biopsy-Proven Mantle Cell Lymphoma in Brain Parenchyma

“…Th ere was a 6-month time window from the initial diagnosis of MCL to CNS relapse, which is a shorter interval than the median in previous reports, in which relapse occurred at 12 months (1), 18 months (3), and 25 months (2). In a retrospective series of 62 MCL patients, four patients developed CNS relapse at a median of 12 months (range, 1-58 months), including two patients with confi rmed blastic variant (1).…”

“…All four of these patients had positive brain magnetic resonance imaging (one patient with blastic variant had multiple parenchymal lesions in the brain, while the other three patients had exclusively leptomeningeal involvement) (1). In a series by Ferrer et al (2007), which followed 82 MCL patients for 5 years, 10 developed CNS involvement (including 5 of 26 patients with blastic variant) with a median of 25 months from diagnosis (range, 5-130 months) (2).…”

“…antle cell lymphoma (MCL) accounts for approximately 5% to 10% of non-Hodgkin's lymphoma cases (1)(2)(3)(4). Typically MCL presents in the fi fth to sixth decades of life with generalized lymphadenopathy and extranodal involvement (bone marrow, gastrointestinal tract, blood) (5).…”

Mantle cell lymphoma: 2015 update on diagnosis, risk‐stratification, and clinical management

“…Evaluation of the gastrointestinal tract with endoscopy is warranted if there are clinical symptoms or if a dose intense regimen will be used. Evaluation of the cerebral spinal fluid is not usually done unless there are neurologic symptoms or if the patient has the blastoid variant or a high Ki-67 [20]. The majority of patients have stage III or IV disease after complete staging is completed.…”