Mantle cell lymphoma with central nervous system involvement: frequency and clinical features

Abstract

SummaryReported rates of central nervous system (CNS) involvement in mantle cell lymphoma (MCL) are highly variable but substantial (4-26%). Data is lacking regarding risk factors for CNS relapse, and for those patients in whom CNS prophylaxis could be beneficial. We present single institution retrospective analysis of data of baseline features, clinical course, rate of CNS disease and putative risk factors in 62 patients with MCL (18 female, 44 male). CNS disease (all cases were symptomatic) occurred in four patients at a median of 12 months (range 1-58) from diagnosis, with a crude incidence of 6AE5% and 5-year actuarial incidence of 5 ± 3%. Two cases had blastic MCL at diagnosis. Survival after CNS relapse ranged from 2-9 months. Patients who developed CNS disease had a significantly shorter survival from diagnosis than those who did not (P = 0AE0024). Symptomatic CNS disease in patients with MCL either at presentation or relapse is an uncommon but devastating complication. In younger patients, more aggressive immunochemotherapy regimens containing CNS-penetrating agents may reduce the incidence of CNS disease. While not routinely justified for all patients, CNS prophylaxis may particularly benefit patients with blastic histology at diagnosis, or those with systemic relapse after first-line treatment.

Central Nervous System Prophylaxis in Non-Hodgkin Lymphoma: Who, What, and When?

“…Blastoid histology stands out as most consistently identified risk factor in smaller studies [84][85][86][87]. Chihara et al analyzed a large cohort of Japanese patients with MCL and found Ki67≥ 30 % to be the only predictor of CNS progression by multivariate analysis (HR 6.0 [95 % CI 1.9-19.4]).…”

Biopsy-Proven Mantle Cell Lymphoma in Brain Parenchyma

“…Additionally, CSF analysis and radiographic imaging may be suffi cient to diagnose CNS involvement when it is present. In our review of the literature, we found four cases of mass lesions in the brain thought to be caused by MCL; however, none of these studies involved a tissue diagnosis (1,7,9). Since brain parenchymal involvement by non-Hodgkin's lymphoma is rare, Doolittle et al suggested that the clinician should strongly consider doing a stereotactic brain biopsy with microscopic examination (9).…”

“…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).…”

“…Th e blastoid variant is associated with aggressive disease and is a predictor of CNS involvement. CNS disease is often identifi ed via analysis of the cerebrospinal fl uid (CSF), radiographic imaging studies, and clinical symptoms (1 Th e patient underwent a supraclavicular lymph node core needle biopsy. Immunostains showed cyclin-D1 positivity and an 80% positive MIB-1/Ki-67 (Figure 1).…”

Neurological Complications of Non-Hodgkin Lymphoma

“…The propensity for CNS involvement by Burkitt's lymphoma has also been well described, with an 8-10% incidence at diagnosis, and up to 30-50% throughout the course of disease [12]. Mantle cell lymphoma (MCL) has been reported to be associated with an unexpected high risk of CNS involvement, estimated at 4-6% [13]; Feugier et al 2004; [14,15]. However, such risks may be attributed to poor systemic control of disease, and CNS relapse in MCL is rare with modern therapies.…”