Primary CNS lymphoma other than DLBCL: a descriptive analysis of clinical features and treatment outcomes

Abstract

Diffuse large B-cell lymphoma (DLBCL) constitutes most primary central nervous system (CNS) lymphoma (PCNSL), whereas T-cell, low-grade and Burkitt’s lymphomas (BL) are rarely encountered. Due to the paucity of cases, little is known about the clinical features and treatment outcomes of PCNSL other than DLBCL. The objective of this study was to describe the clinical characteristics and outcomes for patients with PCNSL other than DLBCL. Fifteen patients, newly diagnosed with PCNSLs other than DLBCL between 2000 and 2010, were included. The male to female ratio was 0.67:1 with a median age of diagnosis of 31 years (range 18–59). Pathologic distributions were as follows: peripheral T-cell lymphoma (PTCL; n = 7), marginal zone B-cell lymphoma (MZBCL; n = 1), lymphoplasmacytic lymphoma (LPL; n = 2), Burkitt’s lymphoma (n = 1), other unspecified (T-cell lineage, n = 2; B-cell lineage, n = 2). Thirteen patients (87%) showed Eastern Cooperative Oncology Group performance score (ECOG PS) 1–2. The remaining two were one PTCL patient and one Burkitt’s lymphoma patient. Of the nine patients with T-cell lymphoma, five (56%) had multifocal lesions, and one (20%) with LPL of the five patients with B-cell lymphoma showed a single lesion. Leptomeningeal lymphomatosis was identified in two patients (one with Burkitt’s lymphoma and one with unspecified B-cell lymphoma). Two patients (22%) with T-cell lymphoma died 7.7 and 23.3 months later, respectively, due to disease progression, despite HD-MTX-based therapy. Six patients with T-cell lymphoma (6/9, 66.7%) and four patients with low-grade B-cell lymphoma (4/5, 80%) achieved complete response and have survived without relapse (Table 3). One patient with Burkitt’s lymphoma showed poor clinical features with ECOG PS 3, deep structure, multifocal, and leptomeningeal lymphomatosis, and died 7.6 months after the initiation of treatment. In comparison with previously reported DLBCLs (median OS 6.4 years, 95% CI 3.7–9.1 years), T-cell lymphoma showed equivocal or favorable clinical outcomes and low-grade B-cell lymphomas, such as MZBCL and LPL, had a good prognosis. However, primary CNS Burkitt’s lymphoma presented poor clinical outcomes and showed a comparatively aggressive clinical course. In conclusion, primary CNS lymphoma other than DLBCL occurred more in younger patients and showed a generally good prognosis, except for Burkitt’s lymphoma. Further research on treatment strategies for Burkitt’s lymphoma is needed.

An unusual presentation of primary malignant B-cell-type dural lymphoma

“…(2,3) PCNSL is histopathologically indistinguishable from NHLs that occur at other body sites, (4) and accounts for up to 1% of NHLs, 3%-6.6% of all primary cerebral neoplasms, (1,2,5) and less than 5% of all lymphomas.…”

“…(1) More than 95% of PCNSLs are diffuse large B-cell lymphomas; (2,4) congenital or acquired immunodeficiency syndromes are well-recognised risk factors. (5) Other factors contributing to its pathogenesis include latent infection by EBV in immunosuppressed patients, the presence of B cell-attracting chemokine BCA-1, and B-cell growth factors such as interleukin 4.…”

“…(2,3) We herein report the case of an immunocompetent man who presented with what was initially diagnosed to be a chronic subdural haematoma, but which turned out to be a primary B-cell lymphomatous lesion. In view of this unexpected diagnosis, we also reviewed the present case in the context of the relevant literature.…”

T-cell primary leptomeningeal lymphoma in cerebellopontine angle

“…Its incidence is unknown (less than 1% of all intracranial tumours) but has apparently increased during the last two decades in immunocompromised and in immunocompetent patients 1 5. Diffuse B cell lymphomas are the most common type (>90%) while those derived from T cells are much more infrequent (<5%) 3 5. The prevalence of T-cell PCNSL is higher is eastern countries such as Japan (14%) compared with western countries 3 6.…”

“…Clinical presentation may occur with a variety of focal or non-focal neurological symptoms being the most frequent; such as hearing loss (resembling a vestibular schwannoma). Cerebellar signs such as ataxia or unsteady gait are the second most common symptoms but without B symptoms such as fever or night sweats 1 5. The most frequent aetiology of opsoclonus-myoclonus syndrome is the idiopathic cause and paraneoplastic manifestation of the underlying malignancy 9.…”