CNS Hodgkin lymphoma

Primary CNS lymphoma (PCNSL) is a rare malignancy with peculiar clinical and biologic features, aggressive course, and unsatisfactory outcome. It represents a challenge for multidisciplinary clinicians and scientists as therapeutic progress is inhibited by several issues. Molecular and biologic knowledge is incomplete, limiting the identification of new therapeutic targets, and the particular microenvironment of this malignancy, and sanctuary sites where tumor cells grow undisturbed, strongly affects treatment efficacy. Moreover, active treatments are known to be associated with disabling neurotoxicity, posing the dilemma of whether to intensify therapy to improve the cure rate or to de-escalate treatment to avoid sequels. The execution of prospective trials is also difficult because of the rarity of the tumor and the impaired general condition and poor performance status of patients. Thus, level of evidence is low, with consequent uncertainties in therapeutic decisions and lack of consensus on primary endpoints for future trials.Despite this unfavorable background, laboratory and clinical researchers are coordinating efforts to develop new ideas, resulting in the recent publication of studies on PCNSL's biology and molecular mechanisms and of the first international randomized trials. Herein, these important contributions are analyzed to provide recommendations for everyday practice and the rationale for future trials. (Blood. 2011;118(3):510-522) IntroductionPrimary CNS lymphoma (PCNSL) is an aggressive malignancy arising exclusively in the CNS, that is, the brain parenchyma, spinal cord, eyes, cranial nerves, and/or meninges. 1 This lymphoma represents 4% of intracranial neoplasms and 4%-6% of all extranodal lymphomas but some registry studies suggest that its incidence in immunocompetent patients is progressively increasing. 2 PCNSL exhibits peculiar clinical and biologic features and constitutes a diagnostic and therapeutic challenge for multidisciplinary clinicians and scientists. Its outcome remains unsatisfactory if compared with that of patients with extra-CNS lymphomas of a similar stage and histotype, and several factors prevent therapeutic progress. First, molecular and biologic knowledge is insignificant compared with other lymphomas, which limits the identification of new therapeutic targets. Second, patients with PCNSL usually exhibit impaired general condition and poor performance status (PS) more often than subjects with other lymphomas. 1 This usually interferes with the enrollment of patients in prospective trials. In fact, current therapeutic knowledge is only based on 3 randomized trials, some single-arm phase 2 trials, anecdotal meta-analyses, and a few multicenter retrospective studies. Thus, available level of evidence is low, with consequent uncertainties in therapeutic decisions and lack of consensus on primary endpoints for future trials.In this manuscript, I discuss available clinical and biologic data on PCNSL in immunocompetent patients and provide recommendations for everyday pract...

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“…74 WBRT 35-45 Gy followed by a 5-15 Gy boost was proposed for CNS Hodgkin lymphoma, with a median OS from CNS involvement of 44 months. 75 …”

Section: Who Are the Best Candidates For Radiation Therapy Alone?mentioning

confidence: 99%

How I treat primary CNS lymphoma

Ferreri¹

2011

Blood

161

109

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“…Parenchymal lesions, when present, typically result from secondary involvement from the leptomeninges via infiltration of the perivascular spaces 1 . We emphasize the finding of three cases of intracranial Hodgkin's lymphoma (Fig 3), since most cases of secondary CNS involvement by Hodgkin's lymphoma are late manifestations of disseminated disease and primary CNS involvement by this type of lymphoma is very rare 1,13,14 .…”

Section: A B Discussionmentioning

confidence: 75%

Central nervous system lymphoma: magnetic resonance imaging features at presentation

Schwingel

Reis

Zanardi

et al. 2012

Arq. Neuro-Psiquiatr.

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OBJECTIVE: This paper aimed at studying presentations of the central nervous system (CNS) lymphoma using structural images obtained by magnetic resonance imaging (MRI). METHODS: The MRI features at presentation of 15 patients diagnosed with CNS lymphoma in a university hospital, between January 1999 and March 2011, were analyzed by frequency and cross tabulation. RESULTS: All patients had supratentorial lesions; and four had infra- and supratentorial lesions. The signal intensity on T1 and T2 weighted images was predominantly hypo- or isointense. In the T2 weighted images, single lesions were associated with a hypointense signal component. Six patients presented necrosis, all of them showed perilesional abnormal white matter, nine had meningeal involvement, and five had subependymal spread. Subependymal spread and meningeal involvement tended to occur in younger patients. CONCLUSION: Presentations of lymphoma are very pleomorphic, but some of them should point to this diagnostic possibility.

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“…In a retrospective case series derived from 18 cancer centers in five countries of 40 patients with low-grade primary CNS lymphoma, a better long-term outcome was shown (7-year median survival) than is associated with the usual aggressive CNS lymphoma [6]. Anecdotal cases of primary CNS Hodgkin lymphoma have also been reported [7].…”

Section: Discussionmentioning

confidence: 99%

Primary cerebellar B cell lymphoma: A case report

Datta¹,

Gupta²,

Choudhury³

et al. 2013

IJCRI

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Introduction: Primary central nervous system lymphoma (PcNsL) is usually a diffuse large b cell non-Hodgkin lymphoma that originates in the brain, spinal cord, leptomeninges or eyes. Primary central nervous system lymphoma is a rare malignant tumor comprising less than 3% of all primary brain tumors. the PcNsL in immunocompetent patients is most commonly supratentorial, found adjacent to the ventricular surfaces and in deep white matter and subcortical structures, such as the basal ganglia, thalamus and corpus callosum. the lesion is single in 60-70% of patients. Lesions are located in the hemispheres (38%), thalamus/basal ganglia (16%), corpus callosum (14%), periventricular region (12%) and cerebellum (9%). Herein, we report an extremely rare case of a primary cNs b cell lymphoma involving cerebellum. case report: A 55-year-old female was presented with vomiting, ataxia and disorientation. t2-

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CNS Hodgkin lymphoma

Cited by 79 publications

References 9 publications

How I treat primary CNS lymphoma

How I treat primary CNS lymphoma

Central nervous system lymphoma: magnetic resonance imaging features at presentation

Primary cerebellar B cell lymphoma: A case report

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