CNS lymphoma consists of 2 major subtypes: secondary CNS involvement by systemic lymphoma and PCNSL. Contrast-enhanced MR imaging is the method of choice for detecting CNS lymphoma. In leptomeningeal CNS lymphoma, representing two-thirds of secondary CNS lymphomas, imaging typically shows leptomeningeal, subependymal, dural, or cranial nerve enhancement. Single or multiple periventricular and/or superficial contrast-enhancing lesions are characteristic of parenchymal CNS lymphoma, representing one-third of secondary CNS lymphomas and almost 100% of PCNSLs. New CT and MR imaging techniques and metabolic imaging have demonstrated characteristic findings in CNS lymphoma, aiding in its differentiation from other CNS lesions. Advanced imaging techniques may, in the future, substantially improve the diagnostic accuracy of imaging, ultimately facilitating a noninvasive method of diagnosis. Furthermore, these imaging techniques may play a pivotal role in planning targeted therapies, prognostication, and monitoring treatment response.

http://www.ajnr.org/content/ajnr/32/6/984.full.pdf

Central Nervous System Lymphoma: Characteristic Findings on Traditional and Advanced Imaging

Distinguishing of primary cerebral lymphoma from high-grade glioma with perfusion-weighted magnetic resonance imaging.

Advanced magnetic resonance (MR) imaging techniques provide physiologic information that complements the anatomic information available from conventional MR imaging. We evaluated the roles of diffusion and perfusion imaging for the assessment of grade and type of histologically proven intraaxial brain tumors. A total of 28 patients with intraaxial brain tumors underwent conventional MR imaging (T2- and T1-weighted sequences after gadobenate dimeglumine injection), diffusion imaging and T2*-weighted echo-planar perfusion imaging. Examinations were performed on 19 patients during initial diagnosis and on nine patients during follow-up therapy. Determinations of relative cerebral blood volume (rCBV) and apparent diffusion coefficient (ADC) were performed in the solid parts of each tumor, peritumoral region and contralateral white matter. For gliomas, rCBV values were greater in high-grade than in low-grade tumors (3.87+/-1.94 versus 1.30+/-0.42) at the time of initial diagnosis. rCBV values were increased in all recurrent tumors, except in one patient who presented with a combination of recurrent glioblastoma and massive radionecrosis on histology. Low-grade gliomas had low rCBV even in the presence of contrast medium enhancement. Differentiation between high- and low-grade gliomas was not possible using diffusion-weighted images and ADC values alone. In the peritumoral areas of untreated high-grade gliomas and metastases, the mean rCBV values were higher for high-grade gliomas (1.7+/-0.37) than for metastases (0.54+/-0.18) while the mean ADC values were higher for metastases. The rCBV values of four lymphomas were low and the signal intensity-time curves revealed a significant increase in signal intensity after the first pass of gadobenate dimeglumine. Diffusion and perfusion imaging, even with relatively short imaging and data processing times, provide important information for lesion characterization.

Clinical relevance of diffusion and perfusion magnetic resonance imaging in assessing intra-axial brain tumors.

Posttransplant lymphoproliferative disorders (PTLDs) represent a spectrum ranging from Epstein-Barr virus (EBV)-driven polyclonal lymphoid proliferations to EBV+ or EBV- malignant lymphomas. Central nervous system (CNS) PTLDs have not been characterized fully. We reviewed the clinical, radiologic, and pathologic features of 12 primary CNS PTLDs to define them more precisely. Patients included 10 males and 2 females (median age, 43.4 years) who were recipients of kidney (n = 5), liver (n = 2), heart (n = 2), peripheral blood stem cells (n = 2), or bone marrow (n = 1). All received immunosuppressive therapy. CNS symptoms developed 3 to 131 months (mean, 31 months) after transplantation. By neuroimaging, most showed multiple (3 to 9) intra-axial, contrast-enhancing lesions. Histologic sections showed marked expansion of perivascular spaces by large, cytologically malignant lymphoid cells that were CD45+, CD20+, EBV+ and showed light chain restriction or immunoglobulin gene rearrangement. In distinction to PTLDs in other organ systems, CNS PTLDs were uniformly high-grade lymphomas that fulfilled the World Health Organization criteria for monomorphic PTLDs. Extremely short survival periods were noted for each CNS PTLD that followed peripheral blood stem cell transplantation. Survival of others with CNS PTLD varied; some lived more than 2 years.

Primary central nervous system posttransplant lymphoproliferative disorders.

Central nervous system (CNS) lymphoma can present a diagnostic challenge. Currently, there is no consensus regarding what presurgical evaluation is warranted or how to proceed when lesions are not surgically accessible. We conducted a review of the literature on CNS lymphoma diagnosis (1966 to October 2011) to determine whether a common diagnostic algorithm can be generated. We extracted data regarding the usefulness of brain and body imaging, serum and cerebrospinal fluid (CSF) studies, ophthalmologic examination, and tissue biopsy in the diagnosis of CNS lymphoma. Contrast enhancement on imaging is highly sensitive at the time of diagnosis: 98.9% in immunocompetent lymphoma and 96.1% in human immunodeficiency virus-related CNS lymphoma. The sensitivity of CSF cytology is low (2%-32%) but increases when combined with flow cytometry. Cerebrospinal fluid lactate dehydrogenase isozyme 5, β2-microglobulin, and immunoglobulin heavy chain rearrangement studies have improved sensitivity over CSF cytology (58%-85%) but have only moderate specificity (85%). New techniques of proteomics and microRNA analysis have more than 95% specificity in the diagnosis of CNS lymphoma. Positive CSF cytology, vitreous biopsy, or brain/leptomeningeal biopsy remain the current standard for diagnosis. A combined stepwise systematic approach outlined here may facilitate an expeditious, comprehensive presurgical evaluation for cases of suspected CNS lymphoma.

https://jamanetwork.com/journals/jamaneurology/articlepdf/1556618/nnr120005_311_319.pdf

A Systematic Approach to the Diagnosis of Suspected Central Nervous System Lymphoma

Because of the increasing incidence of primary central nervous system lymphoma (PCNSL), it is essential to recognize this disease in order to start appropriate treatment. We present the characteristic CT and MRI features of this tumour. The findings of 32 CT and 31 MR of 37 immunocompetent patients with biopsy-proved PCNSL are reviewed. The main features are presented and analysed, and are discussed in comparison with proven literature data. Primary central nervous system lymphoma presents as supratentorial solitary lesions in approximately 80% of the patients and multiple lesions in 20%. In contrast to classical data, the lesions are located in deep structures only in one-third of the cases, and involve posterior fossa in 10% of cases. Most of the lesions are hyperdense or isodense (92%) on CT, hypointense or isointense on T1-weighted images, and only about 40% are hyperintense on T2-weighted images. Nearly all the lesions enhance, except after corticosteroid administration. They produce mild oedema and mass effect. Meningeal or ventricular enhancement are rare but suggestive. Calcification, haemorrhage or necrosis are scarce. Although PCNSL in immunocompetent patients have a variable CT and MR appearance, the imaging data often suggest the diagnosis.

J. Blustajn

Papers

Radiographic findings in 37 cases of primary CNS lymphoma in immunocompetent patients.