Neuroradiological features of lymphomatosis cerebri: A systematic review of the English literature with a new case report

Abstract

Lymphomatosis cerebri is a rare form of diffusely infiltrating primary central nervous system (CNS) lymphoma (PCNSL). The neuroradiological findings of lymphomatosis cerebri have not been adequately characterized, as the relevant literature consists only of case reports and small case series. The present study describes an unusual presentation of lymphomatosis cerebri in a 56-year-old immunocompetent woman who presented with diffusely infiltrating lesions with perivascular curvilinear enhancement on initial magnetic resonance imaging (MRI) and multiple nodules on the later follow-up computed tomography (CT) scan. A systematic review of the literature is also performed searching PubMed between January 1996 and December 2016 to collect all pertinent case reports and series written in the English language with pathologically confirmed lymphomatosis cerebri and diffuse infiltrative PCNSL without cohesive masses on initial MRI. A total of 45 cases were identified from 39 articles and the present case report. The patient ages ranged from 28 to 85 years (mean, 57.3 years). Only 3 patients (6.7%) were immunosuppressed (acquired immune deficiency syndrome patients). The most common clinical presentation was cognitive changes or dementia (46.7%). Cerebrospinal fluid analysis in all cases was non-specific. Diffuse and asymmetric abnormal T2-hyperintensity in deep and subcortical white matter was observed in all cases. Gray matter involvement (17.8%), spreading along the corticospinal tract (35.6%) and a slight mass effect (51.1%) also were observed. Contrast-enhanced patterns on MRI could be divided into three forms of non-enhancement (64.4%) and non-mass-like enhancement (35.6%) on initial MRI, as well as nodular or mass-like enhancement on the later follow-up MRI (15.6%). There were non-specific findings on magnetic resonance spectroscopy for 4 patients, on positron emission tomography/CT for 12 patients and on single-photon emission CT for 1 patient. Diagnosis was established by brain biopsy in 35 cases (77.8%) and autopsy in 9 cases (20%), involving B-cell lymphoma in 40 cases (88.9%) and T-cell lymphoma in 4 cases (8.9%). In conclusion, lymphomatosis cerebri, namely diffuse PCNSL or diffuse lymphoma of the CNS, is characterized by rapidly progressive dementia in the elderly, diffusely infiltrated CNS white matter along the corticospinal tract, possible involvement of the gray matter, a slight mass effect and varied contrast-enhancement patterns on MRI. Non-enhancement or non-mass-like enhancement on MRI may be a special form of diffuse PCNL during disease development and progression.

Diagnosis and treatment of primary central nervous system lymphoma with the primary lesion in the hypothalamus: a case report

“…PCNSL lesions usually appear in the vicinity of the ventricle, and are often multiple. Initial symptoms may include subclinical intracranial pressure and psychiatric conditions in addition to other conditions originating in the brain such as paralysis and aphasia [ 5 ]. PCNSL with hypothalamic-pituitary lesions has been rarely reported [ 6 ].…”

Pathologically Proven Gadolinium-enhanced MRI Lesions in the Bilateral Corticospinal Tracts in Lymphomatosis Cerebri

“…2a ), we assume that the BBB might have been disrupted by the aggressive invasion of these cells, and that this disruption corresponded to the robust contrast enhancement on MRI. This differs from the non-contrast-enhancing lesions in typical LC, where diffusely infiltrative lymphoid cells do not affect the structure of the BBB ( 4 ).…”

“…Originally, the MRI features of LC were reported to consist of diffuse leukoencephalopathy in the cerebral hemispheres with no contrast enhancement, reflecting an intact blood-brain barrier (BBB) ( 2 ). However, subtle, patchy, or even mass-like enhanced lesions have been reported in the later stages of LC ( 4 - 7 ). A recent case series characterized the neuroradiological findings of LC and revealed that a relatively high percentage of patients (35.6%) demonstrated spreading of lymphoid cells along the corticospinal tracts ( 4 ).…”

“…However, subtle, patchy, or even mass-like enhanced lesions have been reported in the later stages of LC ( 4 - 7 ). A recent case series characterized the neuroradiological findings of LC and revealed that a relatively high percentage of patients (35.6%) demonstrated spreading of lymphoid cells along the corticospinal tracts ( 4 ). The patient in our case presented with an impressively robust enhancement of the bilateral corticospinal tracts following the administration of a gadolinium contrast agent.…”

Distribution of Deep Gray Matter Lesions on Magnetic Resonance Imaging in Lymphomatosis Cerebri

“…On MRI, LC is characterized by diffuse, non-enhancing infiltrative lesions without mass formation or mass effect (1)(2)(3)(4)(5)(6)(7)(8)(9). The lesions of LC are likely to be most clearly demonstrated with a fluid-attenuated inversion recovery (FLAIR) sequence (2,3). The lesions also show a high signal intensity on diffusion-weighted imaging (DWI), and most of the lesions exhibit an increase in apparent diffusion coefficient (ADC) values on an ADC map (4), which may reflect the diffuse infiltration of non-cohesive malignant lymphoid cells (4).…”