CASE 16813 Published on 29.06.2020

Atypical form of primary cns lymphoma: lymphomatosis cerebri

Section

Head & neck imaging

Case Type

Clinical Cases

Authors

Authors: Diana Veiga Canuto, Joan Carreres Polo, Luis Martí-Bonmatí

Department of Radiology. Hospital Universitari i Politècnic La Fe, Valencia, Spain.

 

Patient

74 years, male

Categories
Area of Interest CNS ; Imaging Technique MR
Clinical History

A 74-year-old-man with history of headache and fever in the last 3 months that did not respond to antibiotics came to our institution referring to clinical worsening with generalized weakness and altered consciousness.

Imaging Findings

An MRI was performed. T2 and Fluid-attenuated Inversion Recovery (FLAIR) sequences showed patchy and coalescent hyperintensity in subcortical and deep bihemispherical white matter, basal ganglia and corpus callosum. It associated patchy nodular zones of restricted diffusion adjacent to splenium of corpus callosum and left semioval center, and two different zones of contrast enhancement in left semioval centers and anterior left commissure.

Discussion

Brain biopsy was performed and showed perivascular and diffuse infiltration of lymphoid cells, thus, diagnosis of diffuse large B-cell lymphoma was made.

Primary central nervous system (CNS) lymphoma is a rare form of non-Hodgkin lymphoma, usually of diffuse large B-cells, in which brain MRI typically shows single or multiple lesions at the hemispheres or basal ganglia [1]. Nevertheless, primary CNS lymphomas can rarely present as diffuse infiltrative lesions. This uncommon variant was termed in 1999 as lymphomatosis cerebri (LC) [2] as a form of lymphoma that mimics the imaging features of gliomatosis cerebri [3,4]. Pathologically it is seen as diffuse infiltration of the white matter by malignant lymphoid cells [5].

Diagnosis of lymphomatosis cerebri is difficult because of its heterogeneous clinical presentation, such as rapidly progressive dementia, abnormal behavior or gait disturbances [3]. Pleocitosis of cerebrospinal fluid can be detected in the lumbar puncture [1].

Neuroimaging findings are also nonspecific, and misdiagnosis and diagnostic delay are frequent [1]. Thus, it becomes a diagnostic challenge with a wide differential diagnosis to be considered. Typical MRI imaging features consist of extensive diffuse white matter signal abnormalities, with hyperintensity on T2 weighted images, without constitution of a well-defined mass. Infiltrative lymphoma should be considered if there is callosal involvement, patchy enhancement, as well as slight restricted diffusion. Besides, it typically shows a rapid progression [3,5].

Treatment is based on steroids initially, followed by radiotherapy or chemotherapy. Response may be evaluated by MRI [5].

Differential Diagnosis List
Atypical primary CNS lymphoma: lymphomatosis cerebri
Vasculitis
Microangiopathy
Gliomatosis cerebri growth pattern found in many gliomas
Progressive multifocal leukoencephalopaty
Toxic or metabolic white matter lesions
Final Diagnosis
Atypical primary CNS lymphoma: lymphomatosis cerebri
Case information
URL: https://www.eurorad.org/case/16813
ISSN: 1563-4086