Learning objectives
To describe the radiologic characteristics of this rare variant of primary central nervous system lymphoma.
To provide the radiologist the main diagnostic clues for the diagnosis of lymphomatosis cerebri with MRI in order to include this entity in their differential diagnostic list of diffuse white matter disorders.
Background
Primary central nervous system lymphoma (PCNSL) is an unusual form of non-Hodgkin type neoplasm,
which represents 6.2%.
Besides brain,
this tumor can also involve meninges,
eyes and spinal cord.
Its appearance in immunocompetent patients in MRI is characterized by the presence of a unique enhancing mass lesion,
whereas,
in immunocompromized patientsthe typical MRI finding is the presence of enhancing lesions.
Lymphomatsis cerebri (LC) is a rare PCNSL presenting as diffuse non-mass like lesion of white matter,
similar seen in gliomatosis cerebri,
a rare glial tumor,...
Findings and procedure details
We performed a retrospective review of 7 cases in order to depict the typical radiologic presentation of this disease.
At MRI,
LC shows hyperintense T2 and FLAIR signal lesions involving the cerebral subcortex and occasionally extend to involve basal ganglia,
thalamus and brainstem (Fig.2,3,4),
reflecting widespread infiltration of the cerebral white matter by lymphomatous cells without contrast enhancement (Fig.5).
However,
subtle or patchy enhancements may be seen in some cases,
in our series 3 of the 7 patients showed contrast enhancement (Fig.6,7).
On diffusion sequences,...
Conclusion
LC can be a diagnostic challenge.
Vague clinical and radiological findings and a lack of awareness about lymphomatosis cerebri may delay the early diagnosis.
Typical MRI findings are a diffuse bilateral leukoencephalopathy,
without contrast enhancement.
Spinal cord and infratentorial lesions can be seen in more than half of cases.
Although the presentation might not be specific,
the diagnosis should be considered in patients with rapidly progressive subcortical dementia.
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