Can intravascular lymphomatosis mimic sinus thrombosis? A case report with 8 months' follow-up and fatal outcome

Abstract

We report a case of intravascular lymphomatosis of the brain with 8 months' follow-up and fatal outcome. Several MRI investigations revealed variegated, rapidly changing infarct-like lesions and invasion of the walls of the superior sagittal sinus and deep veins. When disturbances of the venous outflow are detected with multifocal infarct-like lesions, intravascular lymphomatosis should be considered in the differential diagnosis. Brain biopsy may ensure the proper diagnosis ante mortem, but failure of biopsy is frequent, as in our case.

Intravascular lymphomatosis of the nervous system

“…The central nervous system is affected in almost two-thirds of cases in Western patients with IVL [1][2][3]5]. As represented by patients 2 and 3, signs caused by obliteration of vessels can remit spontaneously or to steroid treatment, which could erroneously be related to other diseases (Table 1) [4,[7][8][9][10]. Symptoms of polyradiculopathy acutely evolved in our case in contrast to previous reports, and mimicked GBS [11].…”

“…Cases of patients 1 and 2 denote the variable and changing neuroimaging profile (Fig. 1 A) [7,10,13]. Despite suspicion, histopathological diagnosis of IVL could not be made premortem in patient 2, which represents the diagnostic challanges: since obliteration of vessels is only transient and parenchymal invasion rare, stereotactic biopsy may show only necrosis or hemorrhage [1].…”

Intravascular lymphoma: magnetic resonance imaging correlates of disease dynamics within the central nervous system

“…Dural and/or leptomeningeal involvement as indicated by gadolinium enhancement was present in a small number of patients, 4 11 17 as were foci of haemorrhage. 16 Few cases with normal cerebral MRI scans at initial evaluation were reported either with or without neurological deficits. 16 Asymptomatic MR lesions appear to be rare (our case; reference 10) but this number is likely underestimated since complete staging evaluations including imaging of the whole neuraxis are not widely performed.…”

Usefulness of FDG‐PET to diagnose intravascular lymphomatosis presenting as fever of unknown origin

“…The proliferation in the blood vessels of parenchymatous organs results in thrombosis and secondary hypoxic damage [3], and the nervous system and skin have a high incidence of involvement. Infiltration of the lungs, kidneys, adrenal glands, and prostate is also common, but the liver, spleen, bone marrow, brain, and lymph nodes are typically spared until late in the disease [4,5].…”