Intravascular Lymphomatosis Mimicking Primary Central Nervous System Lymphoma: A Case Report and Literature Review

Abstract

We herein report a 75-year-old female patient with intravascular lymphomatosis (IVL) who presented with fever of unknown origin. Examination, including contrast-enhanced CT and ⁶⁷Ga scintigraphy, failed to show any lesions. Her blood levels of lactate dehydrogenase and soluble interleukin-2 receptors were high, suggesting a lymphomatous tumor. A bone marrow puncture was negative, and a random skin biopsy revealed a monoclonal proliferation of naked, large lymphocytes in the vascular space of the subcutaneous tissue, confirming the diagnosis of IVL. MRI, performed 7 weeks after admission, showed a brain mass mimicking primary central nervous system lymphoma. The mass was considered to be a collection of malignant lymphocyte cells invading from the vessels. Without the random skin biopsy, this case may have been misdiagnosed as primary central nervous system lymphoma.

Synchronous brain and intravascular B‐cell lymphoma after remission of an adult hemophagocytic syndrome

“…Besides the unusual association of HLH with IVL in European patients, there are other features that do not favor a classical IVL in this case. In fact, brain pathology resembled primary diffuse large B‐cell lymphoma of the CNS and while some cases of IVL have brain mass‐like lesions 11, IVL with secondary CNS involvement usually have brain vessel and leptomeningeal infiltration 9 not seen in this case. It is thus plausible to assume a primary diffuse large B‐cell lymphoma of the CNS with synchronous IVL.…”

Synchronous brain and intravascular B‐cell lymphoma after remission of an adult hemophagocytic syndrome

“…Besides the unusual association of HLH with IVL in European patients, there are other features that do not favor a classical IVL in this case. In fact, brain pathology resembled primary diffuse large B‐cell lymphoma of the CNS and while some cases of IVL have brain mass‐like lesions 11, IVL with secondary CNS involvement usually have brain vessel and leptomeningeal infiltration 9 not seen in this case. It is thus plausible to assume a primary diffuse large B‐cell lymphoma of the CNS with synchronous IVL.…”

Conventional and Advanced Imaging Features of CNS Intravascular Lymphoma

“…Similar to previous studies, the most common dominant brain imaging finding in IVL was infarct-like lesions (68% of our cases), demonstrating multifocality that does not involve the classical vascular territories and showing the persistence of diffusion abnormalities beyond 6 weeks [3][4][5][6][7][9][10][11][12][13][14][15]. Other dominant findings in our series have been reported less frequently, except for enhancing lesions without mass effect resembling lymphomatosis cerebri, which were found in one patient and had not been previously reported in the literature [5][6][7][14][15][16][17][18][19][20].…”

“…Chronic luminal vascular occlusions could contribute to nonspecific white matter abnormalities [7, 14–16]. The extravascular spread of tumor cells may be responsible for other imaging manifestations, including enhancing parenchymal lesions with or without mass effect, abnormal vessel wall enhancement, and leptomeningeal–perivascular enhancement [6, 7, 14, 17–19, 22, 26]. Central pontine T2/FLAIR hyperintensity is likely caused by microvascular occlusive changes in the pons, combined with venous congestion, as shown by a postmortem examination in a previous study [7, 20].…”

Persistent lesion hyperintensity on brain diffusion-weighted MRI is an early sign of intravascular lymphoma

“…Similar findings of persistent hyperintense lesions on DWI were previously reported in two patients with IVL. Oomura et al reported that an enlarging mass-like IVL lesion in the right basal ganglia showed restricted diffusion over 2 weeks 4. Sato et al also reported the growth of infarct-like lesions, which were hyperintense on DWI, over the course of 21 days in a patient with intravascular large B-cell lymphoma 5.…”

Synchronous brain and intravascular B‐cell lymphoma after remission of an adult hemophagocytic syndrome

“…Besides the unusual association of HLH with IVL in European patients, there are other features that do not favor a classical IVL in this case. In fact, brain pathology resembled primary diffuse large B‐cell lymphoma of the CNS and while some cases of IVL have brain mass‐like lesions 11, IVL with secondary CNS involvement usually have brain vessel and leptomeningeal infiltration 9 not seen in this case. It is thus plausible to assume a primary diffuse large B‐cell lymphoma of the CNS with synchronous IVL.…”

Conventional and Advanced Imaging Features of CNS Intravascular Lymphoma

“…Similar to previous studies, the most common dominant brain imaging finding in IVL was infarct-like lesions (68% of our cases), demonstrating multifocality that does not involve the classical vascular territories and showing the persistence of diffusion abnormalities beyond 6 weeks [3][4][5][6][7][9][10][11][12][13][14][15]. Other dominant findings in our series have been reported less frequently, except for enhancing lesions without mass effect resembling lymphomatosis cerebri, which were found in one patient and had not been previously reported in the literature [5][6][7][14][15][16][17][18][19][20].…”

“…Chronic luminal vascular occlusions could contribute to nonspecific white matter abnormalities [7, 14–16]. The extravascular spread of tumor cells may be responsible for other imaging manifestations, including enhancing parenchymal lesions with or without mass effect, abnormal vessel wall enhancement, and leptomeningeal–perivascular enhancement [6, 7, 14, 17–19, 22, 26]. Central pontine T2/FLAIR hyperintensity is likely caused by microvascular occlusive changes in the pons, combined with venous congestion, as shown by a postmortem examination in a previous study [7, 20].…”

Persistent lesion hyperintensity on brain diffusion-weighted MRI is an early sign of intravascular lymphoma

“…Similar findings of persistent hyperintense lesions on DWI were previously reported in two patients with IVL. Oomura et al reported that an enlarging mass-like IVL lesion in the right basal ganglia showed restricted diffusion over 2 weeks 4. Sato et al also reported the growth of infarct-like lesions, which were hyperintense on DWI, over the course of 21 days in a patient with intravascular large B-cell lymphoma 5.…”

Synchronous brain and intravascular B‐cell lymphoma after remission of an adult hemophagocytic syndrome

“…Besides the unusual association of HLH with IVL in European patients, there are other features that do not favor a classical IVL in this case. In fact, brain pathology resembled primary diffuse large B‐cell lymphoma of the CNS and while some cases of IVL have brain mass‐like lesions 11, IVL with secondary CNS involvement usually have brain vessel and leptomeningeal infiltration 9 not seen in this case. It is thus plausible to assume a primary diffuse large B‐cell lymphoma of the CNS with synchronous IVL.…”

Conventional and Advanced Imaging Features of CNS Intravascular Lymphoma

“…Similar to previous studies, the most common dominant brain imaging finding in IVL was infarct-like lesions (68% of our cases), demonstrating multifocality that does not involve the classical vascular territories and showing the persistence of diffusion abnormalities beyond 6 weeks [3][4][5][6][7][9][10][11][12][13][14][15]. Other dominant findings in our series have been reported less frequently, except for enhancing lesions without mass effect resembling lymphomatosis cerebri, which were found in one patient and had not been previously reported in the literature [5][6][7][14][15][16][17][18][19][20].…”

“…Chronic luminal vascular occlusions could contribute to nonspecific white matter abnormalities [7, 14–16]. The extravascular spread of tumor cells may be responsible for other imaging manifestations, including enhancing parenchymal lesions with or without mass effect, abnormal vessel wall enhancement, and leptomeningeal–perivascular enhancement [6, 7, 14, 17–19, 22, 26]. Central pontine T2/FLAIR hyperintensity is likely caused by microvascular occlusive changes in the pons, combined with venous congestion, as shown by a postmortem examination in a previous study [7, 20].…”

Persistent lesion hyperintensity on brain diffusion-weighted MRI is an early sign of intravascular lymphoma

“…Similar findings of persistent hyperintense lesions on DWI were previously reported in two patients with IVL. Oomura et al reported that an enlarging mass-like IVL lesion in the right basal ganglia showed restricted diffusion over 2 weeks 4. Sato et al also reported the growth of infarct-like lesions, which were hyperintense on DWI, over the course of 21 days in a patient with intravascular large B-cell lymphoma 5.…”