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Intravascular (Angiocentric) Lymphoma
Karen L. Salzman, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Intravascular (angiocentric) lymphoma (IVL)
              • Synonyms

                • Intravascular large B-cell lymphoma (2016 WHO)
                • Intravascular malignant lymphomatosis
                • Angioendotheliotropic lymphoma
                • Malignant angioendotheliomatosis
                • Angiotropic large cell lymphoma
              • Definitions

                • Rare malignancy characterized by intravascular proliferation of lymphoid cells with predilection for CNS and skin
                • Form of non-Hodgkin lymphoma (NHL) characterized by intravascular growth

              IMAGING

              • General Features

                • CT Findings

                  • MR Findings

                    • Angiographic Findings

                      • Imaging Recommendations

                        • Nuclear Medicine Findings

                          DIFFERENTIAL DIAGNOSIS

                            PATHOLOGY

                            • General Features

                              • Gross Pathologic & Surgical Features

                                • Microscopic Features

                                  CLINICAL ISSUES

                                  • Presentation

                                    • Demographics

                                      • Natural History & Prognosis

                                        • Treatment

                                          DIAGNOSTIC CHECKLIST

                                          • Consider

                                            • Image Interpretation Pearls

                                              Selected References

                                              1. Chwalisz BK et al: Episodic visual distortions and stroke-like symptoms in a 56-year-old man with intravascular lymphoma. J Neuroophthalmol. 40(2):265-70, 2020
                                              2. Fujikura K et al: Transcriptome complexity in intravascular NK/T-cell lymphoma. J Clin Pathol. ePub, 2020
                                              3. Kimura M et al: Multiple cerebral hemorrhages with microbleeds in intravascular large B-cell lymphoma. J Stroke Cerebrovasc Dis. 29(6):104798, 2020
                                              4. Lin X et al: Atypical radiological findings of primary central nervous system lymphoma. Neuroradiology. 62(6): 765, 2020
                                              5. Shimada K et al: Rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone combined with high-dose methotrexate plus intrathecal chemotherapy for newly diagnosed intravascular large B-cell lymphoma (PRIMEUR-IVL): a multicentre, single-arm, phase 2 trial. Lancet Oncol. 21(4):593-602, 2020
                                              6. Suzuki Y et al: PD-L1 (SP142) expression in neoplastic cells predicts a poor prognosis for patients with intravascular large B-cell lymphoma treated with rituximab-based multi-agent chemotherapy. Cancer Med. 9(13):4768-76, 2020
                                              7. Yanagihara S et al: Usefulness of dermoscopy-aided skin biopsy for clinically recognizable intravascular large B-cell lymphoma: prediction of histopathological localization of occlusive vessels based on dermoscopic findings. Eur J Dermatol. ePub, 2020
                                              8. Matsue K et al: Diagnosis of intravascular large B-cell lymphoma: novel insights into clinicopathological features from 42 patients at a single institution over 20 years. Br J Haematol. 187(3):328-36, 2019
                                              9. Yamada E et al: Random skin biopsies before brain biopsy for intravascular large B-cell lymphoma. World Neurosurg. 121:e364-9, 2019
                                              10. Schaafsma JD et al: High-Resolution Vessel Wall MRI : Appearance of Intravascular Lymphoma Mimics Central Nervous System Vasculitis. Clin Neuroradiol. 27(1):105-8, 2017
                                              11. de Havenon A et al: Response to "High-Resolution Vessel Wall MRI: Appearance of Intravascular Lymphoma Mimics Central Nervous System Vasculitis". Clin Neuroradiol. 26(4):501, 2016
                                              12. Koeller KK et al: Extranodal lymphoma of the central nervous system and spine. Radiol Clin North Am. 54(4):649-71, 2016
                                              13. Louis DN et al: Lymphomas. In Louis DN et al: WHO Classification of Tumours of the Central Nervous System. IARC Press. 272-7, 2016
                                              14. Colavolpe C et al: FDG-PET/CT is a pivotal imaging modality to diagnose rare intravascular large B-cell lymphoma: case report and review of literature. Hematol Oncol. 33(2):99-109, 2015
                                              15. Nguyen GH et al: Unusual variants of intravascular malignant hematopoietic neoplasms: a report of 4 cases and review of the literature. Am J Dermatopathol. 37(5):360-7, 2015
                                              16. Wang L et al: Intravascular NK/T-cell lymphoma: a report of five cases with cutaneous manifestation from China. J Cutan Pathol. ePub, 2015
                                              17. Sekiguchi Y et al: Intravascular large B-cell lymphoma with pontine involvement successfully treated with R-CHOP therapy and intrathecal administration: a case report and review of literature. Int J Clin Exp Pathol. 7(6):3363-9, 2014
                                              18. Roohi F: Diagnosis of intravascular lymphoma. JAMA Neurol. 70(7):941, 2013
                                              19. Shimada K et al: Central nervous system involvement in intravascular large B-cell lymphoma: a retrospective analysis of 109 patients. Cancer Sci. 101(6):1480-6, 2010
                                              20. Liu H et al: Spinal cord infarct as the initial clinical presentation of intravascular malignant lymphomatosis. J Clin Neurosci. 16(4):570-3, 2009
                                              21. Pusch G et al: Intravascular lymphoma presenting with neurological signs but diagnosed by prostate biopsy: suspicion as a key to early diagnosis. Eur J Neurol. 16(3):e39-41, 2009
                                              22. Sumer M et al: Intravascular lymphoma masquerading as multiembolic stroke developing after coronary artery by-pass surgery. Neurologist. 15(2):98-101, 2009
                                              23. Yago K et al: Usefulness of FDG-PET/CT for the diagnosis of intravascular large B-cell lymphoma presenting with fever of unknown origin and renal dysfunction. Rinsho Ketsueki. 50(6):499-502, 2009
                                              24. Anda T et al: Ruptured distal middle cerebral artery aneurysm filled with tumor cells in a patient with intravascular large B-cell lymphoma. J Neurosurg. 109(3):492-6, 2008
                                              25. Balkema C et al: Usefulness of FDG-PET to diagnose intravascular lymphoma with encephalopathy and renal involvement. Acta Clin Belg. 63(3):185-9, 2008
                                              26. Cerroni L et al: Intravascular large T-cell or NK-cell lymphoma: a rare variant of intravascular large cell lymphoma with frequent cytotoxic phenotype and association with Epstein-Barr virus infection. Am J Surg Pathol. 32(6):891-8, 2008
                                              27. Grove CS et al: Intravascular lymphoma presenting as progressive paraparesis. J Clin Neurosci. 15(9):1056-8, 2008
                                              28. Im SH et al: Headache as the only presentation of intravascular lymphoma. Headache. 48(4):627-9, 2008
                                              29. Kinoshita M et al: Immunohistochemical analysis of adhesion molecules and matrix metalloproteinases in malignant CNS lymphomas: a study comparing primary CNS malignant and CNS intravascular lymphomas. Brain Tumor Pathol. 25(2):73-8, 2008
                                              30. Matsue K et al: A clinicopathological study of 13 cases of intravascular lymphoma: experience in a single institution over a 9-yr period. Eur J Haematol. 80(3):236-44, 2008
                                              31. Savard M et al: Intravascular lymphoma with conus medullaris syndrome followed by encephalopathy. Can J Neurol Sci. 35(3):366-71, 2008
                                              32. Srivatsa S et al: Intravascular lymphoma: an unusual diagnostic outcome of an incidentally detected adrenal mass. Endocr Pract. 14(7):884-8, 2008
                                              33. Szots M et al: Intravascular lymphomatosis of the nervous system. J Neurol. 255(10):1590-2, 2008
                                              34. Takahashi T et al: Successful treatment of intravascular large B-cell lymphoma diagnosed by bone marrow biopsy and FDG-PET scan. Intern Med. 47(10):975-9, 2008
                                              35. Ganguly S: Acute intracerebral hemorrhage in intravascular lymphoma: a serious infusion related adverse event of rituximab. Am J Clin Oncol. 30(2):211-2, 2007
                                              36. Hofman MS et al: Meningeal recurrence of intravascular large B-cell lymphoma: early diagnosis with PET-CT. Br J Haematol. 137(5):386, 2007
                                              37. Holmøy T et al: Intravascular large B-cell lymphoma presenting as cerebellar and cerebral infarction. Arch Neurol. 64(5):754-5, 2007
                                              38. Iijima M et al: Change of brain MRI findings in a patient with intravascular malignant lymphomatosis. Eur J Neurol. 14(5):e4-5, 2007
                                              39. Smirniotopoulos JG et al: Patterns of contrast enhancement in the brain and meninges. Radiographics. 27(2):525-51, 2007
                                              40. Song DE et al: Intravascular large cell lymphoma of the natural killer cell type. J Clin Oncol. 25(10):1279-82, 2007
                                              41. Gaul C et al: Intravascular lymphomatosis mimicking disseminated encephalomyelitis and encephalomyelopathy. Clin Neurol Neurosurg. 108(5):486-9, 2006
                                              42. Zuckerman D et al: Intravascular lymphoma: the oncologist's "great imitator". Oncologist. 11(5):496-502, 2006
                                              43. Kinoshita T et al: Intravascular malignant lymphomatosis: diffusion-weighted magnetic resonance imaging characteristics. Acta Radiol. 46(3):246-9, 2005
                                              44. Burger PC et al: The brain: tumors. In Burger PC et al: Surgical Pathology of the Nervous System and its Coverings. 4th ed. Philadelphia: Churchill Livingstone. 323-4, 2002
                                              45. Martin-Duverneuil N et al: Intravascular malignant lymphomatosis. Neuroradiology. 44(9):749-54, 2002
                                              46. Williams RL et al: Cerebral MR imaging in intravascular lymphomatosis. AJNR Am J Neuroradiol. 19(3):427-31, 1998
                                              Related Anatomy
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                                              Related Differential Diagnoses
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                                              References
                                              Tables

                                              Tables

                                              KEY FACTS

                                              • Terminology

                                                • Imaging

                                                  • Top Differential Diagnoses

                                                    • Pathology

                                                      • Clinical Issues

                                                        • Diagnostic Checklist

                                                          TERMINOLOGY

                                                          • Abbreviations

                                                            • Intravascular (angiocentric) lymphoma (IVL)
                                                          • Synonyms

                                                            • Intravascular large B-cell lymphoma (2016 WHO)
                                                            • Intravascular malignant lymphomatosis
                                                            • Angioendotheliotropic lymphoma
                                                            • Malignant angioendotheliomatosis
                                                            • Angiotropic large cell lymphoma
                                                          • Definitions

                                                            • Rare malignancy characterized by intravascular proliferation of lymphoid cells with predilection for CNS and skin
                                                            • Form of non-Hodgkin lymphoma (NHL) characterized by intravascular growth

                                                          IMAGING

                                                          • General Features

                                                            • CT Findings

                                                              • MR Findings

                                                                • Angiographic Findings

                                                                  • Imaging Recommendations

                                                                    • Nuclear Medicine Findings

                                                                      DIFFERENTIAL DIAGNOSIS

                                                                        PATHOLOGY

                                                                        • General Features

                                                                          • Gross Pathologic & Surgical Features

                                                                            • Microscopic Features

                                                                              CLINICAL ISSUES

                                                                              • Presentation

                                                                                • Demographics

                                                                                  • Natural History & Prognosis

                                                                                    • Treatment

                                                                                      DIAGNOSTIC CHECKLIST

                                                                                      • Consider

                                                                                        • Image Interpretation Pearls

                                                                                          Selected References

                                                                                          1. Chwalisz BK et al: Episodic visual distortions and stroke-like symptoms in a 56-year-old man with intravascular lymphoma. J Neuroophthalmol. 40(2):265-70, 2020
                                                                                          2. Fujikura K et al: Transcriptome complexity in intravascular NK/T-cell lymphoma. J Clin Pathol. ePub, 2020
                                                                                          3. Kimura M et al: Multiple cerebral hemorrhages with microbleeds in intravascular large B-cell lymphoma. J Stroke Cerebrovasc Dis. 29(6):104798, 2020
                                                                                          4. Lin X et al: Atypical radiological findings of primary central nervous system lymphoma. Neuroradiology. 62(6): 765, 2020
                                                                                          5. Shimada K et al: Rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone combined with high-dose methotrexate plus intrathecal chemotherapy for newly diagnosed intravascular large B-cell lymphoma (PRIMEUR-IVL): a multicentre, single-arm, phase 2 trial. Lancet Oncol. 21(4):593-602, 2020
                                                                                          6. Suzuki Y et al: PD-L1 (SP142) expression in neoplastic cells predicts a poor prognosis for patients with intravascular large B-cell lymphoma treated with rituximab-based multi-agent chemotherapy. Cancer Med. 9(13):4768-76, 2020
                                                                                          7. Yanagihara S et al: Usefulness of dermoscopy-aided skin biopsy for clinically recognizable intravascular large B-cell lymphoma: prediction of histopathological localization of occlusive vessels based on dermoscopic findings. Eur J Dermatol. ePub, 2020
                                                                                          8. Matsue K et al: Diagnosis of intravascular large B-cell lymphoma: novel insights into clinicopathological features from 42 patients at a single institution over 20 years. Br J Haematol. 187(3):328-36, 2019
                                                                                          9. Yamada E et al: Random skin biopsies before brain biopsy for intravascular large B-cell lymphoma. World Neurosurg. 121:e364-9, 2019
                                                                                          10. Schaafsma JD et al: High-Resolution Vessel Wall MRI : Appearance of Intravascular Lymphoma Mimics Central Nervous System Vasculitis. Clin Neuroradiol. 27(1):105-8, 2017
                                                                                          11. de Havenon A et al: Response to "High-Resolution Vessel Wall MRI: Appearance of Intravascular Lymphoma Mimics Central Nervous System Vasculitis". Clin Neuroradiol. 26(4):501, 2016
                                                                                          12. Koeller KK et al: Extranodal lymphoma of the central nervous system and spine. Radiol Clin North Am. 54(4):649-71, 2016
                                                                                          13. Louis DN et al: Lymphomas. In Louis DN et al: WHO Classification of Tumours of the Central Nervous System. IARC Press. 272-7, 2016
                                                                                          14. Colavolpe C et al: FDG-PET/CT is a pivotal imaging modality to diagnose rare intravascular large B-cell lymphoma: case report and review of literature. Hematol Oncol. 33(2):99-109, 2015
                                                                                          15. Nguyen GH et al: Unusual variants of intravascular malignant hematopoietic neoplasms: a report of 4 cases and review of the literature. Am J Dermatopathol. 37(5):360-7, 2015
                                                                                          16. Wang L et al: Intravascular NK/T-cell lymphoma: a report of five cases with cutaneous manifestation from China. J Cutan Pathol. ePub, 2015
                                                                                          17. Sekiguchi Y et al: Intravascular large B-cell lymphoma with pontine involvement successfully treated with R-CHOP therapy and intrathecal administration: a case report and review of literature. Int J Clin Exp Pathol. 7(6):3363-9, 2014
                                                                                          18. Roohi F: Diagnosis of intravascular lymphoma. JAMA Neurol. 70(7):941, 2013
                                                                                          19. Shimada K et al: Central nervous system involvement in intravascular large B-cell lymphoma: a retrospective analysis of 109 patients. Cancer Sci. 101(6):1480-6, 2010
                                                                                          20. Liu H et al: Spinal cord infarct as the initial clinical presentation of intravascular malignant lymphomatosis. J Clin Neurosci. 16(4):570-3, 2009
                                                                                          21. Pusch G et al: Intravascular lymphoma presenting with neurological signs but diagnosed by prostate biopsy: suspicion as a key to early diagnosis. Eur J Neurol. 16(3):e39-41, 2009
                                                                                          22. Sumer M et al: Intravascular lymphoma masquerading as multiembolic stroke developing after coronary artery by-pass surgery. Neurologist. 15(2):98-101, 2009
                                                                                          23. Yago K et al: Usefulness of FDG-PET/CT for the diagnosis of intravascular large B-cell lymphoma presenting with fever of unknown origin and renal dysfunction. Rinsho Ketsueki. 50(6):499-502, 2009
                                                                                          24. Anda T et al: Ruptured distal middle cerebral artery aneurysm filled with tumor cells in a patient with intravascular large B-cell lymphoma. J Neurosurg. 109(3):492-6, 2008
                                                                                          25. Balkema C et al: Usefulness of FDG-PET to diagnose intravascular lymphoma with encephalopathy and renal involvement. Acta Clin Belg. 63(3):185-9, 2008
                                                                                          26. Cerroni L et al: Intravascular large T-cell or NK-cell lymphoma: a rare variant of intravascular large cell lymphoma with frequent cytotoxic phenotype and association with Epstein-Barr virus infection. Am J Surg Pathol. 32(6):891-8, 2008
                                                                                          27. Grove CS et al: Intravascular lymphoma presenting as progressive paraparesis. J Clin Neurosci. 15(9):1056-8, 2008
                                                                                          28. Im SH et al: Headache as the only presentation of intravascular lymphoma. Headache. 48(4):627-9, 2008
                                                                                          29. Kinoshita M et al: Immunohistochemical analysis of adhesion molecules and matrix metalloproteinases in malignant CNS lymphomas: a study comparing primary CNS malignant and CNS intravascular lymphomas. Brain Tumor Pathol. 25(2):73-8, 2008
                                                                                          30. Matsue K et al: A clinicopathological study of 13 cases of intravascular lymphoma: experience in a single institution over a 9-yr period. Eur J Haematol. 80(3):236-44, 2008
                                                                                          31. Savard M et al: Intravascular lymphoma with conus medullaris syndrome followed by encephalopathy. Can J Neurol Sci. 35(3):366-71, 2008
                                                                                          32. Srivatsa S et al: Intravascular lymphoma: an unusual diagnostic outcome of an incidentally detected adrenal mass. Endocr Pract. 14(7):884-8, 2008
                                                                                          33. Szots M et al: Intravascular lymphomatosis of the nervous system. J Neurol. 255(10):1590-2, 2008
                                                                                          34. Takahashi T et al: Successful treatment of intravascular large B-cell lymphoma diagnosed by bone marrow biopsy and FDG-PET scan. Intern Med. 47(10):975-9, 2008
                                                                                          35. Ganguly S: Acute intracerebral hemorrhage in intravascular lymphoma: a serious infusion related adverse event of rituximab. Am J Clin Oncol. 30(2):211-2, 2007
                                                                                          36. Hofman MS et al: Meningeal recurrence of intravascular large B-cell lymphoma: early diagnosis with PET-CT. Br J Haematol. 137(5):386, 2007
                                                                                          37. Holmøy T et al: Intravascular large B-cell lymphoma presenting as cerebellar and cerebral infarction. Arch Neurol. 64(5):754-5, 2007
                                                                                          38. Iijima M et al: Change of brain MRI findings in a patient with intravascular malignant lymphomatosis. Eur J Neurol. 14(5):e4-5, 2007
                                                                                          39. Smirniotopoulos JG et al: Patterns of contrast enhancement in the brain and meninges. Radiographics. 27(2):525-51, 2007
                                                                                          40. Song DE et al: Intravascular large cell lymphoma of the natural killer cell type. J Clin Oncol. 25(10):1279-82, 2007
                                                                                          41. Gaul C et al: Intravascular lymphomatosis mimicking disseminated encephalomyelitis and encephalomyelopathy. Clin Neurol Neurosurg. 108(5):486-9, 2006
                                                                                          42. Zuckerman D et al: Intravascular lymphoma: the oncologist's "great imitator". Oncologist. 11(5):496-502, 2006
                                                                                          43. Kinoshita T et al: Intravascular malignant lymphomatosis: diffusion-weighted magnetic resonance imaging characteristics. Acta Radiol. 46(3):246-9, 2005
                                                                                          44. Burger PC et al: The brain: tumors. In Burger PC et al: Surgical Pathology of the Nervous System and its Coverings. 4th ed. Philadelphia: Churchill Livingstone. 323-4, 2002
                                                                                          45. Martin-Duverneuil N et al: Intravascular malignant lymphomatosis. Neuroradiology. 44(9):749-54, 2002
                                                                                          46. Williams RL et al: Cerebral MR imaging in intravascular lymphomatosis. AJNR Am J Neuroradiol. 19(3):427-31, 1998