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Spine
Diagnosis
Neoplasms, Cysts, and Other Masses
Neoplasms
Intramedullary
Hemangioblastoma
Hemangioblastoma
Jeffrey S. Ross, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Synonyms

              • Capillary hemangioblastoma (HB)

            • Definitions

              • Low-grade, capillary-rich neoplasms of cerebellum and spinal cord (SC) that occur sporadically or in setting of von Hippel-Lindau syndrome (VHL)

            IMAGING

            • General Features

              • CT Findings

                • MR Findings

                  • Angiographic Findings

                    • Nonvascular Interventions

                      • Other Modality Findings

                        • Imaging Recommendations

                          DIFFERENTIAL DIAGNOSIS

                            PATHOLOGY

                            • General Features

                              • Staging, Grading, & Classification

                                • Gross Pathologic & Surgical Features

                                  • Microscopic Features

                                    CLINICAL ISSUES

                                    • Presentation

                                      • Demographics

                                        • Natural History & Prognosis

                                          • Treatment

                                            DIAGNOSTIC CHECKLIST

                                            • Consider

                                              • Image Interpretation Pearls

                                                Selected References

                                                1. Krüger MT et al: Minimally invasive resection of spinal hemangioblastoma: feasibility and clinical results in a series of 18 patients. J Neurosurg Spine. 1-10, 2019
                                                2. Yousef A et al: Sporadic and Von-Hippel Lindau disease-associated spinal hemangioblastomas: institutional experience on their similarities and differences. J Neurooncol. 143(3):547-52, 2019
                                                3. Dornbos D 3rd et al: Review of the neurological implications of von Hippel-Lindau disease. JAMA Neurol. 75(5):620-7, 2018
                                                4. Deng X et al: Intraspinal hemangioblastomas: analysis of 92 cases in a single institution. J Neurosurg Spine. 1-10, 2014
                                                5. Mechtler LL et al: Spinal cord tumors: new views and future directions. Neurol Clin. 31(1):241-68, 2013
                                                6. Rykken JB et al: Rim and flame signs: postgadolinium MRI findings specific for non-CNS intramedullary spinal cord metastases. AJNR Am J Neuroradiol. 34(4):908-15, 2013
                                                7. Mehta GU et al: Functional outcome after resection of spinal cord hemangioblastomas associated with von Hippel-Lindau disease. J Neurosurg Spine. 12(3):233-42, 2010
                                                8. Myong NH et al: Malignant glioma arising at the site of an excised cerebellar hemangioblastoma after irradiation in a von Hippel-Lindau disease patient. Yonsei Med J. 50(4):576-81, 2009
                                                9. Sharma GK et al: Spontaneous intramedullary hemorrhage of spinal hemangioblastoma: case report. Neurosurgery. 65(3):E627-8; discussion E628, 2009
                                                10. Leung RS et al: Imaging features of von Hippel-Lindau disease. Radiographics. 28(1):65-79; quiz 323, 2008
                                                11. Lee DK et al: Spinal cord hemangioblastoma: surgical strategy and clinical outcome. J Neurooncol. 61(1):27-34, 2003
                                                12. Pluta RM et al: Comparison of anterior and posterior surgical approaches in the treatment of ventral spinal hemangioblastomas in patients with von Hippel-Lindau disease. J Neurosurg. 98(1):117-24, 2003
                                                13. Wanebo JE et al: The natural history of hemangioblastomas of the central nervous system in patients with von Hippel-Lindau disease. J Neurosurg. 98(1):82-94, 2003
                                                14. Chu BC et al: MR findings in spinal hemangioblastoma: correlation with symptoms and with angiographic and surgical findings. AJNR Am J Neuroradiol. 22(1):206-17, 2001
                                                15. Conway JE et al: Hemangioblastomas of the central nervous system in von Hippel-Lindau syndrome and sporadic disease. Neurosurgery. 48(1):55-62; discussion 62-3, 2001
                                                16. Hamazaki S et al: Metastasis of renal cell carcinoma to central nervous system hemangioblastoma in two patients with von Hippel-Lindau disease. Pathol Int. 51(12):948-53, 2001
                                                17. Baker KB et al: MR imaging of spinal hemangioblastoma. AJR Am J Roentgenol. 174(2):377-82, 2000
                                                18. Couch V et al: von Hippel-Lindau disease. Mayo Clin Proc. 75(3):265-72, 2000
                                                19. Miller DJ et al: Hemangioblastomas and other uncommon intramedullary tumors. J Neurooncol. 47(3):253-70, 2000
                                                20. Friedrich CA: Von Hippel-Lindau syndrome. A pleomorphic condition. Cancer. 86(11 Suppl):2478-82, 1999
                                                21. Roessler K et al: Multiple spinal "miliary" hemangioblastomas in von Hippel-Lindau (vHL) disease without cerebellar involvement. A case report and review of the literature. Neurosurg Rev. 22(2-3):130-4, 1999
                                                22. Irie K et al: Spinal cord hemangioblastoma presenting with subarachnoid hemorrhage. Neurol Med Chir (Tokyo). 38(6):355-8, 1998
                                                23. Bakshi R et al: Spinal leptomeningeal hemangioblastomatosis in von Hippel-Lindau disease: magnetic resonance and pathological findings. J Neuroimaging. 7(4):242-4, 1997
                                                24. Eskridge JM et al: Preoperative endovascular embolization of craniospinal hemangioblastomas. AJNR Am J Neuroradiol. 17(3):525-31, 1996
                                                25. Richards FM et al: Expression of the von Hippel-Lindau disease tumour suppressor gene during human embryogenesis. Hum Mol Genet. 5(5):639-44, 1996
                                                26. Spetzger U et al: Hemangioblastomas of the spinal cord and the brainstem: diagnostic and therapeutic features. Neurosurg Rev. 19(3):147-51, 1996
                                                27. Wizigmann-Voos S et al: Pathology, genetics and cell biology of hemangioblastomas. Histol Histopathol. 11(4):1049-61, 1996
                                                28. Crossey PA et al: Identification of intragenic mutations in the von Hippel-Lindau disease tumour suppressor gene and correlation with disease phenotype. Hum Mol Genet. 3(8):1303-8, 1994
                                                29. Lunardi P et al: Isolated haemangioblastoma of spinal cord: report of 18 cases and a review of the literature. Acta Neurochir (Wien). 122(3-4):236-9, 1993
                                                30. Resche F et al: Haemangioblastoma, haemangioblastomatosis, and von Hippel-Lindau disease. Adv Tech Stand Neurosurg. 20:197-304, 1993
                                                31. Neumann HP et al: Central nervous system lesions in von Hippel-Lindau syndrome. J Neurol Neurosurg Psychiatry. 55(10):898-901, 1992
                                                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

                                                          TERMINOLOGY

                                                          • Synonyms

                                                            • Capillary hemangioblastoma (HB)

                                                          • Definitions

                                                            • Low-grade, capillary-rich neoplasms of cerebellum and spinal cord (SC) that occur sporadically or in setting of von Hippel-Lindau syndrome (VHL)

                                                          IMAGING

                                                          • General Features

                                                            • CT Findings

                                                              • MR Findings

                                                                • Angiographic Findings

                                                                  • Nonvascular Interventions

                                                                    • Other Modality Findings

                                                                      • Imaging Recommendations

                                                                        DIFFERENTIAL DIAGNOSIS

                                                                          PATHOLOGY

                                                                          • General Features

                                                                            • Staging, Grading, & Classification

                                                                              • Gross Pathologic & Surgical Features

                                                                                • Microscopic Features

                                                                                  CLINICAL ISSUES

                                                                                  • Presentation

                                                                                    • Demographics

                                                                                      • Natural History & Prognosis

                                                                                        • Treatment

                                                                                          DIAGNOSTIC CHECKLIST

                                                                                          • Consider

                                                                                            • Image Interpretation Pearls

                                                                                              Selected References

                                                                                              1. Krüger MT et al: Minimally invasive resection of spinal hemangioblastoma: feasibility and clinical results in a series of 18 patients. J Neurosurg Spine. 1-10, 2019
                                                                                              2. Yousef A et al: Sporadic and Von-Hippel Lindau disease-associated spinal hemangioblastomas: institutional experience on their similarities and differences. J Neurooncol. 143(3):547-52, 2019
                                                                                              3. Dornbos D 3rd et al: Review of the neurological implications of von Hippel-Lindau disease. JAMA Neurol. 75(5):620-7, 2018
                                                                                              4. Deng X et al: Intraspinal hemangioblastomas: analysis of 92 cases in a single institution. J Neurosurg Spine. 1-10, 2014
                                                                                              5. Mechtler LL et al: Spinal cord tumors: new views and future directions. Neurol Clin. 31(1):241-68, 2013
                                                                                              6. Rykken JB et al: Rim and flame signs: postgadolinium MRI findings specific for non-CNS intramedullary spinal cord metastases. AJNR Am J Neuroradiol. 34(4):908-15, 2013
                                                                                              7. Mehta GU et al: Functional outcome after resection of spinal cord hemangioblastomas associated with von Hippel-Lindau disease. J Neurosurg Spine. 12(3):233-42, 2010
                                                                                              8. Myong NH et al: Malignant glioma arising at the site of an excised cerebellar hemangioblastoma after irradiation in a von Hippel-Lindau disease patient. Yonsei Med J. 50(4):576-81, 2009
                                                                                              9. Sharma GK et al: Spontaneous intramedullary hemorrhage of spinal hemangioblastoma: case report. Neurosurgery. 65(3):E627-8; discussion E628, 2009
                                                                                              10. Leung RS et al: Imaging features of von Hippel-Lindau disease. Radiographics. 28(1):65-79; quiz 323, 2008
                                                                                              11. Lee DK et al: Spinal cord hemangioblastoma: surgical strategy and clinical outcome. J Neurooncol. 61(1):27-34, 2003
                                                                                              12. Pluta RM et al: Comparison of anterior and posterior surgical approaches in the treatment of ventral spinal hemangioblastomas in patients with von Hippel-Lindau disease. J Neurosurg. 98(1):117-24, 2003
                                                                                              13. Wanebo JE et al: The natural history of hemangioblastomas of the central nervous system in patients with von Hippel-Lindau disease. J Neurosurg. 98(1):82-94, 2003
                                                                                              14. Chu BC et al: MR findings in spinal hemangioblastoma: correlation with symptoms and with angiographic and surgical findings. AJNR Am J Neuroradiol. 22(1):206-17, 2001
                                                                                              15. Conway JE et al: Hemangioblastomas of the central nervous system in von Hippel-Lindau syndrome and sporadic disease. Neurosurgery. 48(1):55-62; discussion 62-3, 2001
                                                                                              16. Hamazaki S et al: Metastasis of renal cell carcinoma to central nervous system hemangioblastoma in two patients with von Hippel-Lindau disease. Pathol Int. 51(12):948-53, 2001
                                                                                              17. Baker KB et al: MR imaging of spinal hemangioblastoma. AJR Am J Roentgenol. 174(2):377-82, 2000
                                                                                              18. Couch V et al: von Hippel-Lindau disease. Mayo Clin Proc. 75(3):265-72, 2000
                                                                                              19. Miller DJ et al: Hemangioblastomas and other uncommon intramedullary tumors. J Neurooncol. 47(3):253-70, 2000
                                                                                              20. Friedrich CA: Von Hippel-Lindau syndrome. A pleomorphic condition. Cancer. 86(11 Suppl):2478-82, 1999
                                                                                              21. Roessler K et al: Multiple spinal "miliary" hemangioblastomas in von Hippel-Lindau (vHL) disease without cerebellar involvement. A case report and review of the literature. Neurosurg Rev. 22(2-3):130-4, 1999
                                                                                              22. Irie K et al: Spinal cord hemangioblastoma presenting with subarachnoid hemorrhage. Neurol Med Chir (Tokyo). 38(6):355-8, 1998
                                                                                              23. Bakshi R et al: Spinal leptomeningeal hemangioblastomatosis in von Hippel-Lindau disease: magnetic resonance and pathological findings. J Neuroimaging. 7(4):242-4, 1997
                                                                                              24. Eskridge JM et al: Preoperative endovascular embolization of craniospinal hemangioblastomas. AJNR Am J Neuroradiol. 17(3):525-31, 1996
                                                                                              25. Richards FM et al: Expression of the von Hippel-Lindau disease tumour suppressor gene during human embryogenesis. Hum Mol Genet. 5(5):639-44, 1996
                                                                                              26. Spetzger U et al: Hemangioblastomas of the spinal cord and the brainstem: diagnostic and therapeutic features. Neurosurg Rev. 19(3):147-51, 1996
                                                                                              27. Wizigmann-Voos S et al: Pathology, genetics and cell biology of hemangioblastomas. Histol Histopathol. 11(4):1049-61, 1996
                                                                                              28. Crossey PA et al: Identification of intragenic mutations in the von Hippel-Lindau disease tumour suppressor gene and correlation with disease phenotype. Hum Mol Genet. 3(8):1303-8, 1994
                                                                                              29. Lunardi P et al: Isolated haemangioblastoma of spinal cord: report of 18 cases and a review of the literature. Acta Neurochir (Wien). 122(3-4):236-9, 1993
                                                                                              30. Resche F et al: Haemangioblastoma, haemangioblastomatosis, and von Hippel-Lindau disease. Adv Tech Stand Neurosurg. 20:197-304, 1993
                                                                                              31. Neumann HP et al: Central nervous system lesions in von Hippel-Lindau syndrome. J Neurol Neurosurg Psychiatry. 55(10):898-901, 1992