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Hemangioblastoma, Craniovertebral Junction
Lubdha M. Shah, 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 neoplasm of cerebellum and spinal cord (SC) that occurs sporadically or in setting of von Hippel-Lindau (VHL) syndrome

            IMAGING

            • General Features

              • CT Findings

                • MR Findings

                  • Angiographic Findings

                    • Nonvascular Interventions

                      • Other Modality Findings

                        • Imaging Recommendations

                          • Positron Emission Tomography

                            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. Kanno H et al: Clinical features of patients bearing central nervous system hemangioblastoma in von Hippel-Lindau disease. Acta Neurochir (Wien). 155(1):1-7, 2013
                                                  2. Rykken JB et al: Rim and Flame Signs: Postgadolinium MRI Findings Specific for Non-CNS Intramedullary Spinal Cord Metastases. AJNR Am J Neuroradiol. Epub ahead of print, 2012
                                                  3. Tomura N et al: PET Findings of Intramedullary Tumors of the Spinal Cord Using [18F] FDG and [11C] Methionine. AJNR Am J Neuroradiol. Epub ahead of print, 2012
                                                  4. 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
                                                  5. 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
                                                  6. Sharma GK et al: Spontaneous intramedullary hemorrhage of spinal hemangioblastoma: case report. Neurosurgery. 65(3):E627-8; discussion E628, 2009
                                                  7. Leung RS et al: Imaging features of von Hippel-Lindau disease. Radiographics. 28(1):65-79; quiz 323, 2008
                                                  8. Lee DK et al: Spinal cord hemangioblastoma: surgical strategy and clinical outcome. J Neurooncol. 61(1):27-34, 2003
                                                  9. 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
                                                  10. 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
                                                  11. 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
                                                  12. 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
                                                  13. 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
                                                  14. Baker KB et al: MR imaging of spinal hemangioblastoma. AJR Am J Roentgenol. 174(2):377-82, 2000
                                                  15. Couch V et al: von Hippel-Lindau disease. Mayo Clin Proc. 75(3):265-72, 2000
                                                  16. Miller DJ et al: Hemangioblastomas and other uncommon intramedullary tumors. J Neurooncol. 47(3):253-70, 2000
                                                  17. Friedrich CA: Von Hippel-Lindau syndrome. A pleomorphic condition. Cancer. 86(11 Suppl):2478-82, 1999
                                                  18. 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
                                                  19. Irie K et al: Spinal cord hemangioblastoma presenting with subarachnoid hemorrhage. Neurol Med Chir (Tokyo). 38(6):355-8, 1998
                                                  20. Bakshi R et al: Spinal leptomeningeal hemangioblastomatosis in von Hippel-Lindau disease: magnetic resonance and pathological findings. J Neuroimaging. 7(4):242-4, 1997
                                                  21. Eskridge JM et al: Preoperative endovascular embolization of craniospinal hemangioblastomas. AJNR Am J Neuroradiol. 17(3):525-31, 1996
                                                  22. 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
                                                  23. Spetzger U et al: Hemangioblastomas of the spinal cord and the brainstem: diagnostic and therapeutic features. Neurosurg Rev. 19(3):147-51, 1996
                                                  24. Wizigmann-Voos S et al: Pathology, genetics and cell biology of hemangioblastomas. Histol Histopathol. 11(4):1049-61, 1996
                                                  25. 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
                                                  26. 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
                                                  27. Resche F et al: Haemangioblastoma, haemangioblastomatosis, and von Hippel-Lindau disease. Adv Tech Stand Neurosurg. 20:197-304, 1993
                                                  28. Neumann HP et al: Central nervous system lesions in von Hippel-Lindau syndrome. J Neurol Neurosurg Psychiatry. 55(10):898-901, 1992
                                                  29. Omar AI: Bevacizumab for the treatment of surgically unresectable cervical cord hemangioblastoma: a case report. J Med Case Rep. 2012 Aug 10;6(1):238. doi: 10. PubMed Central PMCID: PMC3471004, 1186
                                                  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 neoplasm of cerebellum and spinal cord (SC) that occurs sporadically or in setting of von Hippel-Lindau (VHL) syndrome

                                                            IMAGING

                                                            • General Features

                                                              • CT Findings

                                                                • MR Findings

                                                                  • Angiographic Findings

                                                                    • Nonvascular Interventions

                                                                      • Other Modality Findings

                                                                        • Imaging Recommendations

                                                                          • Positron Emission Tomography

                                                                            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. Kanno H et al: Clinical features of patients bearing central nervous system hemangioblastoma in von Hippel-Lindau disease. Acta Neurochir (Wien). 155(1):1-7, 2013
                                                                                                  2. Rykken JB et al: Rim and Flame Signs: Postgadolinium MRI Findings Specific for Non-CNS Intramedullary Spinal Cord Metastases. AJNR Am J Neuroradiol. Epub ahead of print, 2012
                                                                                                  3. Tomura N et al: PET Findings of Intramedullary Tumors of the Spinal Cord Using [18F] FDG and [11C] Methionine. AJNR Am J Neuroradiol. Epub ahead of print, 2012
                                                                                                  4. 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
                                                                                                  5. 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
                                                                                                  6. Sharma GK et al: Spontaneous intramedullary hemorrhage of spinal hemangioblastoma: case report. Neurosurgery. 65(3):E627-8; discussion E628, 2009
                                                                                                  7. Leung RS et al: Imaging features of von Hippel-Lindau disease. Radiographics. 28(1):65-79; quiz 323, 2008
                                                                                                  8. Lee DK et al: Spinal cord hemangioblastoma: surgical strategy and clinical outcome. J Neurooncol. 61(1):27-34, 2003
                                                                                                  9. 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
                                                                                                  10. 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
                                                                                                  11. 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
                                                                                                  12. 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
                                                                                                  13. 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
                                                                                                  14. Baker KB et al: MR imaging of spinal hemangioblastoma. AJR Am J Roentgenol. 174(2):377-82, 2000
                                                                                                  15. Couch V et al: von Hippel-Lindau disease. Mayo Clin Proc. 75(3):265-72, 2000
                                                                                                  16. Miller DJ et al: Hemangioblastomas and other uncommon intramedullary tumors. J Neurooncol. 47(3):253-70, 2000
                                                                                                  17. Friedrich CA: Von Hippel-Lindau syndrome. A pleomorphic condition. Cancer. 86(11 Suppl):2478-82, 1999
                                                                                                  18. 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
                                                                                                  19. Irie K et al: Spinal cord hemangioblastoma presenting with subarachnoid hemorrhage. Neurol Med Chir (Tokyo). 38(6):355-8, 1998
                                                                                                  20. Bakshi R et al: Spinal leptomeningeal hemangioblastomatosis in von Hippel-Lindau disease: magnetic resonance and pathological findings. J Neuroimaging. 7(4):242-4, 1997
                                                                                                  21. Eskridge JM et al: Preoperative endovascular embolization of craniospinal hemangioblastomas. AJNR Am J Neuroradiol. 17(3):525-31, 1996
                                                                                                  22. 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
                                                                                                  23. Spetzger U et al: Hemangioblastomas of the spinal cord and the brainstem: diagnostic and therapeutic features. Neurosurg Rev. 19(3):147-51, 1996
                                                                                                  24. Wizigmann-Voos S et al: Pathology, genetics and cell biology of hemangioblastomas. Histol Histopathol. 11(4):1049-61, 1996
                                                                                                  25. 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
                                                                                                  26. 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
                                                                                                  27. Resche F et al: Haemangioblastoma, haemangioblastomatosis, and von Hippel-Lindau disease. Adv Tech Stand Neurosurg. 20:197-304, 1993
                                                                                                  28. Neumann HP et al: Central nervous system lesions in von Hippel-Lindau syndrome. J Neurol Neurosurg Psychiatry. 55(10):898-901, 1992
                                                                                                  29. Omar AI: Bevacizumab for the treatment of surgically unresectable cervical cord hemangioblastoma: a case report. J Med Case Rep. 2012 Aug 10;6(1):238. doi: 10. PubMed Central PMCID: PMC3471004, 1186