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Brain
Diagnosis
Pathology-Based Diagnoses
Neoplasms
Mesenchymal, Nonmeningolial Tumors Involving CNS
Vascular Tumors
Hemangioblastoma
Hemangioblastoma
Karen L. Salzman, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • Hemangioblastoma (HGBL)

              • Synonyms

                • Capillary hemangioma

              • Definitions

                • Benign, slow-growing, highly vascular tumor of adults most commonly found in cerebellum, brainstem, spinal cord

              IMAGING

              • General Features

                • CT Findings

                  • MR Findings

                    • Angiographic Findings

                      • Nuclear Medicine 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

                                                • Reporting Tips

                                                  Selected References

                                                  1. Gläsker S et al: Von Hippel-Lindau disease: current challenges and future prospects. Onco Targets Ther. 13:5669-90, 2020
                                                  2. Klingler JH et al: Hemangioblastoma and von Hippel-Lindau disease: genetic background, spectrum of disease, and neurosurgical treatment. Childs Nerv Syst. ePub, 2020
                                                  3. Qiu J et al: Stereotactic radiosurgery for central nervous system hemangioblastoma in von Hippel-Lindau disease: a systematic review and meta-analysis. Clin Neurol Neurosurg. 195:105912, 2020
                                                  4. Darbari S et al: Optic nerve hemangioblastoma: review. World Neurosurg. 128:211-5, 2019
                                                  5. Schattner A et al: Cerebellar hemangioblastoma. Am J Med. 131(1):e15-16, 2018
                                                  6. Bisceglia M et al: Extraneuraxial hemangioblastoma: clinicopathologic features and review of the literature. Adv Anat Pathol. 25(3):197-215, 2017
                                                  7. Goo HW et al: Medullary hemangioblastoma in a child with von Hippel-Lindau disease: vascular tumor perfusion depicted by arterial spin labeling and dynamic contrast-enhanced imaging. J Neurosurg Pediatr. 1-4, 2015
                                                  8. Kano H et al: Stereotactic radiosurgery for intracranial hemangioblastomas: a retrospective international outcome study. J Neurosurg. 1-10, 2015
                                                  9. Gorovoy IR et al: Retinal hemangioblastoma. JAMA Ophthalmol. 132(3):325, 2014
                                                  10. Liao CC et al: Clinical features and surgical outcomes of sporadic cerebellar hemangioblastomas. Clin Neurol Neurosurg. 125:160-5, 2014
                                                  11. Shin GW et al: Preoperative embolization of cerebellar hemangioblastoma with onyx: report of three cases. Neurointervention. 9(1):45-9, 2014
                                                  12. Courcoutsakis NA et al: Aggressive leptomeningeal hemangioblastomatosis of the central nervous system in a patient with von Hippel-Lindau disease. AJNR Am J Neuroradiol. 30(4):758-60, 2009
                                                  13. Parker F et al: Results of microsurgical treatment of medulla oblongata and spinal cord hemangioblastomas: a comparison of two distinct clinical patient groups. J Neurooncol. 93(1):133-7, 2009
                                                  14. Rachinger J et al: Solid haemangioblastomas of the CNS: a review of 17 consecutive cases. Neurosurg Rev. 32(1):37-47; discussion 47-8, 2009
                                                  15. Ros de San Pedro J et al: Massive hemorrhage in hemangioblastoma: literature review. Neurosurg Rev. 33(1):11-26, 2009
                                                  16. Shekhar H et al: Von Hippel-Lindau disease: delayed presentation as a cerebellar haemangioblastoma in an elderly patient. Br J Neurosurg. 23(1):97-8, 2009
                                                  17. Barrett R et al: Optic nerve hemangioblastoma. Ophthalmology. 115(11):2095, 2008
                                                  18. Jagannathan J et al: Surgical management of cerebellar hemangioblastomas in patients with von Hippel-Lindau disease. J Neurosurg. 108(2):210-22, 2008
                                                  19. Kano H et al: The role of stereotactic radiosurgery for intracranial hemangioblastomas. Neurosurgery. 63(3):443-50; discussion 450-1, 2008
                                                  20. Montano N et al: Embolization of hemangioblastomas. J Neurosurg. 108(5):1063-4; author reply 1064-5, 2008
                                                  21. Takeuchi H et al: A report of supratentorial leptomeningeal hemangioblastoma and a literature review. Neuropathology. 28(1):98-102, 2008
                                                  22. Innus C et al: Hemangioblastoma without von Hippel-Lindau disease. JAAPA. 20(11):28, 31-2, 2007
                                                  23. Hoang MP et al: Inhibin alpha distinguishes hemangioblastoma from clear cell renal cell carcinoma. Am J Surg Pathol. 27(8):1152-6, 2003
                                                  24. Reyns N et al: Leptomeningeal hemangioblastomatosis in a case of von Hippel-Lindau disease: case report. Neurosurgery. 52(5):1212-5; discussion 1215-6, 2003
                                                  25. Sarkar C et al: August 2002: 21-year-old male with cystic intracerebral tumor. Brain Pathol. 13(1):113-4, 117, 2003
                                                  26. 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
                                                  27. Weil RJ et al: Clinical and molecular analysis of disseminated hemangioblastomatosis of the central nervous system in patients without von Hippel-Lindau disease. Report of four cases. J Neurosurg. 96(4):775-87, 2002
                                                  28. 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
                                                  29. Kondo T et al: Diagnostic value of 201Tl-single-photon emission computerized tomography studies in cases of posterior fossa hemangioblastomas. J Neurosurg. 95(2):292-7, 2001
                                                  30. Wang C et al: Surgical management of medullary hemangioblastoma. Report of 47 cases. Surg Neurol. 56(4):218-26; discussion 226-7, 2001
                                                  31. Bohling T et al: von Hippel-Lindau disease and capillary hemangioblastoma. In Kleihues P et al: Tumours of the Nervous System. Lyon: IARC Press. 223-6, 2000
                                                  32. Miyagami M et al: Clinicopathological study of vascular endothelial growth factor (VEGF), p53, and proliferative potential in familial von Hippel-Lindau disease and sporadic hemangioblastomas. Brain Tumor Pathol. 17(3):111-20, 2000
                                                  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

                                                                • Hemangioblastoma (HGBL)

                                                              • Synonyms

                                                                • Capillary hemangioma

                                                              • Definitions

                                                                • Benign, slow-growing, highly vascular tumor of adults most commonly found in cerebellum, brainstem, spinal cord

                                                              IMAGING

                                                              • General Features

                                                                • CT Findings

                                                                  • MR Findings

                                                                    • Angiographic Findings

                                                                      • Nuclear Medicine 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

                                                                                                • Reporting Tips

                                                                                                  Selected References

                                                                                                  1. Gläsker S et al: Von Hippel-Lindau disease: current challenges and future prospects. Onco Targets Ther. 13:5669-90, 2020
                                                                                                  2. Klingler JH et al: Hemangioblastoma and von Hippel-Lindau disease: genetic background, spectrum of disease, and neurosurgical treatment. Childs Nerv Syst. ePub, 2020
                                                                                                  3. Qiu J et al: Stereotactic radiosurgery for central nervous system hemangioblastoma in von Hippel-Lindau disease: a systematic review and meta-analysis. Clin Neurol Neurosurg. 195:105912, 2020
                                                                                                  4. Darbari S et al: Optic nerve hemangioblastoma: review. World Neurosurg. 128:211-5, 2019
                                                                                                  5. Schattner A et al: Cerebellar hemangioblastoma. Am J Med. 131(1):e15-16, 2018
                                                                                                  6. Bisceglia M et al: Extraneuraxial hemangioblastoma: clinicopathologic features and review of the literature. Adv Anat Pathol. 25(3):197-215, 2017
                                                                                                  7. Goo HW et al: Medullary hemangioblastoma in a child with von Hippel-Lindau disease: vascular tumor perfusion depicted by arterial spin labeling and dynamic contrast-enhanced imaging. J Neurosurg Pediatr. 1-4, 2015
                                                                                                  8. Kano H et al: Stereotactic radiosurgery for intracranial hemangioblastomas: a retrospective international outcome study. J Neurosurg. 1-10, 2015
                                                                                                  9. Gorovoy IR et al: Retinal hemangioblastoma. JAMA Ophthalmol. 132(3):325, 2014
                                                                                                  10. Liao CC et al: Clinical features and surgical outcomes of sporadic cerebellar hemangioblastomas. Clin Neurol Neurosurg. 125:160-5, 2014
                                                                                                  11. Shin GW et al: Preoperative embolization of cerebellar hemangioblastoma with onyx: report of three cases. Neurointervention. 9(1):45-9, 2014
                                                                                                  12. Courcoutsakis NA et al: Aggressive leptomeningeal hemangioblastomatosis of the central nervous system in a patient with von Hippel-Lindau disease. AJNR Am J Neuroradiol. 30(4):758-60, 2009
                                                                                                  13. Parker F et al: Results of microsurgical treatment of medulla oblongata and spinal cord hemangioblastomas: a comparison of two distinct clinical patient groups. J Neurooncol. 93(1):133-7, 2009
                                                                                                  14. Rachinger J et al: Solid haemangioblastomas of the CNS: a review of 17 consecutive cases. Neurosurg Rev. 32(1):37-47; discussion 47-8, 2009
                                                                                                  15. Ros de San Pedro J et al: Massive hemorrhage in hemangioblastoma: literature review. Neurosurg Rev. 33(1):11-26, 2009
                                                                                                  16. Shekhar H et al: Von Hippel-Lindau disease: delayed presentation as a cerebellar haemangioblastoma in an elderly patient. Br J Neurosurg. 23(1):97-8, 2009
                                                                                                  17. Barrett R et al: Optic nerve hemangioblastoma. Ophthalmology. 115(11):2095, 2008
                                                                                                  18. Jagannathan J et al: Surgical management of cerebellar hemangioblastomas in patients with von Hippel-Lindau disease. J Neurosurg. 108(2):210-22, 2008
                                                                                                  19. Kano H et al: The role of stereotactic radiosurgery for intracranial hemangioblastomas. Neurosurgery. 63(3):443-50; discussion 450-1, 2008
                                                                                                  20. Montano N et al: Embolization of hemangioblastomas. J Neurosurg. 108(5):1063-4; author reply 1064-5, 2008
                                                                                                  21. Takeuchi H et al: A report of supratentorial leptomeningeal hemangioblastoma and a literature review. Neuropathology. 28(1):98-102, 2008
                                                                                                  22. Innus C et al: Hemangioblastoma without von Hippel-Lindau disease. JAAPA. 20(11):28, 31-2, 2007
                                                                                                  23. Hoang MP et al: Inhibin alpha distinguishes hemangioblastoma from clear cell renal cell carcinoma. Am J Surg Pathol. 27(8):1152-6, 2003
                                                                                                  24. Reyns N et al: Leptomeningeal hemangioblastomatosis in a case of von Hippel-Lindau disease: case report. Neurosurgery. 52(5):1212-5; discussion 1215-6, 2003
                                                                                                  25. Sarkar C et al: August 2002: 21-year-old male with cystic intracerebral tumor. Brain Pathol. 13(1):113-4, 117, 2003
                                                                                                  26. 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
                                                                                                  27. Weil RJ et al: Clinical and molecular analysis of disseminated hemangioblastomatosis of the central nervous system in patients without von Hippel-Lindau disease. Report of four cases. J Neurosurg. 96(4):775-87, 2002
                                                                                                  28. 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
                                                                                                  29. Kondo T et al: Diagnostic value of 201Tl-single-photon emission computerized tomography studies in cases of posterior fossa hemangioblastomas. J Neurosurg. 95(2):292-7, 2001
                                                                                                  30. Wang C et al: Surgical management of medullary hemangioblastoma. Report of 47 cases. Surg Neurol. 56(4):218-26; discussion 226-7, 2001
                                                                                                  31. Bohling T et al: von Hippel-Lindau disease and capillary hemangioblastoma. In Kleihues P et al: Tumours of the Nervous System. Lyon: IARC Press. 223-6, 2000
                                                                                                  32. Miyagami M et al: Clinicopathological study of vascular endothelial growth factor (VEGF), p53, and proliferative potential in familial von Hippel-Lindau disease and sporadic hemangioblastomas. Brain Tumor Pathol. 17(3):111-20, 2000