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von Hippel-Lindau Syndrome
Anne G. Osborn, MD, FACR; Gilbert Vézina, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            • Diagnostic Checklist

              TERMINOLOGY

              • Abbreviations

                • von Hippel-Lindau (VHL) syndrome
              • Definitions

                • Autosomal dominant familial syndrome with hemangioblastomas (HGBLs), clear cell renal carcinoma, cystadenomas, pheochromocytomas
                  • Affects 6 different organ systems, including eye, ear, and central nervous system (CNS)
                  • Involved tissues often have multiple lesions
                  • Lesions → benign cysts, vascular tumors, carcinomas

              IMAGING

              • General Features

                • CT Findings

                  • MR Findings

                    • Angiographic 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. Bausch B et al: Characterization of endolymphatic sac tumors and von Hippel-Lindau disease in the international ELST registry. Head Neck. ePub, 2015
                                              2. Binderup ML et al: Risk of new tumors in von Hippel-Lindau patients depends on age and genotype. Genet Med. ePub, 2015
                                              3. Gossage L et al: VHL, the story of a tumour suppressor gene. Nat Rev Cancer. 15(1):55-64, 2015
                                              4. Kano H et al: Stereotactic radiosurgery for intracranial hemangioblastomas: a retrospective international outcome study. J Neurosurg. 1-10, 2015
                                              5. Hanakita S et al: The long-term outcomes of radiosurgery for intracranial hemangioblastomas. Neuro Oncol. 16(3):429-33, 2014
                                              6. Bamps S et al: What the neurosurgeon should know about hemangioblastoma, both sporadic and in Von Hippel-Lindau disease: A literature review. Surg Neurol Int. 4:145, 2013
                                              7. Kim H et al: Meningeal supratentorial hemangioblastoma in a patient with von hippel-lindau disease mimicking angioblastic menigioma. J Korean Neurosurg Soc. 54(5):415-9, 2013
                                              8. Lonser RR et al: Pituitary stalk hemangioblastomas in von Hippel-Lindau disease. J Neurosurg. 110(2):350-3, 2009
                                              9. Jagannathan J et al: Surgical management of cerebellar hemangioblastomas in patients with von Hippel-Lindau disease. J Neurosurg. 108(2):210-22, 2008
                                              10. Ammerman JM et al: Long-term natural history of hemangioblastomas in patients with von Hippel-Lindau disease: implications for treatment. J Neurosurg. 105(2):248-55, 2006
                                              11. Fisher C et al: Central nervous system hemangioblastoma and von Hippel-Lindau syndrome: a familial presentation. Clin Pediatr (Phila). 45(5):456-62, 2006
                                              12. 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
                                              13. Weil RJ et al: Surgical management of brainstem hemangioblastomas in patients with von Hippel-Lindau disease. J Neurosurg. 98(1):95-105, 2003
                                              14. 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
                                              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

                                                            • von Hippel-Lindau (VHL) syndrome
                                                          • Definitions

                                                            • Autosomal dominant familial syndrome with hemangioblastomas (HGBLs), clear cell renal carcinoma, cystadenomas, pheochromocytomas
                                                              • Affects 6 different organ systems, including eye, ear, and central nervous system (CNS)
                                                              • Involved tissues often have multiple lesions
                                                              • Lesions → benign cysts, vascular tumors, carcinomas

                                                          IMAGING

                                                          • General Features

                                                            • CT Findings

                                                              • MR Findings

                                                                • Angiographic 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. Bausch B et al: Characterization of endolymphatic sac tumors and von Hippel-Lindau disease in the international ELST registry. Head Neck. ePub, 2015
                                                                                          2. Binderup ML et al: Risk of new tumors in von Hippel-Lindau patients depends on age and genotype. Genet Med. ePub, 2015
                                                                                          3. Gossage L et al: VHL, the story of a tumour suppressor gene. Nat Rev Cancer. 15(1):55-64, 2015
                                                                                          4. Kano H et al: Stereotactic radiosurgery for intracranial hemangioblastomas: a retrospective international outcome study. J Neurosurg. 1-10, 2015
                                                                                          5. Hanakita S et al: The long-term outcomes of radiosurgery for intracranial hemangioblastomas. Neuro Oncol. 16(3):429-33, 2014
                                                                                          6. Bamps S et al: What the neurosurgeon should know about hemangioblastoma, both sporadic and in Von Hippel-Lindau disease: A literature review. Surg Neurol Int. 4:145, 2013
                                                                                          7. Kim H et al: Meningeal supratentorial hemangioblastoma in a patient with von hippel-lindau disease mimicking angioblastic menigioma. J Korean Neurosurg Soc. 54(5):415-9, 2013
                                                                                          8. Lonser RR et al: Pituitary stalk hemangioblastomas in von Hippel-Lindau disease. J Neurosurg. 110(2):350-3, 2009
                                                                                          9. Jagannathan J et al: Surgical management of cerebellar hemangioblastomas in patients with von Hippel-Lindau disease. J Neurosurg. 108(2):210-22, 2008
                                                                                          10. Ammerman JM et al: Long-term natural history of hemangioblastomas in patients with von Hippel-Lindau disease: implications for treatment. J Neurosurg. 105(2):248-55, 2006
                                                                                          11. Fisher C et al: Central nervous system hemangioblastoma and von Hippel-Lindau syndrome: a familial presentation. Clin Pediatr (Phila). 45(5):456-62, 2006
                                                                                          12. 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
                                                                                          13. Weil RJ et al: Surgical management of brainstem hemangioblastomas in patients with von Hippel-Lindau disease. J Neurosurg. 98(1):95-105, 2003
                                                                                          14. 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