eMedicine Specialties > Neurosurgery > Neoplasm

Hemangioblastoma: Follow-up

Author: Konstantin V Slavin, MD, Associate Professor, Department of Neurosurgery, University of Illinois at Chicago; Head, Section of Stereotactic and Functional Neurosurgery, University of Illinois at Chicago
Contributor Information and Disclosures

Updated: Mar 24, 2009

Outcome and Prognosis

Long-term results of hemangioblastoma management generally are favorable. Advancement of neuroimaging methods, improvements in microsurgical technique, and the addition of preoperative embolization have significantly lowered morbidity and mortality associated with hemangioblastoma surgery.

Subarachnoid dissemination of hemangioblastomas is extremely rare,34 and local recurrences after complete tumor resection seem to be more frequent in patients with von Hippel-Lindau (VHL) disease, in patients diagnosed at a young age, and in patients with multiple hemangioblastomas. The recurrence rate varies in different surgical series but generally remains less than 25%. Recently, histological subtype was found to correlate with a probability of hemangioblastoma recurrence, with a 25% recurrence rate in cellular subtype and an 8% recurrence rate in reticular subtype.24

Conclusion

Hemangioblastomas are benign tumors of uncertain origin that are located predominantly in the posterior cranial fossa and the spinal cord. Although most hemangioblastomas are sporadic, they are associated with autosomally dominant VHL disease in approximately 25% of cases. The tumors may be solid or cystic, and patients usually present with either focal neurological symptoms or increased intracranial pressure due to obstruction of CSF pathways. Most hemangioblastomas can be cured with surgical resection, and long-term recurrence rates seem to depend on the presence of VHL disease and multicentric lesions.

Future and Controversies

Future treatment of hemangioblastoma will greatly depend on gaining an understanding of its genetic background. Obviously, if identifying a genetic defect responsible for tumor formation and growth becomes possible, this defect could be reversed and tumor growth could be prevented. Also, finding specific genetic and molecular targets in hemangioblastomas may enable treatment using nonsurgical means, with higher success rates and lower risks of complications.

 


More on Hemangioblastoma

Overview: Hemangioblastoma
Workup: Hemangioblastoma
Treatment: Hemangioblastoma
Follow-up: Hemangioblastoma
Multimedia: Hemangioblastoma
References
[ CLOSE WINDOW ]

References

  1. Cushing H, Bailey P. Tumors Arising from Blood Vessels of the Brain: Angiomatous Malformations and Hemangioblastomas. Springfield, Ill: Charles C Thomas Publisher; 1928.

  2. Rubinstein LJ. Atlas of Tumor Pathology: Tumors of the Central Nervous System. Washington, DC: US Government Printing Office; 1972:235.

  3. Gonzales MF. Classification and pathogenesis of brain tumors. In: Kaye AH, Laws ER, eds. Brain Tumors. ed. New York, NY: Churchill Livingstone; 1995:31-45.

  4. Zulch KJ. Brain Tumors: Their Biology and Pathology. New York, NY: Springer-Verlag; 1986.

  5. Neumann HP, Eggert HR, Weigel K, Friedburg H, Wiestler OD, Schollmeyer P. Hemangioblastomas of the central nervous system. A 10-year study with special reference to von Hippel-Lindau syndrome. J Neurosurg. Jan 1989;70(1):24-30. [Medline].

  6. Constans JP, Meder F, Maiuri F, Donzelli R, Spaziante R, de Divitiis E. Posterior fossa hemangioblastomas. Surg Neurol. Mar 1986;25(3):269-75. [Medline].

  7. Lindau A. Studien uber Kleinhirncysten. Bau, Pathogenese und Beizeihunger zur Angiomatosis Retinae. Acta Pathol Microbiol Scand. 1926;1(Suppl):3-120.

  8. Yasargil MG, Antic J, Laciga R, de Preux J, Fideler RW, Boone SC. The microsurgical removal of intramedullary spinal hemangioblastomas. Report of twelve cases and a review of the literature. Surg Neurol. Sep 1976;141-8. [Medline].

  9. Murota T, Symon L. Surgical management of hemangioblastoma of the spinal cord: a report of 18 cases. Neurosurgery. Nov 1989;25(5):699-707; discussion 708. [Medline].

  10. Fischer G, Brotchi J. Intramedullary Spinal Cord Tumors. ed. Stuttgart, Germany: Thieme Medical Publishers; 1996:72-7.

  11. Roonprapunt C, Silvera VM, Setton A, Freed D, Epstein FJ, Jallo GI. Surgical management of isolated hemangioblastomas of the spinal cord. Neurosurgery. Aug 2001;49(2):321-7; discussion 327-8. [Medline].

  12. Adegbite AB, Rozdilsky B, Varughese G. Supratentorial capillary hemangioblastoma presenting with fatal spontaneous intracerebral hemorrhage. Neurosurgery. Mar 1983;12(3):327-30. [Medline].

  13. Goto T, Nishi T, Kunitoku N, et al. Suprasellar hemangioblastoma in a patient with von Hippel-Lindau disease confirmed by germline mutation study: case report and review of the literature. Surg Neurol. Jul 2001;56(1):22-6. [Medline].

  14. Peker S, Kurtkaya-Yapicier O, Sun I, Sav A, Pamir MN. Suprasellar haemangioblastoma. Report of two cases and review of the literature. J Clin Neurosci. Jan 2005;12(1):85-9. [Medline].

  15. Kerr DJ, Scheithauer BW, Miller GM, Ebersold MJ, McPhee TJ. Hemangioblastoma of the optic nerve: case report. Neurosurgery. Mar 1995;36(3):573-80; discussion 580-1. [Medline].

  16. Giannini C, Scheithauer BW, Hellbusch LC, et al. Peripheral nerve hemangioblastoma. Mod Pathol. Oct 1998;11(10):999-1004. [Medline].

  17. Patton KT, Satcher RL Jr, Laskin WB. Capillary hemangioblastoma of soft tissue: report of a case and review of the literature. Hum Pathol. Oct 2005;36(10):1135-9. [Medline].

  18. Bradley S, Dumas N, Ludman M, Wood L. Hereditary renal cell carcinoma associated with von Hippel-Lindau disease: a description of a Nova Scotia cohort. Can Urol Assoc J. Feb 2009;3(1):32-6. [Medline].

  19. Zagzag D, Krishnamachary B, Yee H, et al. Stromal cell-derived factor-1alpha and CXCR4 expression in hemangioblastoma and clear cell-renal cell carcinoma: von Hippel-Lindau loss-of-function induces expression of a ligand and its receptor. Cancer Res. Jul 15 2005;65(14):6178-88. [Medline].

  20. Agostinelli C, Roncaroli F, Galassi E, Bernardi B, Acciarri N, Tani G. Leptomeningeal hemangioblastoma. Case illustration. J Neurosurg. Aug 2004;101(1 Suppl):122. [Medline].

  21. Gläsker S, Van Velthoven V. Risk of hemorrhage in hemangioblastomas of the central nervous system. Neurosurgery. Jul 2005;57(1):71-6; discussion 71-6. [Medline].

  22. Lee SR, Sanches J, Mark AS, Dillon WP, Norman D, Newton TH. Posterior fossa hemangioblastomas: MR imaging. Radiology. May 1989;171(2):463-8. [Medline].

  23. Lach B, Gregor A, Rippstein P, Omulecka A. Angiogenic histogenesis of stromal cells in hemangioblastoma: ultrastructural and immunohistochemical study. Ultrastruct Pathol. Sep-Oct 1999;23(5):299-310. [Medline].

  24. Hasselblatt M, Jeibmann A, Gerss J, et al. Cellular and reticular variants of haemangioblastoma revisited: a clinicopathologic study of 88 cases. Neuropathol Appl Neurobiol. Dec 2005;31(6):618-22. [Medline].

  25. Symon L, Murota T, Pell M, Bordi L. Surgical management of haemangioblastoma of the posterior fossa. Acta Neurochir (Wien). 1993;120(3-4):103-10. [Medline].

  26. Zhou LF, Du G, Mao Y, Zhang R. Diagnosis and surgical treatment of brainstem hemangioblastomas. Surg Neurol. Apr 2005;63(4):307-15; discussion 315-6. [Medline].

  27. Eskridge JM, McAuliffe W, Harris B, Kim DK, Scott J, Winn HR. Preoperative endovascular embolization of craniospinal hemangioblastomas. AJNR Am J Neuroradiol. Mar 1996;17(3):525-31. [Medline].

  28. Takeuchi S, Tanaka R, Fujii Y, Abe H, Ito Y. Surgical treatment of hemangioblastomas with presurgical endovascular embolization. Neurol Med Chir (Tokyo). May 2001;41(5):246-51; discussion 251-2. [Medline].

  29. Chang SD, Meisel JA, Hancock SL, Martin DP, McManus M, Adler JR Jr. Treatment of hemangioblastomas in von Hippel-Lindau disease with linear accelerator-based radiosurgery. Neurosurgery. Jul 1998;43(1):28-34; discussion 34-5. [Medline].

  30. Niemela M, Lim YJ, Soderman M, Jaaskelainen J, Lindquist C. Gamma knife radiosurgery in 11 hemangioblastomas. J Neurosurg. Oct 1996;85(4):591-6. [Medline].

  31. Patrice SJ, Sneed PK, Flickinger JC, et al. Radiosurgery for hemangioblastoma: results of a multiinstitutional experience. Int J Radiat Oncol Biol Phys. Jun 1 1996;35(3):493-9. [Medline].

  32. Pan L, Wang EM, Wang BJ, et al. Gamma knife radiosurgery for hemangioblastomas. Stereotact Funct Neurosurg. Oct 1998;70 Suppl 1:179-86. [Medline].

  33. Schuch G, de Wit M, Holtje J, et al. Case 2. Hemangioblastomas: diagnosis of von Hippel-Lindau disease and antiangiogenic treatment with SU5416. J Clin Oncol. May 20 2005;23(15):3624-6. [Medline].

  34. Hande AM, Nagpal RD. Cerebellar haemangioblastoma with extensive dissemination. Br J Neurosurg. Oct 1996;10(5):507-11. [Medline].

  35. Akil H, Statham PF, Gotz M, Bramley P, Whittle IR. Adult cerebellar mutism and cognitive-affective syndrome caused by cystic hemangioblastoma. Acta Neurochir (Wien). May 2006;148(5):597-8. [Medline].

  36. Chung Y, Horoupian DS. Pathology of benign brain tumors. In: Morantz RA, Walsh JW, eds. Brain Tumors. New York, NY: Marcel Dekker; 1994:19-44.

  37. Conway JE, Chou D, Clatterbuck RE, Brem H, Long DM, Rigamonti D. Hemangioblastomas of the central nervous system in von Hippel-Lindau syndrome and sporadic disease. Neurosurgery. Jan 2001;48(1):55-62; discussion 62-3. [Medline].

  38. de la Monte SM, Horowitz SA. Hemangioblastomas: clinical and histopathological factors correlated with recurrence. Neurosurgery. Nov 1989;25(5):695-8. [Medline].

  39. Ercan M, Kahraman S, Basgül E, Aypar U. Anaesthetic management of a patient with von Hippel-Lindau disease: a combination of bilateral phaeochromocytoma and spinal cord haemangioblastoma. Eur J Anaesthesiol. Jan 1996;13(1):81-3. [Medline].

  40. Johnson J, Junewick J, Cottingham S. Phrenic nerve hemangioblastoma in a von Hippel-Lindau patient. AJR Am J Roentgenol. Mar 2005;184(3 Suppl):S10-1. [Medline].

  41. Miyagami M, Katayama Y. Long-term prognosis of hemangioblastomas of the central nervous system: clinical and immunohistochemical study in relation to recurrence. Brain Tumor Pathol. 2004;21(2):75-82. [Medline].

[ CLOSE WINDOW ]

Further Reading

[ CLOSE WINDOW ]

Keywords

hemangioblastoma, Lindau tumor, Lindau's tumor, capillary hemangioblastoma, vascular neoplasm, tumor, tumors, brain tumors, brain tumor, head tumors, neoplasm, meningeal tumor, intracranial neoplasm, intracranial tumor, vascular tumor, von Hippel-Lindau disease, VHL disease, Gamma Knife 

[ CLOSE WINDOW ]

Contributor Information and Disclosures

Author

Konstantin V Slavin, MD, Associate Professor, Department of Neurosurgery, University of Illinois at Chicago; Head, Section of Stereotactic and Functional Neurosurgery, University of Illinois at Chicago
Konstantin V Slavin, MD is a member of the following medical societies: American Association of Neurological Surgeons, American Medical Association, American Pain Society, American Society of Stereotactical and Functional Neurosurgery, and Congress of Neurological Surgeons
Disclosure: Nothing to disclose.

Medical Editor

Duc Hoang Duong, MD, Professor, Chief Physician, Departments of Neurological Surgery and Neuroscience, Epilepsy Center, Charles R Drew University
Duc Hoang Duong, MD is a member of the following medical societies: American Neurological Association, Congress of Neurological Surgeons, and North American Skull Base Society
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Ryszard M Pluta, MD, PhD, Associate Professor, Neurosurgical Department Medical Research Center, Polish Academy of Sciences at Warsaw, Poland; Senior Researcher, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, NIH
Ryszard M Pluta, MD, PhD is a member of the following medical societies: Congress of Neurological Surgeons and Polish Society of Neurosurgeons
Disclosure: Nothing to disclose.

CME Editor

Paolo Zamboni, MD, Professor of Surgery, Chief of Day Surgery Unit, Chair of Vascular Diseases Center, University of Ferrara, Italy
Paolo Zamboni, MD is a member of the following medical societies: American Venous Forum and New York Academy of Sciences
Disclosure: Nothing to disclose.

Chief Editor

Allen R Wyler, MD, Former Medical Director, Northstar Neuroscience, Inc
Allen R Wyler, MD is a member of the following medical societies: American Academy of Neurological and Orthopaedic Surgeons, American Association of Neurological Surgeons, and Society of Neurological Surgeons
Disclosure: Nothing to disclose.

RELATED MEDSCAPE ARTICLES
Articles
 
 
HONcode

We subscribe to the
HONcode principles of the
Health On the Net Foundation

All material on this website is protected by copyright, Copyright© 1994- by Medscape.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.