Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

Hemangioblastoma Workup

  • Author: Konstantin V Slavin, MD; Chief Editor: Brian H Kopell, MD  more...
 
Updated: Oct 28, 2015
 

Laboratory Studies

Perform blood tests to help reveal associated lesions that may be a part of the VHL disease complex. Unfortunately, finding polycythemia does not help in diagnosing the tumor.

Next

Imaging Studies

The diagnostic workup of suspected hemangioblastomas must include, in addition to history, physical, and thorough neurological examination, complete neural axis imaging and abdominal CT scan or ultrasound. The goal of these additional tests is to reveal associated lesions that may be a part of VHL disease complex.

Radiographically, hemangioblastomas are best diagnosed with MRI.[24] MRI of hemangioblastomas usually shows an enhancing mass clearly delineated from the surrounding brain or spinal cord tissue. The tumor tissue may be hypointense or isointense on precontrast T1-weighted images and hyperintense on T2-weighted images.[25]

Plain radiographs usually do not aid in diagnosis. Myelography and cisternography, which were considered the tests of choice in the past, now are almost never used in the diagnostic workup of hemangioblastomas.

Plain computed tomography (CT) scan may reveal hypodensity of the tumoral cyst and associated hydrocephalus. CT scans with intravenous contrast show uniform enhancement of the tumor nodule that, in association with the adjacent cyst, may be extremely characteristic of posterior fossa hemangioblastomas.

Cerebral and spinal angiography reveals a highly vascular tumor blush, and this diagnostic modality may be extremely useful for assessing the vascular supply to the tumor. This information may help the surgeon during tumor resection.

In patients with hemangioblastomas, complete neural axis imaging usually is recommended in order to rule out multiple lesions, especially in those cases in which VHL syndrome is either diagnosed or clinically suspected.

Previous
Next

Other Tests

Perform a detailed ophthalmologic evaluation to help reveal associated lesions that may be a part of the VHL disease complex.

Previous
Next

Histologic Findings

Histologically, hemangioblastomas are vascular neoplasms. In addition to relatively normal-appearing endothelial cells that line capillary spaces, hemangioblastomas have 2 distinct cellular components that may occur in the same tumor in different proportions. The first type is small, perivascular, endothelial cells that have dark compact nuclei and sparse cytoplasm. Cells of the second type contain multiple vacuoles and granular eosinophilic cytoplasm rich in lipids. These stromal cells may show some nuclear pleomorphism, but mitotic figures rarely are seen. The exact histogenetic origin of stromal cells is unknown, but the latest studies indicate that they may represent a heterogeneous population of abnormally differentiating mesenchymal cells of angiogenic lineage, with some morphological features of endothelium, pericytes, and smooth-muscle cells.[26]

Two histological subtypes (cellular and reticular) have been described in primary hemangioblastomas of the central nervous system and have been found to correlate with the probability of tumor recurrence.[27] The reticular subtype is more commonly encountered; the cellular subtype is associated with higher probability of recurrence.

No histologic grading system exists for hemangioblastomas.

Previous
 
 
Contributor Information and Disclosures
Author

Konstantin V Slavin, MD Head, Section of Stereotactic and Functional Neurosurgery, Professor, Department of Neurosurgery, University of Illinois at Chicago College of Medicine

Konstantin V Slavin, MD is a member of the following medical societies: American Association of Neurological Surgeons, Congress of Neurological Surgeons, American Society for Stereotactic and Functional Neurosurgery, American Medical Association, American Pain Society

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Ryszard M Pluta, MD, PhD Associate Professor, Neurosurgical Department Medical Research Center, Polish Academy of Sciences, Poland; Clinical Staff Scientist, Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health (NIH); Fishbein Fellow, JAMA

Ryszard M Pluta, MD, PhD is a member of the following medical societies: Polish Society of Neurosurgeons, Congress of Neurological Surgeons

Disclosure: Nothing to disclose.

Chief Editor

Brian H Kopell, MD Associate Professor, Department of Neurosurgery, Icahn School of Medicine at Mount Sinai

Brian H Kopell, MD is a member of the following medical societies: Alpha Omega Alpha, American Association of Neurological Surgeons, International Parkinson and Movement Disorder Society, Congress of Neurological Surgeons, American Society for Stereotactic and Functional Neurosurgery, North American Neuromodulation Society

Disclosure: Received consulting fee from Medtronic for consulting; Received consulting fee from St Jude Neuromodulation for consulting; Received consulting fee from MRI Interventions for consulting.

Additional Contributors

Duc Hoang Duong, MD Professor, Chief Physician, Departments of Neurological Surgery and Neuroscience, Epilepsy Center, Charles Drew University of Medicine and Science

Duc Hoang Duong, MD is a member of the following medical societies: American Neurological Association, Congress of Neurological Surgeons, North American Skull Base Society

Disclosure: Nothing to disclose.

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. Kaye AH, Laws ER, eds. Brain Tumors. 1st 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. 1989 Jan. 70(1):24-30. [Medline].

  6. Constans JP, Meder F, Maiuri F, Donzelli R, Spaziante R, de Divitiis E. Posterior fossa hemangioblastomas. Surg Neurol. 1986 Mar. 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. 1976 Sep. 141-8. [Medline].

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

  10. Fischer G, Brotchi J. Intramedullary Spinal Cord Tumors. 1st 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. 2001 Aug. 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. 1983 Mar. 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. 2001 Jul. 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. 2005 Jan. 12(1):85-9. [Medline].

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

  16. Giannini C, Scheithauer BW, Hellbusch LC, et al. Peripheral nerve hemangioblastoma. Mod Pathol. 1998 Oct. 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. 2005 Oct. 36(10):1135-9. [Medline].

  18. Kassardjian CD, Macdonald RL, Munoz DG. Hemangioblastomas in the elderly: epidemiology and clinical characteristics. J Clin Neurosci. 2014 Jul. 21 (7):1205-8. [Medline].

  19. 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. 2009 Feb. 3(1):32-6. [Medline].

  20. 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. 2005 Jul 15. 65(14):6178-88. [Medline].

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

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

  23. Doyle LA, Fletcher CD. Peripheral hemangioblastoma: clinicopathologic characterization in a series of 22 cases. Am J Surg Pathol. 2014 Jan. 38 (1):119-27. [Medline].

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

  25. Arima H, Hasegawa T, Togawa D, Yamato Y, Kobayashi S, Yasuda T, et al. Feasibility of a novel diagnostic chart of intramedullary spinal cord tumors in magnetic resonance imaging. Spinal Cord. 2014 Oct. 52 (10):769-73. [Medline].

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

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

  28. 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].

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

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

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

  32. 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. 1998 Jul. 43(1):28-34; discussion 34-5. [Medline].

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

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

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

  36. 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. 2005 May 20. 23(15):3624-6. [Medline].

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

  38. Wind JJ, Bakhtian KD, Sweet JA, Mehta GU, Thawani JP, Asthagiri AR, et al. Long-term outcome after resection of brainstem hemangioblastomas in von Hippel-Lindau disease. J Neurosurg. 2011 May. 114(5):1312-8. [Medline].

  39. Fukuda M, Takao T, Hiraishi T, Yoshimura J, Yajima N, Saito A, et al. Clinical factors predicting outcomes after surgical resection for sporadic cerebellar hemangioblastomas. World Neurosurg. 2014 Nov. 82 (5):815-21. [Medline].

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

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

  42. 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. 2001 Jan. 48(1):55-62; discussion 62-3. [Medline].

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

  44. 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. 1996 Jan. 13(1):81-3. [Medline].

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

  46. 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].

 
Previous
Next
 
Photomicrograph shows the classic microscopic appearance of a cerebellar hemangioblastoma with numerous capillaries and polygonal stroma cells shows vacuoles of cytoplasm and hyperchromatic nucleus (hematoxylin-eosin stain, high-power magnification). Courtesy of Dr Paul Fiedler.><
Supratentorial hemangioblastoma proved by histologic analysis. Carotid arteriogram demonstrates a vascular, dense, tumor filled from the anterior cerebral vessels and not involving the sagittal sinus.
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.