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Hemangioblastoma Treatment & Management

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

Medical Therapy

Because hemangioblastomas are benign tumors and generally are not invasive in nature, they may be cured by surgical excision. Therefore, surgical resection is considered a standard of treatment and should be offered to the patient unless the risk of operation outweighs its potential benefits.[8, 6, 9, 28, 10, 11, 29]

Other therapeutic modalities include endovascular embolization of the solid component of the tumor,[30, 31] which may decrease the vascularity of the tumor and lower blood loss during its resection, and stereotactic radiosurgery of the tumor using either a linear accelerator[32] or a Gamma Knife.[33, 34, 35] Antiangiogenic treatment of hemangioblastoma has also been recently described.[36]

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Surgical Therapy

Surgical treatment of hemangioblastomas is total resection, with the main goal being the preservation of surrounding neural tissue.

The tumors usually are well demarcated from the surrounding brain or spinal cord, but this border of separation does not contain any particular membrane or capsule.

The surgical approach must be wide enough to avoid compression of the healthy tissues during retraction. Thorough evaluation of preoperative imaging studies is the key to the safest possible exposure of the tumor. In addition to MRI and CT scans, review the angiography findings to identify the principal blood supply to the tumor mass.

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Preoperative Details

Prior to surgery, patients should undergo adequate medical evaluation and complete neural axis imaging. Patients and their families must be informed about the risks and possible complications of surgery, particularly the potential for neurological deterioration.

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Intraoperative Details

The tumor is usually easy to visualize because of its reddish-colored solid component and the yellow fluid inside the cyst.

If the cyst is present, it may be emptied by cutting the covering pial membrane or by aspirating the cystic contents using a syringe with a short small-caliber needle. Decompression of the cyst allows for improved delineation of the interface between the tumor and the brain or spinal cord.

The surface of the tumor may be coagulated with wide bipolar forceps; however, avoid penetration of the tumor itself because of its extreme vascularity and difficulties with hemostasis. Try to dissect the tumor circumferentially by careful coagulation and cutting the small feeding vessels and adhesions between the tumor and the surrounding brain or spinal cord and by putting cottonoid strips into the developing plane to avoid direct pressure on the brain or spinal cord tissue.

Once the feeding vessels are identified, they are coagulated and cut. Try to coagulate the arterial feeders prior to the draining veins, but this is not as crucial as it is in arteriovenous malformations.

After the tumor is totally removed, the raw surface of the brain or spinal cord remains relatively bloodless, and the oozing blood stops after a few minutes of gently packing the resection cavity with wet cotton balls, avoiding the need for additional coagulation.

If an associated hydrocephalus exists, it must be addressed separately, usually by means of external ventricular drainage (EVD) prior to tumor resection. After the tumor is removed, the need for permanent shunt placement may be determined by the patient's response to EVD clamping. In most cases, an intramedullary syrinx does not require a separate drainage procedure because it usually resolves after tumor removal.

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Postoperative Details

In regards to general surgical management, having blood products available for transfusion is very important because the vascular character of hemangioblastomas may result in serious intraoperative blood loss. Additionally, anesthesia for patients with VHL disease may be quite challenging due to the presence of associated renal and endocrine dysfunction.

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Follow-up

Follow-up care for patients with hemangioblastomas should include regular neurological and imaging checks to confirm the absence of tumor recurrence and/or development of distant lesions.

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Complications

With an adequate preoperative workup, most complications of surgery for hemangioblastoma may be avoided. Meticulous maintenance of hemostasis, attention to minor details, and great respect for neural and vascular elements may significantly decrease the risk of postoperative complications. The main emphasis, as usual, should be placed on preventing complications rather than on treatment.

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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,[37] 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 results of one study found that resection of brainstem hemangioblastomas is generally a safe and effective treatment for patients with VHL disease. However, due to VHL disease–associated progression, long-term decline in functional status may occur.[38] 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.[27]

At final follow-up examination of patients who underwent resection of sporadic hemangioblastoma in the cerebellum, patients with solid tumors more frequently showed poor outcomes than patients with cystic tumors. According to the study authors, the solid configuration observed on preoperative images of sporadic cerebellar hemangioblastomas is one of the most important clinical factors related to both immediate and long-term outcomes after surgery.[39]

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.

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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.

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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.

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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.
 
 
 
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