Hemangioblastoma Treatment & Management

Updated: Sep 26, 2017
  • Author: Konstantin V Slavin, MD; Chief Editor: Brian H Kopell, MD  more...
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Treatment

Approach Considerations

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. [16, 18, 19, 20, 21, 40, 41, 42]

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.

Other therapeutic modalities include endovascular embolization of the solid component of the tumor, [43, 44] 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 [45] or a gamma knife. [46, 47, 48, 5] After studying 186 patients from 6 centers in the North American Gamma Knife Consortium, Kano et al concluded that stereotactic radiosurgery provided control of growing tumors seen on serial imaging studies in 79-92% of cases. [6] . Antiangiogenic treatment of hemangioblastoma has also been described. [49]

Regarding 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 because of associated renal and endocrine dysfunction.

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.

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

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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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Outcome and Prognosis

Long-term results of hemangioblastoma management are generally 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, [50] 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. [51] The recurrence rates vary in different surgical series but generally remain less than 25%. Histologic subtype has been 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. [39]

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. [52]

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