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Hemangioblastomas of the CNS

The hemangioblastoma (HB) is a highly vascular, benign and well-circumscribed, slowly growing solid or cystic neoplasm of the CNS and retina composed of stromal cells, endothelial cells, pericytes, and mast cells. The origin of the stromal cells, believed to be the true neoplastic cells of HB tissue, is still undefined. HB may cause polycytemia by secreting erythropoietin from stromal or mast cells. HB of the CNS is one of the manifestations of von Hippel-Lindau disease (VHL), but is regarded to be sporadic in approximately 80% of the cases, and then it is typically a single, cystic lesion of the cerebellum, brain stem or upper cervical medulla, presenting at the average age of somewhat over 40 years. Supratentorial HBs are rare. Microsurgery is still the treatment of choice aided, if necessary, by preoperative embolization to reduce vascularity. The intraoperative MRI scanner at is utilized in collaboration with the Surgical Planning Lab to provide detailed real-time imaging information for surgical guidance. It allows accurate localization and targeting as well as confirmation of the extent of resection of the tumors. Stereotactic radiotherapy may be considered in multiple small solid HBs in VHL. Internal organ cysts in the HB patients do not necessarily indicate VHL as they are common in other wise healthy subjects increasing in incidence with age

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Gamma knife radiosurgery for hemangioblastomas: clinical results and pathological findings

Wang EM, .Zhonghua Wai Ke Za Zhi. 2003 Jul;41(7):516-9.

Department of Neurosurgery, Huashan Hospital, Fudan University, Shanghai 200233, China.

OBJECTIVE: To retrospectively evaluate the effects of Gamma knife in the treatment of cerebral hemangioblastomas. METHODS: From 1993 to 1996, seventeen patients with 29 hemangioblastomas were treated with Gamma knife. The patients mean age was 35 years (range: 16 - 61 years). The mean tumor diameter was 16 mm (range: 6 - 55 mm). Thirteen patients had recurrent or residual hemangioblastomas. Four with primary hemangioblastomas were diagnosed using CT, MRI and DSA. The maximum dose to the tumors was 21.0 - 50.0 Gy, with mean dose of 33.7 Gy. The radiation dose to the periphery of tumors was 12.0 - 24.0 Gy, with mean dose of 17.6 Gy. RESULTS: All the patients had been followed up for 18 to 62 months, with mean 46 months. Five patients experienced clinical improvement and reduction in tumor volume, and 5 remained stable and tumor unchanged in volume during the follow-up period. Three patients died of tumor progression, surgery and cancer after treatment 18, 22, 25 months respectively. Four patients underwent surgery respectively at 3, 4, 29 and 48 months after gamma knife operation. The local control rate of the tumors at 1 year was 92%, 2 years 88%, 3 years 80% and 4 years 75%. Pathological findings in these patients showed varying degrees of small vessel thickening and occlusion together with degeneration, necrosis in the center of tumor and loss of tumor cells at periphery. CONCLUSIONS: Gamma knife is not adequately reliable for the control of hemangioblastoma cysts, it is an effective treatment of small or medium-size solid tumors, but long-term follow-up is needed. The recommended dose is 16 to 20 Gy.

Gamma knife radiosurgery in 11 hemangioblastomas.

Niemela M, .J Neurosurg. 1996 Oct;85(4):591-6.

Department of Neurosurgery, Helsinki University Hospital, Finland.

One suprasellar, one mesencephalic, and nine cerebellar hemangioblastomas were treated with the gamma knife in 10 patients (median age 48 years) in Stockholm between 1978 and 1993. Four patients had von Hippel-Lindau disease, a dominant inherited trait predisposing to multiple hemangioblastomas. Six hemangioblastomas were treated with radiotherapy at a median margin dose of 25 Gy (20-35 Gy) before 1990 and the next five with a median of 10 Gy (5-19 Gy). Computerized tomography or magnetic resonance images were available for 10 of the 11 hemangioblastomas at a median follow-up time of 26 months (4-68 months) after radiosurgery. The solid part of six hemangioblastomas shrank in a median of 30 months, whereas four hemangioblastomas were unchanged at a median of 14 months. Five hemangioblastomas had an adjoining cyst and three of these cysts had to be evacuated after radiosurgery. One solitary hemangioblastoma later developed a de novo cyst that also needed evacuation. One patient with two cerebellar hemangioblastomas (margin dose 25 Gy each) developed edema at 6 months and required a shunt and prolonged corticosteroid treatment. The combined follow-up data of the 23 hemangioblastomas in 15 patients from previous literature and the present series indicate that, first, a solitary small- or medium-sized hemangioblastoma usually shrinks or stops growing after radiosurgery. The recommended margin dose is 10 to 15 Gy. Second, the adjoining cyst often does not respond to radiosurgery but requires later, sometimes repeated evacuation.

Treatment of hemangioblastomas in von Hippel-Lindau disease with linear accelerator-based radiosurgery.

Chang SD, Neurosurgery. 1998 Jul;43(1):28-34
Department of Neurosurgery, Stanford University Medical Center, California 94305, USA.

OBJECTIVE: Stereotactic radiosurgery is increasingly being used to treat hemangioblastomas, particularly those that are in surgically inaccessible locations or that are multiple, as is common in von Hippel-Lindau disease. The purpose of this study was to retrospectively evaluate the effectiveness of radiosurgery in the treatment of hemangioblastomas. METHODS: From 1989 to 1996, 29 hemangioblastomas in 13 patients with von Hippel-Lindau disease were treated with linear accelerator-based radiosurgery. The mean patient age was 40 years (range, 31-57 yr). The radiation dose to the tumor periphery averaged 23.2 Gy (range, 18-40 Gy). The mean tumor volume was 1.6 cm3 (range, 0.07-65.4 cm3). Tumor response was evaluated in serial, contrast-enhanced, computed tomographic and magnetic resonance imaging scans. The mean follow-up period was 43 months (range, 11-84 mo). RESULTS: Only one (3%) of the treated hemangioblastomas progressed. Five tumors (17%) disappeared, 16 (55%) regressed, and 7 (24%) remained unchanged in size. Five of nine patients with symptoms referable to treated hemangioblastomas experienced symptomatic improvement. During the follow-up period, one patient died as a result of progression of untreated hemangioblastomas in the cervical spine. Three patients developed radiation necrosis, two of whom were symptomatic. CONCLUSION: Although follow-up monitoring is limited, stereotactic radiosurgery provides a high likelihood of local control of hemangioblastomas and is an attractive alternative to multiple surgical procedures for patients with von Hippel-Lindau disease.