Multiple Recurrences of Cerebellar Hemangioblastoma after 20 Years from Initial Total Removal of the Tumor

Department of Neurosurgery, Hiroshima General Hospital, Hiroshima, Japan.
No shinkei geka. Neurological surgery (Impact Factor: 0.13). 04/2004; 32(3):263-8.
Source: PubMed


Cerebellar hemangioblastoma develops alone or develops as part of von Hippel-Lindau disease. Moreover, multiple hemangioblastomas are found in 10-15%. It was reported that some hemangioblastomas recur with multiple recurrence in long time follow-up period. A 51 years old male was referred to our hospital because of headache and found a cerebellar tumor which was totally removed and diagnosed as hemangioblastoma pathologically. He presented no deficit after first surgery, but he did not come our hospital. In May 2000, he was referred to our hospital because of headache again and found five cerebellar tumors on MRI. Angiography showed markedly tumor stain. Retinal and spinal lesions were not seen. Two of five tumors was removed. The remaining three small tumors were received gamma knife surgery. In December 2000, because of cyst enlargement, one tumor was removed and the cyst was opened. In June 2001, he presented right hearing disturbance due to enlargement of cerebellar pontine angle tumor. The tumor was removed after tumor embolization, but hearing disturbance and slight dizziness remained. The remaining 5 mm tumor is followed.

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    • "Radiotherapy is recommended for unresectable, incompletely excised, or recurrent haemangioblastomas. It is also indicated for medically inoperable patients, multifocal disease and for discrete recurrences after surgery (Horrax & Vinas 1956; Patrice et al. 1996; Sumida et al. 2004). "
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    ABSTRACT: Haemangioblastomas are rare neoplasms of uncertain histogenesis. They represent 1.5-2.5% of intracranial tumours. While the cerebellum is by far the most frequent site, these lesions also tend to occur in the medulla and spinal cord. Most haemangioblastomas are sporadic but up to one quarter are associated with Von Hippel-Lindau disease (VHL). Although a fairly good number of haemangioblastomas were reported, a lack of side-by-side analysis of these reports has resulted in tentative conclusions that merely offer a first glimpse at their clinicopathologic diversity and histogenesis. To remedy this issue, this study presents a literature review concerning these lesions. Medline literature including both relevant monographs and clinicopathological case series. Haemangioblastomas occur either as a part of VHL disease (25-30%, inherited mutation of VHL gene on 3p25-26 chromosome) or as sporadic tumours (often with somatic mutation of VHL gene). They have diverse clinicopathologic presentations with cerebellar lesions having a better prognosis than their brainstem counterparts. Immunostaining is important for separation of haemangioblastomas from other tumours with similar histology. The rich vascularity of haemangioblastomas is due to overexpression of vascular endothelial growth factors. Moreover, 'stromal' cells represent the neoplastic cells of haemangioblastomas and are capable of forming blood islands with extramedullary haematopoiesis.
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    ABSTRACT: In this study the authors compare the clinical outcomes after particle embolization of hemangioblastomas in the cerebellum and spinal cord. They also review the literature of similar cases. Seven patients with hemangioblastomas in the spinal cord (four patients) and cerebellum (three patients) underwent preoperative embolization at the authors' center. Magnetic resonance imaging and selective angiography studies as well as histological diagnoses were available in all patients. Embosphere particles (trisacryl gelatin micro-spheres) were used in all cases. The smallest particle diameter ranged from 100 to 300 microm at the beginning of embolization in all patients. The outcome of embolization was favorable in patients with spinal cord hemangioblastomas, but it was unfavorable for those with cerebellar hemangioblastomas; acute tumor bleeding and death occurred in all of the latter cases. The outcomes following embolization are very different for these two locations possibly because of the different capillary sizes. The authors no longer use particle embolization to treat cerebellar hemangioblastomas.
    No preview · Article · Jul 2007 · Journal of Neurosurgery