Disseminated haemangioblastoma without evidence of the von Hippel–Lindau syndrome or haemangioblastomatosis—A case report and clinico-pathological correlation

Lightfoot, Nicholas J; Lucas, P.; Finnis, N.D.M.

doi:10.1016/j.clineuro.2006.12.007

Cited by 7 publications

(7 citation statements)

References 32 publications

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“…Literature review revealed only 12 patients reported with cerebrospinal fluid dissemination of cerebellar hemangioblastoma. [ 1 2 3 4 5 6 11 14 15 ] With our report, the total number of patients described in literature would be 14. The demographic characteristics and various other factors been delineated in the Table 1 .…”

Section: Discussionmentioning

confidence: 85%

Disseminated cerebellar hemangioblastoma in two patients without von Hippel-Lindau disease

et al. 2014

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Background:Two patients who had received a total resection of cerebellar hemangioblastoma developed cerebrospinal fluid dissemination during a long-term follow-up period. We present this rare disease with discussion based on the literature.Case Description:The patients were two women aged 45 and 57 years. In the cerebellar hemisphere, one patient had cystic hemangioblastoma of mural nodule type and the other had solid type. Both the patients successfully underwent total resection by craniotomy. They presented no mutations in the von Hippel-Lindau disease (VHL) gene or lesions in the other organs. One patient developed local recurrence 38 months after the initial surgery, and received stereotactic radiosurgery. Three spinal cord tumors developed 91 months later, and the tumors were disseminated to the entire cerebrospinal cavity 107 months later. The other patient developed hydrocephalus 53 months after the initial surgery with tumor tissues disseminated in the intracranial subarachnoid space. The conditions of the two patients gradually aggravated despite treatment with ventriculo-peritoneal shunt and irradiation to the whole brain and whole spinal cord.Conclusion:Cerebrospinal fluid dissemination of cerebellar hemangioblastoma was found dominantly in non-VHL patients. The diagnosis was made 10 years after the initial surgery. Irradiation therapy was performed, but the patients died about 2 years after the diagnosis was given. Molecular targeted therapies including vascular proliferation suppression have been attempted lately, but no effective therapy has been established. Early diagnosis of dissemination as well as combination of aggressive excision and stereotactic radiosurgery are considered to be appropriate for current interventions.

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Section: Discussionmentioning

confidence: 85%

Disseminated cerebellar hemangioblastoma in two patients without von Hippel-Lindau disease

et al. 2014

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“…Fewer than 20 individual cases of disseminated disease are reported in the literature [4-6, [13][14][15][16][17][18][19][20][21]. The most typical scenario entails discovery of a relatively small number of tumor deposits along the brain stem and spinal cord, generally years after initial diagnosis and/or treatment of a cerebellar hemangioblastoma.…”

Section: Discussionmentioning

confidence: 99%

CNS hemangioblastomatosis in a patient without von Hippel–Lindau disease

et al. 2017

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We report on a case of disseminated CNS hemangioblastoma, also referred to as hemangioblastomatosis, involving the supratentorial compartment and the entire spine. The patient presented with new onset headache, gait difficulties and memory deficits many years following resection of a hemangioblastoma from the cerebellum. The patient's family history was negative for von Hippel-Lindau (VHL) disease, and his personal history was negative for any additional VHL-defining lesions. Imaging revealed extensive dural caking and nodularity both supratentorially and in the spine, along with scattered parenchymal tumors showing a more typical appearance for hemangioblastoma. Biopsy of the dural thickening revealed histologic features compatible with hemangioblastoma. Genetic testing for VHL was eventually completed, and no evidence of a germline VHL mutation was detected.

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“…After an extensive review of the literature in PubMed and Google Scholar using the terms “Hemangioblastoma,” “Hemangioblastomatosis,” “Leptomeningeal dissemination,” and “von Hippel–Lindau,” we found 30 similar cases reported in the past 46 years [ Table 1 ]. [ 1 - 4 , 6 , 8 , 10 , 13 , 15 , 17 , 19 , 20 , 22 - 27 , 30 , 32 , 33 ]…”

Section: Discussionmentioning

confidence: 99%

“…The mean age of patients at the first surgery was 44.8 years old (range, 4–75);[ 13 , 26 ] In some reports, it was difficult to precisely determine the age of the patients at the first surgery, mainly due to incomplete or unavailable medical records. [ 3 , 20 , 25 ]…”

Section: Discussionmentioning

confidence: 99%

Aggressive dissemination of central nervous system hemangioblastoma without association with von Hippel–Lindau disease: A case report and literature review

Dantas¹,

Raso²,

Braga

et al. 2022

Surgical Neurology International

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Background: Hemangioblastomas (HBs) typically present with benign behavior and are most commonly found in the posterior fossa. Multiple central nervous system (CNS) HBs are usually associated with von Hippel–Lindau disease, and leptomeningeal dissemination of sporadic HBs is extremely rare. A review of the literature identified 30 cases of leptomeningeal dissemination of sporadic HBs previously published in the literature. Case Description: We report the case of a patient who was diagnosed with multiple CNS HBs with aggressive progression 6 years after resection of a posterior fossa HB. He underwent multiple surgeries and died 4 years after the diagnosis of the first spinal dissemination. Conclusion: Dissemination of sporadic HBs is rare and aggressive disease evolution is usually observed. Further studies are necessary to determine the optimal therapeutic options.

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Disseminated haemangioblastoma without evidence of the von Hippel–Lindau syndrome or haemangioblastomatosis—A case report and clinico-pathological correlation

Cited by 7 publications

References 32 publications

Disseminated cerebellar hemangioblastoma in two patients without von Hippel-Lindau disease

Disseminated cerebellar hemangioblastoma in two patients without von Hippel-Lindau disease

CNS hemangioblastomatosis in a patient without von Hippel–Lindau disease

Aggressive dissemination of central nervous system hemangioblastoma without association with von Hippel–Lindau disease: A case report and literature review

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