A Very Aggressive Hemangioblastomatosis Without VHL Gene Mutation

Park, Jeong Goo; Song, Sang Woo; Koh, Young-Cho

doi:10.21129/nerve.2015.1.1.44

Cited by 2 publications

(5 citation statements)

References 9 publications

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“…Hemangioblastomatosis has been reported in several studies. [ 3 , 10 , 15 , 17 , 19 , 24 , 26 , 27 , 33 ] The mechanisms by which HBs disseminate in the CNS are still unclear; some authors believe that it may be caused by the spread of tumor cells through the cerebrospinal fluid space after a lesion resection. [ 19 ]…”

Section: Discussionmentioning

confidence: 99%

“…There was also great variety among the studies regarding the interval to the diagnosis of CNS dissemination. The interval ranged from 1 month[ 24 ] to 32 years. [ 2 ] Our patient presented with spinal dissemination 6 years after the first surgery.…”

Section: Discussionmentioning

confidence: 99%

“…One patient was treated with Interferon-2 α and minocycline,[ 33 ] sunitinib was used in one patient,[ 27 ] and three patients were treated with bevacizumab. [ 8 , 24 , 30 ]…”

Section: Discussionmentioning

confidence: 99%

“…[ 30 ] In three studies, patients with disseminated sporadic HBs were treated with bevacizumab; two patients had sustained stable disease for 12 months before evolving with progression of the lesions,[ 8 , 30 ] and one patient evolved with immediate neurological improvement, but long-term follow-up was not established. [ 24 ]…”

Section: Discussionmentioning

confidence: 99%

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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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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

“…One patient was treated with Interferon-2 α and minocycline,[ 33 ] sunitinib was used in one patient,[ 27 ] and three patients were treated with bevacizumab. [ 8 , 24 , 30 ]…”

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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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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show abstract

“…Aggressive Hemangioblastoma without VHL is also discussed in a report in which a 55-year-old woman w h o w a s d i a g n o s e d w i t h s p o r a d i c Hemangioblastoma but the VHL gene test was proved negative [21]. However, the woman had temporal mass was thought-out as meningioma.…”

Section: Literature Reviewmentioning

confidence: 99%

Symptoms, Diagnosis and Treatment of Hemangioblastoma Dissemination

Halim

2019

OAJ Radiol.

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Haemangioblastomas present in nervous system are noticed to occur in correspondence with Hippel-Lindau diseases (VHL). With uncertain origins, Haemangioblastomas are tumours that does not poses any serious threats to an individual’s health but may become risky in various cases. There are various symptoms of this disease with easily approachable treatment methods. Some of the methods of treatments are mentioned in this report, namely; radiotherapy, Everolimus as systemic therapy, numerous types of chemotherapy suitable for this disease, resection surgery, microsurgical surgery, Bevacizumab treatment, and craniospinal radiation. With the evaluation and examination of the HB (Haemangioblastomas) tumour it is considered that the occurrence of this specific tumour is still unidentified. In some cases it is considered that individuals having the hereditary disease of Hippel-Lindau (VHL) are affected with the existence of Haemangioblastomas. According to the researchers, HB tumour has the capability of occurring at any age, with differing symptoms. The symptoms usually appear or disappear according to their nature depending on the size and location of the Tumour. Referring 25 different researches and case studies, the researchers are unaware of the real reason of its occurrence, however the symptoms and location of this tumour are similar in most of the cases. Most of the common locations are known to be cerebellum and spinal cord. Within these researches, other case studies are involved that are referring the similar scenarios. A number of treatments are provided according to the nature and condition of each patient as conditions differ with reference to the nature of patients, as with reference to the case studies; most common patients residing are between the age of 40’s-70’s. Keywords: Hemangioblastoma, Dissemination, VHL, Symptoms, Diagnosis, Treatments.

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A Very Aggressive Hemangioblastomatosis Without VHL Gene Mutation

Cited by 2 publications

References 9 publications

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

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

Symptoms, Diagnosis and Treatment of Hemangioblastoma Dissemination

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