CNS hemangioblastomatosis in a patient without von Hippel–Lindau disease

Franco, Arie; Pytel, Peter; Lukas, Rimas V.; Chennamaneni, Rupa; Collins, John M.

doi:10.2217/cns-2016-0027

Cited by 6 publications

(6 citation statements)

References 24 publications

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“…Another similar study conducted by Franco, Arie et al reports a case of a 74-year old male patient who has a resection of HB, 12 years prior [4]. The study suggests that the gold standard for the diagnostics of VHL can be through genetic testing.…”

Section: Literature Reviewmentioning

confidence: 78%

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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Section: Literature Reviewmentioning

confidence: 78%

Symptoms, Diagnosis and Treatment of Hemangioblastoma Dissemination

Halim

2019

OAJ Radiol.

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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%

“…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%

“…[ 8 ] In some studies, the patients were still alive at the time of publication, but with short follow-up periods or ongoing cases. [ 3 , 10 , 13 , 20 , 25 ] Some patients who were alive at the time of publication presented with poor neurological status, and some were bedridden. [ 1 , 15 , 23 ] The most common causes of death were infection and respiratory failure due to pontomedullary or cervical spinal cord compression.…”

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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“…Hemangioblastomatosis has been documented in patients with VHL disease following surgical removal of a cerebellar HB 9 11 13–16. It has also been reported in patients without VHL disease after surgical removal of a cerebellar HB 3 4 8 17–24. The literature contains only one case of hemangioblastomatosis occurring without surgical resection of the primary lesion in a patient without VHL disease 25.…”

Section: Introductionmentioning

confidence: 99%

Spinal leptomeningeal hemangioblastomatosis occurring without craniospinal surgery in von Hippel-Lindau disease

Jesús

Rosado-Philippi

2022

BMJ Case Rep

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Hemangioblastomatosis represents an unusual and malignant leptomeningeal dissemination of hemangioblastoma (HB). It has been reported in patients with sporadic HB or von Hippel-Lindau (VHL) disease. Hemangioblastomatosis had been reported following resection of a primary HB lesion in all cases except one patient with a sporadic HB. We present a patient with VHL with several HBs at the brainstem, cerebellum, pituitary stalk and retina who developed spinal hemangioblastomatosis without previous craniospinal surgery. A whole spine MRI showed the spinal dissemination from the primary lesions. The patient received craniospinal radiotherapy due to the extensive spinal leptomeningeal dissemination and multiple HBs. MRI performed 12 months after the radiotherapy showed stability of the lesions.

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CNS hemangioblastomatosis in a patient without von Hippel–Lindau disease

Cited by 6 publications

References 24 publications

Symptoms, Diagnosis and Treatment of Hemangioblastoma Dissemination

Symptoms, Diagnosis and Treatment of Hemangioblastoma Dissemination

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

Spinal leptomeningeal hemangioblastomatosis occurring without craniospinal surgery in von Hippel-Lindau disease

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