cerebellar_hemangioblastoma_associated_with_von_hippel-lindau_disease

Cerebellar hemangioblastoma associated with Von Hippel-Lindau disease

The prevalence of cerebellar hemangioblastoma associated with Von Hippel-Lindau disease: 44–72%

The mean age of diagnosis inVHL patients with cerebellar hemangioblastomas are at least 10 years younger than sporadic cerebellar hemangioblastomas

Cysts are commonly associated with cerebellar, brainstem and spinal cord hemangioblastomas

Cysts grow at a faster rate than the HGBs, symptoms related to mass effect are frequently secondary to the cysts

Cerebellar HGBs were located in the superficial, posterior and superior half of the cerebellar hemispheres 1).

93% of the cerebellar HGBs were located in the cerebellar hemispheres and 7% in the vermis

The HGBs are also more frequently found in the superficial posterior half of the brainstem and the spinal cord

The HGBs have multiple sequential growth and quiescent phases


Symptoms and signs caused by cerebellar hemangioblastomas in von Hippel-Lindau disease are associated with edema and peritumoral cyst formation/propagation and are treated safely and effectively with resection. Cerebrospinal fluid diversion is rarely necessary after complete tumor removal in patients with preoperative hydrocephalus. Cerebellar hemangioblastomas are preferentially distributed in the posterior half of the cerebellum, as they are in the brainstem and spinal cord. Tumor recurrence is avoided by meticulous extracapsular resection 2).

see also Cerebellar hemangioblastoma treatment.

As multiple surgeries involve an increased risk of access-related morbidity, a minimally invasive approach can be favorable for specific types of tumors in the posterior fossa.

Krüger et al. presented the case of a minimally invasive removal of a superficial cerebellar hemangioblastoma with tumor-associated cyst and indicate the potential benefits and limitations of this technique 3).

Solitary hemangioblastomas are for the most part considered benign, curable by total resection, except in those cases associated with Von Hippel-Lindau disease.

A 37-year-old male patient who was diagnosed vHLD with multiple spinal and one infratentorial hemangioblastomas and holocord syrinx formation of the whole spinal cord. Combined microsurgical approaches to two spinal lesions and the cerebellar lesion followed by external beam radiotherapy of the posterior fossa and the whole spinal axis stabilized tumor growth of the asymptomatic lesions, while no recurrent tumors were detected at the site of surgery. A clinical deterioration connected to early postoperative deficits stabilized to a moderate gait ataxia. The follow-up after radiotherapy covered 60 months. A combination of microsurgery and radiosurgery for the surgically not amenable lesions is an adequate treatment regimen to stabilize tumor growth and clinical symptoms of multifocal spinal hemangioblastomas in vHLD, though the therapy should be limited to symptomatic or growing lesions 4).


Krüger et al. presented the case of a minimally invasive removal of a superficial cerebellar hemangioblastoma with tumor-associated cyst and indicate the potential benefits and limitations of this technique 5).


1) , 2)
Jagannathan J, Lonser RR, Smith R, DeVroom HL, Oldfield EH. Surgical management of cerebellar hemangioblastomas in patients with von Hippel-Lindau disease. J Neurosurg. 2008 Feb;108(2):210-22. doi: 10.3171/JNS/2008/108/2/0210. PubMed PMID: 18240914.
3) , 5)
Krüger MT, Klingler JH. Resection of a cerebellar hemangioblastoma via a minimally invasive tubular approach. J Clin Neurosci. 2019 May;63:240-243. doi: 10.1016/j.jocn.2019.01.045. Epub 2019 Feb 4. PubMed PMID: 30732985.
4)
Knoop N, Seidel C, Frydrychowicz C, Meixensberger J. Combined Microsurgery and Radiotherapy for Multiple Spinal Cord Hemangioblastomas with Holocord Syrinx in von Hippel-Lindau Disease: A Case Report. J Neurol Surg Rep. 2019 Oct;80(4):e46-e50. doi: 10.1055/s-0039-3401808. Epub 2019 Dec 31. PubMed PMID: 31908906; PubMed Central PMCID: PMC6938460.
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