Artikel
Influence of Von Hippel-Lindau syndrome and the tumour morphology on central nervous system haemangioblastoma
Einfluss des Von-Hippel-Lindau-Syndroms und der Tumor-Morphologie auf Hämangioblastome des Zentralnervensystems
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Autoren
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Adrian Liebert
- Paracelsus Medical University, Department of Neurosurgery, Nürnberg, Deutschland
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Ghassan Kerry
- Paracelsus Medical University, Department of Neurosurgery, Nürnberg, Deutschland
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Hans-Herbert Steiner
- Paracelsus Medical University, Department of Neurosurgery, Nürnberg, Deutschland
| Veröffentlicht: | 4. Juni 2021 |
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Gliederung
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Objective: This study examines the influence of Von Hippel-Lindau syndrome (VHL) and the tumour morphology on central nervous system (CNS) Haemangioblastoma (HB).
Methods: We retrospectively analysed patients who underwent surgery for at least one CNS HB in our clinic from January 2008 to December 2019. Data collection included the presence of VHL, age, gender, morphology (solid/cystic), location, complications, and outcome at discharge (Glasgow outcome scale). We compared solid with cystic HBs as well as sporadic HBs with HBs associated with VHL. Statistical analysis was performed using Fisher’s exact test (significance level two-sided p-value ≤ .05).
Results: From 2008 to 2019, 29 patients underwent surgery for 30 HBs. Four patients were positive for VHL. The resected HB appeared cystic in 13 patients and predominantly solid in 15 patients. A cystic and a solid HB were resected during one surgery in one case. The gender distribution was 1:1 in the VHL group, but 2,5:1 male predominance in the sporadic group. Mean age in the sporadic solid group (M=62,36 y; SD=17,8) was higher than in the sporadic cystic group (M= 44,36 y; SD=12.9). VHL-patients were younger (M= 41,5 y; SD= 11,9) than patients with sporadic HBs (M=54,44 y; SD=18). Four patients presented with multiple HBs, all of them were positive for VHL. The cerebellum was the most common location (67%), especially in the cystic group (79%) and less common in the solid group (56%). Eight patients presented with a recurrent HB in the study period; 4 of them were positive for VHL. The complication rate was 40% (n6) in the solid group and 15% (n2) in the cystic one (p = .22). However, only one complication was severe – a severe postoperative bleeding. The good recovery outcome was 69 % (n9) in the cystic group and 53% (n8) in the solid one (p = .46). One VHL-patient had a postoperative complication (p = 1) and 2 VHL-patients had good recovery (p = 1).
Conclusion: This study demonstrates the impact of VHL on HBs in terms of earlier onset of the disease, multiple occurrences of HBs and the recurrence rate. Sporadic solid HBs present at a higher age compared to cystic HBs. Cystic HBs are located mainly in the cerebellum, whereas solid HBs show a higher distribution in CNS locations. Neither the tumour morphology nor the presence of VHL seem to affect the short-term outcome and the complication rate. A limitation of this study is the small number of cases due to the low prevalence of the disease.
