TU Ming,HU Shaobo,ZHENG Weiming. Long-term follow-up of cerebellar hemangioblastomas in von Hippel-Lindau disease[J]. JOURNAL OF WEZHOU MEDICAL UNIVERSITY, 2018, 48(5): 350-354.
Abstract：Objective: To observe the long-term outcome of patients with VHL associated cerebellar hemangioblastomas after tumors resection, compared with patients with sporadic cerebellar hemangioblastomas.Methods: Clinical characteristics and long-term outcomes of VHL related cerebellar gioblastomas (n=31) patients were compared with patients with sporadic cerebellar gioblastomas (n=31), matched for tumor size. Sequencing of VHL gene of patients with VHL disease was conducted to determine the gene mutations. Results: Patients with VHL disease were younger than sporadic patients (P<0.001). The median size of tumors showed no difference in two groups. Median postoperative follow-up was 99 (69, 137) months in VHL group and 101 (67, 135) months in sporadic group. Mean RFS was shorter in VHL related patients, compared with sporadic patients (P<0.001). Overall survival was also worse in VHL group (P=0.037). Nine patients in VHL group died (6 from cerebellar hemangioblastomas, 2 from renal cell carcinomas, 1 from pancreatic neuroendocrine tumors). Two sporadic patients died from recurrent cerebellar hemangioblastomas. Four different mutations were found in patients with VHL disease (559c→g, 716g→c, 697c→t, 746tgDel). Conclusion: The long-term prognosis of VHL associated cerebellar hemangioblastomas is still worse than sporadic cerebellar hemangioblastomas even after complete resection. Regular postoperative follow-up and examination of multiple organs is necessary for patients with VHL disease.
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