P4 Spinal cord haemangioblastomas: surgical management and clinical outcome

Objectives Spinal haemangioblastomas are rare tumours that can be either sporadic or as part of the CNS expression of Von Hippel Lindau syndrome (VHL). The aim of this study was to identify the characteristics of the haemangioblastomas both in sporadic and in VHL cases and to assess the outcome of the surgical management. Methods We retrospectively reviewed all spinal haemangioblastomas that were operated between 2004 and 2017 including clinical letters, MRIs and pathology reports. Results A total of 14 patients underwent excision of a spinal haemangioblastoma. Average follow up time was 50 months (range 4–164 months). The location was: medulla n=3, cervical region n=4, thoracic region n=5 and conus n=2. Six patients had VHL (42%) and from those n=5 had more than one lesion. A syrinx was present in n=9 cases (VHL n=6, multiple lesions n=5) and 2 had a syringoperitoneal shunt inserted followed by excision of the haemangioblastoma. In all but one case a total excision was achieved and the syrinx disappeared or improved in 6 cases. A clinical improvement was seen in n=10 patients, in n=2 a deterioration was seen post-operatively and in n=2 new symptoms developed during their follow up from the other lesions. None of the haemangioblastomas that were removed recurred. Conclusions Total excision of spinal haemangioblastomas offers a good outcome with possible cure in cases of a single lesion. Patients with VHL present more commonly with multiple lesions and large syrinxes which pose a challenge to the surgical management.