gms | German Medical Science

Objective: Hemangioblastomas are vascular tumors of the central nervous system (CNS), which may occur as part of von Hippel-Lindau disease (VHL). Although histologically benign, these tumors are notorious for hemorrhagic complications.

Methods: We reviewed our clinical database of patients with CNS hemangioblastomas for the incidence of spontaneous or perioperative hemorrhage. Clinical characteristics such as tumor size, tumor location, VHL status and clinical symptoms before hemorrhage were correlated with the risk of bleeding.

Results: Data of 277 patients with hemangioblastomas were available. The spontaneous bleeding probability in our cohort of hemangioblastomas was 0.0024 per person and year. The average diameter of tumors that had spontaneous hemorrhage was 3 cm, which is significantly larger than the average diameter of hemangioblastomas without hemorrhage. We have furthermore observed severe postoperative hemorrhage in three hemangioblastomas. All of them were extraordinarily large (>4 cm) and solid tumors.

Conclusions: The overall incidence of spontaneous hemorrhage in hemangioblastoma patients is low. An important indicator for the bleeding probability is the size of the tumor, since all spontaneous or postoperative bleedings occurred exclusively in extraordinarily large tumors. Hemangioblastomas with a size of less than 1.5 cm (the vast majority of these tumors) harbor virtually no risk of spontaneous hemorrhage. Large solid hemangioblastomas do have a significant risk of postoperative hemorrhage. Preoperative embolization should be considered in these tumors.