Hemangioblastomas: clinical and histopathological factors correlated with recurrence

Abstract

Although hemangioblastomas are regarded as benign neoplasms, they may recur in up to 25% of cases. The clinicopathological correlates of this more aggressive behavior have not been identified. Among 26 patients with hemangioblastomas who had been evaluated and treated at the Massachusetts General Hospital between 1974 and 1986, 7 (27%) developed recurrences after surgical extirpation. Recurrence was correlated with younger age, that is less than 30 years at the time of diagnosis (P less than 0.01), von Hippel-Lindau syndrome (P less than 0.001), and the presence of multicentric tumors of the central nervous system at initial diagnosis (P less than 0.005). Histopathologically, recurring hemangioblastomas had lower frequencies of cyst formation (P = 0.10) and lower proportions of lipid-laden stromal cells (P less than 0.05). The findings suggest that a particular constellation of clinical and pathological features can be used to predict the likelihood of recurrence of a hemangioblastoma, and therefore to identify patients in need of long-term follow-up or, possibly adjunctive therapy.