de la Monte S M, Horowitz S A
Department of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston.
Neurosurgery. 1989 Nov;25(5):695-8.
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.
尽管血管母细胞瘤被视为良性肿瘤,但在高达25%的病例中可能会复发。这种更具侵袭性的行为的临床病理相关性尚未明确。在1974年至1986年间于麻省总医院接受评估和治疗的26例血管母细胞瘤患者中,7例(27%)在手术切除后出现复发。复发与以下因素相关:年龄较轻,即诊断时小于30岁(P<0.01);冯·希佩尔-林道综合征(P<0.001);初次诊断时存在中枢神经系统多中心肿瘤(P<0.005)。组织病理学上,复发性血管母细胞瘤形成囊肿的频率较低(P=0.10),富含脂质的基质细胞比例较低(P<0.05)。这些发现表明,特定的临床和病理特征组合可用于预测血管母细胞瘤复发的可能性,从而识别需要长期随访或可能需要辅助治疗的患者。