Lossignol D, Grossman S A, Sheidler V R, Griffin C A, Piantadosi S
Johns Hopkins Oncology Center, Baltimore, MD.
J Neurooncol. 1990 Oct;9(2):139-45. doi: 10.1007/BF02427834.
Three of 32 consecutive patients with astrocytomas enrolled in an experimental chemotherapy protocol had at least one first degree relative with an astrocytoma. These patients did not have family members with an excess of malignancies, known exposure to a specific environmental factor, or a recognized neurocutaneous syndrome. In all three families, the illnesses temporally overlapped and the age of the patients affected in the second generation was less than those in the first generation. An estimation of the probability of developing astrocytomas in families with one affected individual strongly suggests a familial effect. Our observations, coupled with other cases reported in the literature, suggest that familial astrocytomas occur more frequently than is currently recognized. Identification and careful study of such families may provide important clues to the etiology of these malignancies.
在一项实验性化疗方案中登记的32例连续性星形细胞瘤患者中有3例至少有一位患星形细胞瘤的一级亲属。这些患者没有恶性肿瘤过多的家庭成员,没有已知的特定环境因素暴露史,也没有公认的神经皮肤综合征。在所有三个家庭中,疾病在时间上有重叠,第二代受影响患者的年龄小于第一代。对有一名受影响个体的家庭中发生星形细胞瘤概率的估计强烈提示存在家族效应。我们的观察结果,再加上文献中报道的其他病例,表明家族性星形细胞瘤的发生比目前所认识的更为频繁。识别并仔细研究这些家庭可能为这些恶性肿瘤的病因提供重要线索。