Spigelman A D, Murday V, Phillips R K
St Mark's Hospital, London.
Gut. 1989 Nov;30(11):1588-90. doi: 10.1136/gut.30.11.1588.
Among 72 patients with the Peutz-Jeghers syndrome malignant tumours have developed in 16 (22%) of whom all but one have died. There were nine gastrointestinal and seven nongastrointestinal tumours. The relative risks of death from gastrointestinal cancer and all cancers were 13 (95% CI 2.7-38.1) and 9 (95% CI 4.2-17.3) respectively. The chance of dying of cancer by the age of 57 was 48%. There is evidence for a hamartoma/carcinoma sequence in the Peutz-Jeghers syndrome, suggesting that the gene locus involved is relevant to the development of malignancy in general.
在72例黑斑息肉综合征患者中，16例（22%）发生了恶性肿瘤，其中除1例之外均已死亡。有9例胃肠道肿瘤和7例非胃肠道肿瘤。因胃肠道癌和所有癌症死亡的相对风险分别为13（95%可信区间2.7 - 38.1）和9（95%可信区间4.2 - 17.3）。到57岁时死于癌症的几率为48%。有证据表明黑斑息肉综合征存在错构瘤/癌序列，提示所涉及的基因位点通常与恶性肿瘤的发生有关。
