COMPLICATIONS OF THE NEVOID BASAL-CELL CARCINOMA SYNDROME - RESULTS OF A POPULATION-BASED STUDY
COMPLICATIONS OF THE NEVOID BASAL-CELL CARCINOMA SYNDROME - RESULTS OF A POPULATION-BASED STUDY
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DOI：
10.1136/jmg.30.6.460
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发表时间：
1993-06-01
影响因子：
4
通讯作者：
FARNDON, PA
中科院分区：
文献类型：
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作者：
EVANS, DGR;LADUSANS, EJ;FARNDON, PA
有许多潜在的并发症，已报道与基底细胞瘤瘤样癌综合征。我们通过对英格兰西北部84个病例的人口研究，得出了这些问题的相对频率。基底细胞癌和颌骨囊肿的主要并发症发生在超过90%的患者40岁之前，但两者都可能发生在10岁之前。较少描述的并发症有卵巢钙化或纤维瘤（24%）、髓母细胞瘤（5%）、心脏纤维瘤（3%）、腭裂（5%）和眼科异常，如斜视或白内障（26%）。这项研究更清楚地定义了该综合征可能的并发症，并为受影响和有风险的人提供了更明确的咨询和筛查指南。
There are many potential complications which have been reported in association with the naevoid basal cell carcinoma syndrome. We have been able to show the relative frequencies of these problems in a population based study of 84 cases in the north west of England. The major complications of basal cell carcinomas and jaw cysts occur in over 90% of patients by 40 years of age, but may both occur before 10 years of age. Less well described complications are ovarian calcification or fibroma (24%), medullo-blastoma (5%), cardiac fibroma (3%), cleft palate (5%), and ophthalmic abnormalities such as squint or cataract (26%). This study more clearly defines the possible complications of the syndrome and gives clearer guidelines for counselling and screening affected and at risk persons.