Wells S A, Johnson A H, Gregory R O
Int Adv Surg Oncol. 1978;1:29-52.
Numerous clinical syndromes associated with multiple polypoid lesions of the gastrointestinal tract have been described. These syndromes generally have two striking characteristics: 1) Most of them are familial with the disease being inherited as a mendelian autosomal dominant trait, and 2) most of the affected patients have moderate to marked propensity to develop gastrointestinal carcinoma, usually of the large bowel. Whereas previously many of these syndromes were thought to be well-defined entities, it now appears that there is overlap between certain of them such that they appear more similar than different. The clinical and pathologic characteristics of the various gastrointestinal polyposis syndromes and the methods of diagnosis and treatment are reviewed in this manuscript.
已经描述了许多与胃肠道多发性息肉样病变相关的临床综合征。这些综合征通常有两个显著特征：1）它们大多是家族性的，疾病以孟德尔常染色体显性性状遗传；2）大多数受影响的患者有中度至明显的倾向发展为胃肠道癌，通常是大肠癌。虽然以前许多这些综合征被认为是明确的实体，但现在看来其中某些综合征之间存在重叠，以至于它们看起来相似之处多于不同之处。本文综述了各种胃肠道息肉病综合征的临床和病理特征以及诊断和治疗方法。
