Multiple polyposis, gardner’s syndrome and desmoid tumors
Multiple polyposis, gardner’s syndrome and desmoid tumors
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多发性息肉病、加德纳综合征和硬纤维瘤
DOI：
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发表时间：
1958
影响因子：
3.9
通讯作者：
William G. Smith
中科院分区：
文献类型：
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作者：
William G. Smith
总结与结论Gardner最近描述了一种家族性综合征，它在某些方面与多发性结肠息肉病的关系与Peutz-Jeghers综合征与小肠息肉病的关系相同。除了家族性多发性息肉病外，该综合征的显著特征是(1)多发表皮样细胞增生症和(2)间充质细胞增生倾向，导致骨瘤、纤维瘤和硬纤维瘤的形成。表皮样囊肿和骨瘤产生明确的外部畸形，这可能导致医生怀疑结肠多发性息肉病。本文回顾201例多发性息肉病患者的临床资料。201例患者中有17例被发现表现出上述一种或多种异常。在这17名患者中，有8名患者在考虑到加德纳综合症的情况下进行了深入研究。17例患者中有6例有与腹部切口疤痕相关的硬纤维瘤，另外1例有腹外硬纤维瘤。在报告的17例患者中，有12例发现表皮样囊肿，8例发现骨瘤。人们认为，加德纳综合征是一系列病理变化的体现，这些变化可能以不同的数量和组合影响任何患有多发性息肉病的患者。
Summary and ConclusionsA familial syndrome recently has been described by Gardner which in some respects bears the same relationship to multiple polyposis of the colon that the Peutz-Jeghers syndrome does to polyposis of the small intestine. In addition to familial multiple polyposis, the salient features of the syndrome are (1) multiple epidermoid cystomatosis and (2) a tendency toward proliferation of cells of mesenchymal origin, resulting in the formation of osteomas, fibromas and desmoid tumors. The epidermoid cysts and osteomas produce definite external deformities which may lead the physician to suspect multiple polyposis of the colon.In this study, the records of 201 patients with multiple polyposis were reviewed. Seventeen of the 201 patients were found to exhibit one or more of the abnormalities listed above. Eight of these 17 patients were studied intensively with Gardner’s syndrome in mind. Six of the 17 patients had desmoid tumors associated with their abdominal incisional scars, and an additional patient had an extra-abdominal desmoid tumor. Epidermoid cysts were found in 12, and osteomas in eight, of the 17 patients reported upon.It is felt that Gardner’s syndrome is the embodiment of a spectrum of pathologic changes which could affect, in variable numbers and combinations, any patient who has multiple polyposis.