Clark S K, Smith T G, Katz D E, Reznek R H, Phillips R K
The Polyposis Registry, St Mark's Hospital, Harrow, UK.
Br J Surg. 1998 Jul;85(7):970-3. doi: 10.1046/j.1365-2168.1998.00773.x.
Desmoid tumours occur in about 10 per cent of patients with familial adenomatous polyposis (FAP), and are an important cause of morbidity and death. The natural history of desmoids was investigated by documenting prospectively the prevalence and progression of possible precursor lesions.
A group of patients with FAP and controls were examined at laparotomy. Another group, with FAP and no clinical evidence of desmoid, and a group of controls, underwent abdominopelvic computed tomography.
At laparotomy 13 of 42 patients with FAP had fibromatous mesenteric plaques; seven of these had not had surgery. Seven had more extensive mesenteric fibromatosis and had undergone significantly more laparotomies than the rest. Of 103 patients scanned, two had desmoid tumours and 22 (21 per cent) had mesenteric fibromatosis. On follow-up both desmoid tumours grew rapidly; mesenteric fibromatosis was unchanged in eight and resolved in four of the 12 patients rescanned.
A model of desmoid tumour development is suggested, analogous to the adenoma-carcinoma sequence, in which a less benign phenotype emerges as molecular genetic abnormalities accumulate: mesenteric plaque-like desmoid precursor lesions arise in many patients with FAP before surgery as a result of abnormal fibroblast function; some, perhaps stimulated by surgery, progress to mesenteric fibromatosis; these in turn can give rise to desmoid tumours.
韧带样瘤发生于约10%的家族性腺瘤性息肉病（FAP）患者中，是发病和死亡的重要原因。通过前瞻性记录可能的前驱病变的患病率和进展情况，对韧带样瘤的自然史进行了研究。
一组FAP患者和对照组在剖腹手术时接受检查。另一组无韧带样瘤临床证据的FAP患者及一组对照组接受腹部盆腔计算机断层扫描。
在剖腹手术中，42例FAP患者中有13例有纤维瘤性肠系膜斑块；其中7例未接受过手术。7例有更广泛的肠系膜纤维瘤病，且接受剖腹手术的次数明显多于其余患者。在接受扫描的103例患者中，2例有韧带样瘤，22例（21%）有肠系膜纤维瘤病。随访中，两个韧带样瘤均迅速生长；12例再次扫描的患者中，8例肠系膜纤维瘤病无变化，4例消退。
提出了一种韧带样瘤发展模型，类似于腺瘤-癌序列，其中随着分子遗传异常的积累出现一种良性程度较低的表型：由于成纤维细胞功能异常，许多FAP患者在手术前出现肠系膜斑块样韧带样瘤前驱病变；一些病变可能受手术刺激进展为肠系膜纤维瘤病；这些病变继而可发展为韧带样瘤。
