Järvinen H J
Second Department of Surgery, University Central Hospital, Helsinki, Finland.
Acta Chir Scand. 1987;153(5-6):379-83.
In the Finnish Polyposis Registry with 71 known families and 168 verified adenomatosis coli patients, 19 (11%) cases were complicated by desmoid tumors. Six polyposis patients (3.6%) had a desmoid before colectomy and the cumulative risk of postoperative desmoid tumors was 16% at ten years after colectomy. The median interval between the operation and the diagnosis of desmoid was 2.3 years. Eleven women and eight men were affected; the mean age was 28.5 years. Development of postoperative desmoid tumors was associated with early age at the time of colectomy, 26 years versus 38 years in those without subsequent desmoids (p less than 0.01). In two families more than one polyposis patient were affected by desmoids. All patients with desmoid tumors had other accompanying extracolonic lesions. No deaths from desmoid disease have occurred, even though only four of eight mesenteric tumors and five of nine abdominal wall tumors (53%) were excised without evidence of recurrence. Operative treatment is required in most cases, but resection may involve great hazard especially in mesenteric desmoids.
在拥有71个已知家族和168名经证实的结肠腺瘤病患者的芬兰息肉病登记处中，19例（11%）患者并发了硬纤维瘤。6例息肉病患者（3.6%）在结肠切除术之前就患有硬纤维瘤，结肠切除术后10年发生术后硬纤维瘤的累积风险为16%。手术与硬纤维瘤诊断之间的中位间隔时间为2.3年。11名女性和8名男性患病；平均年龄为28.5岁。术后硬纤维瘤的发生与结肠切除术时的年龄较小有关，有后续硬纤维瘤的患者结肠切除时年龄为26岁，无后续硬纤维瘤的患者为38岁（p小于0.01）。在两个家族中，不止一名息肉病患者患有硬纤维瘤。所有患有硬纤维瘤的患者都有其他伴随的结肠外病变。尽管8例肠系膜肿瘤中只有4例以及9例腹壁肿瘤中有5例（53%）被切除且无复发迹象，但尚未有因硬纤维瘤病死亡的病例。大多数情况下需要进行手术治疗，但切除可能具有很大风险，尤其是肠系膜硬纤维瘤。
