Kinzbrunner B, Ritter S, Domingo J, Rosenthal C J
Cancer. 1983 Dec 15;52(12):2201-4. doi: 10.1002/1097-0142(19831215)52:12<2201::aid-cncr2820521204>3.0.co;2-#.
A patient is described with Gardner's syndrome manifested initially by an extra-abdominal desmoid which was resected. The case was complicated by metastatic adenocarcinoma of the colon and recurrence of several large painful desmoid lesions. In view of the predilection of desmoids to occur in women in their childbearing years, it was decided to treat these painful lesions with an anti-estrogen, tamoxifen (20 mg orally, four times daily). This therapy led to a complete relief of pain within 1 week and a progressive decrease in the size of the desmoid tumors to less than 50% of their initial volume by the end of the second week. Unfortunately, the patient's metastatic adenocarcinoma progressed and was complicated by sepsis leading to her death. This case suggests that the growth of desmoid tumors is under hormonal influence, a suggestion which deserves further investigation.
本文描述了一名患有加德纳综合征的患者，最初表现为切除的腹外硬纤维瘤。该病例并发结肠转移性腺癌以及多个大型疼痛性硬纤维瘤病变复发。鉴于硬纤维瘤在育龄女性中更为常见，决定使用抗雌激素药物他莫昔芬（口服20毫克，每日四次）治疗这些疼痛性病变。该治疗在1周内使疼痛完全缓解，到第二周结束时，硬纤维瘤肿瘤大小逐渐减小至初始体积的50%以下。不幸的是，患者的转移性腺癌进展，并因败血症而死亡。该病例表明硬纤维瘤肿瘤的生长受激素影响，这一观点值得进一步研究。
