Lynch H T, Fitzgibbons R, Chong S, Cavalieri J, Lynch J, Wallace F, Patel S
Department of Preventive Medicine and Public Health, Creighton University School of Medicine, Omaha, Nebraska.
Dis Colon Rectum. 1994 Mar;37(3):260-7. doi: 10.1007/BF02048164.
The aim of this study was to describe the natural history and management of surgically unresectable intra-abdominal desmoid tumors in two patients with Gardner's syndrome from two unrelated families, where each had failed on conventional therapy.
Two patients with Gardner's syndrome were placed on a chemotherapy regimen which included doxorubicin (90 mg/m2) and dacarbazine (900 mg/m2) in divided doses over four days of continuous infusion. Their progress on chemotherapy was assessed by abdominal computerized tomography and laparoscopy.
The computerized abdominal tomography scans proved difficult to interpret because of adhesions and matted small bowel resulting from the patients original colectomies. These findings made it difficult to differentiate postoperative changes from residual desmoid tumor. Second-look laparotomy in such patients was contraindicated as this may predispose to further desmoid production. Laparoscopy disclosed a complete response to this chemotherapy. Nevertheless, we had an iatrogenic small bowel perforation in one of these patients. Each patient showed a complete response to chemotherapy.
Surgical resection remains the first-line treatment of intraabdominal desmoid tumors. However, doxorubicin/dacarbazine chemotherapy on a clinical trial basis may be indicated in patients whose intra-abdominal desmoid is unresectable, or who have failed to respond to treatment with hormones (tamoxifen, Toremifene), steroids (prednisone), and nonsteroidal anti-inflammatory agents (Clinoril; Merck & Co., Inc., West Point, PA).
本研究旨在描述两名来自两个无亲缘关系家庭的加德纳综合征患者，其腹腔内不可切除的硬纤维瘤的自然病史及治疗情况，这两名患者均对传统治疗无效。
两名加德纳综合征患者接受了化疗方案，该方案包括阿霉素（90毫克/平方米）和达卡巴嗪（900毫克/平方米），分剂量在连续四天的输注过程中给药。通过腹部计算机断层扫描和腹腔镜检查评估他们的化疗进展。
由于患者最初的结肠切除术导致粘连和肠管缠结，腹部计算机断层扫描结果难以解读。这些发现使得难以区分术后改变与残留的硬纤维瘤。此类患者禁忌进行二次剖腹探查，因为这可能会促使进一步产生硬纤维瘤。腹腔镜检查显示对该化疗有完全反应。然而，其中一名患者出现了医源性小肠穿孔。每名患者对化疗均显示出完全反应。
手术切除仍然是腹腔内硬纤维瘤的一线治疗方法。然而，对于腹腔内硬纤维瘤不可切除或对激素（他莫昔芬、托瑞米芬）、类固醇（泼尼松）和非甾体抗炎药（氯诺昔康；默克公司，西点，宾夕法尼亚州）治疗无反应的患者，基于临床试验的阿霉素/达卡巴嗪化疗可能是适用的。
