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Purpose: 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., Methods: 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., Results: 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., Conclusion: 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).

Desmoid tumors are rare, but not in patients with Gardner's syndrome. Although standard treatment is wide surgical excision, we prescribed chemotherapy for a 30-year-old woman with intra-abdominal desmoids, a complication after subtotal colectomy for the treatment of familial polyposis coli. Exploratory laparotomy revealed that the desmoids were not resectable. Chemotherapy with combinations of vincristine, azathioprine, cyclophosphamide, and prednisone was prescribed for over 3 months, and a partial regression of the tumors occurred. Two years after the initiation of the chemotherapy, complete regression of tumors was achieved with cyclophosphamide and prednisone. Surgical excision, irradiation, and chemotherapy treatments are discussed.

A 48-year-old man with Gardner's syndrome, who had abdominoperineal resection for rectal carcinoma in 1962, was found to have an ulcerating growth of the duodenum, and pancreaticoduodenectomy was performed in 1979. Histologic examination by complete step-serial sectioning disclosed a well-differentiated adenocarcinoma with adenomatous remnants, a large adenoma with focal carcinoma, 256 adenomas of the duodenum, and 91 adenomas of the gastric antrum. The world medical literature was reviewed, and 29 cases of periampullary carcinoma and 12 cases of gastric carcinoma complicating familial polyposis coli or Gardner's syndrome were analyzed.

Mesenteric desmoid tumors are a recognized sequela of colectomy for polyposis coli of Gardner's type. Relentless growth and recurrence carry a poor prognosis. Recently, nonsteroidal anti-inflammatory drugs have been used to halt the growth of these tumors, presumably by interfering with prostaglandin metabolism. A 36-year-old man presented with small-bowel obstruction secondary to a large, diffuse mesenteric desmoid six years following colectomy and ileoproctostomy. Laparotomy revealed it to be unresectable. Postoperatively, he was started on sulindac (Clinoril) 100 mg twice a day. His obstruction resolved, and he remains well at 11 months. A CT scan shows diminution in the size of the tumor. Nonsteroidal anti-inflammatory agents may be an alternative to chemotherapy and radiotherapy in treating mesenteric desmoids.

A case is reported of a 33-year-old man with hepatocellular carcinoma associated with familial polyposis of the colon. During subtotal colectomy for diffuse colonic polyposis, a small tumor was excised from the right lobe of the liver. Histologic examination revealed hepatocellular carcinoma. The association of primary hepatoma in familial polyposis of the colon is very rare. However, it is well known that familial polyposis of the colon has a potent oncogenicity not only in the colon but also in the extracolonic organs. The hepatoma may also be one of the extracolonic malignant manifestations in this inherited disease.