Multidisciplinary Treatment of Persistent Desmoid Tumor in a Patient with Gardner Syndrome

Tsutomu Nomura, Masahiko Onda, Akira Tokunaga, Kiyohiko Yamashita, Noritake Tanaka, Kiyonori Furukawa, Seizi Ito, Norio Matsukura, Kimiyoshi Yokoi, Shigeki Okazaki, Teruo Kiyama, Shigehiko Yokoyama

First Department of Surgery, Nippon Medical School

We report multidisciplinary treatment including surgery, immunotherapy, endocrine therapy, radiation and medication for a desmoid tumor in a Japanese patient with Gardner's syndrome. The patient was found to have polyposis coli with osteoma of the mandible at the age of 14 yr. syndrome. The patient was found to have polyposis coli with osteoma of the mandible at the age of 14 yr, and was diagnosed as having Gardner's syndrome. In 1984 he underwent total colectomy because of cancer of the sigmoid colon and polyposis coli. A mass removed from the left lower rectal sheath of the abdominal wall 2 years after colectomy was found to be a desmoid tumor histologically. In 1987, he underwent re-excision of a recurrent tumor in the abdominal wall and intratumor injeciton of OK-432, an immunostimulant bacterial preparation, for a retroperitoneal tumor which could not be removed because of infiltration into neighboring tissues. Postoperatively, he was given toremifene, an anti-estrogen, orally daily for 6 months. A CT series revealed that the retroperitoneal tumor had become small after treatment with OK-432 and toremifene. In 1988, he underwent re-excision of the recurrent tumor in the abdominal wall and radiation treatment for the retroperitoneal tumor. Thereafter no marked change in the abdominal and retroperitoneal tumors was observed for 2 years until metastatic lung cancer was revealed by chest X-ray in April 1990. The patient died 7 years after the colostomy. The multidisciplinary treatment employed might have contributed to the control of the desmoid tumor in this patient, although attempts to control the spread of cancer derived from polyposis coli failed.

Key words
Gardner's syndrome, desmoid tumor, toremifene

Reprint requests
Tsutomu Nomura First Department of Surgery, Nippon Medical School
1-1-5 Sendagi, Bunkyo-ku, Tokyo, 113 JAPAN

Accepted
March 11, 1992

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