Gardner症候群の1例

A Case of Gardner's Syndrome

　1950年　Gardnerによって初めて遺伝的疾患で軟部組織腫瘍，骨腫，多発性結腸polyposisの三症候を有するものをGardner症候群として記載して以来，現在までに100例にみたない甚だまれな疾患である．最近われわれは家族的発生は認められなかったが前額部および下顎部の対称性骨腫，腹壁に生じたDesmoid，左半結腸に多発性polyposisを有しGardner症候群と思われる1例を経験したのでこれを報告し若干の文献的考察を加える．

　A man, 35 years old, entered the hospital to have his tumors on the jaw removed. Tumors were hard, bilateral and symmetrical. Although painless, they had begun to grow up since one and a half year ago. Detailed inquiry of his past history revealed that since his childhood he had noticed numerous uneven tumors on the frontal area as well as a painless mass in the lower left abdomen. For a long time the abdominal tumor remained unaltered in its size. Since several years ago he had also noticed slight anal bleeding at defecation. He is the only child and his father was known to have died from diabetic coma. None of his relatives has died of rectal cancer.

　At admission, little finger tip sized multiple osteomas were noticed on his forehead, in addition to symmetrical osteomas on his lower jaw. In the abdominal wall a hen-egg-sized subcutaneous tumor, hard yet elastic, was noticed in the left lower quadrant. Digital as well as barium enema examination of the rectum disclosed multiple polypoid tumors scattered in the descending colon, sigmoid and rectum. These numerous polyps were proved by biopsy as papillar adenoma with no malignant changes. On the basis of the above-mentioned findings, a diagnosis of Gardner's syndrome was made.

　Removal of the bilateral tumors on the lower jaw as well as the mass in the abdominal wall was then carried out. At the same time, the left hemicolectomy was also performed because malignant change of colonic polyps in the future must be taken into account.

　The postoperative course was uneventful and the patient is now under observation.