直腸癌を合併したGardner症候群の1例

A Case Report of Gardner's Syndrome Associated with Rectal Cancer

　1950年Gardner⁷⁾はhardtumor（骨腫），soft tumor（軟部組織腫瘍）の2徴候に消化管polyposisを合併した症例を報告して以来，欧米では同様の特徴を持つ症例が次々と報告させ，これがGardner症候群と命名された．本邦では1966年小坂ら¹⁶⁾¹⁷⁾が発表して以来，10例余の報告をみるにすぎない．

　最近，私共は家族的発生は認められなかったが，頭蓋骨，四肢骨の骨腫，胸背部のfibroma，歯芽形成異常，開腹術創部に発生したdesmoid tumor，消化管polyposis等の所見の他に直腸癌の併存をみた症例を経験，Gardner症候群と考えられるので報告する．

　The clinical features of Gardner's syndrome are recognized as familial polyposis, osteomas, soft tissue tumors and dental anomalies.

　We have found one patient of Gardner's syndrome with rectal cancer.

　A 35-year-old man was admitted to our hospital on November 26, 1971, with chief complaint of lower abdominal tumor. Detailed inquiry into his past history revealed that he had noticed a hard swelling on the occipital area since the age of five as well as another on the forehead since the age of 16 after contusion. In 1966, fibromas on the frontal chest and the right back were surgically removed. In May 1969, he was operated on for rectal cancer. Successively multiple polyposis of the sigmoid colon and rectum was found. In January 1971, a nut-sized tumor in the lower abdomen was noticed. It gradually grew up.

　Physical examination showed multiple hard swelling with the size of a finger-tip on the head, revealing also a hen-egg-sized, fixed elastic mass on the incisional scar in the lower abdomen. Roentgenograms of the skull and skeleton revealed osteomas involving the skull, angle of the jaw, right fibula and left tibia, disclosing also impacted teeth of both lower canines.

　Roentgenograms and fiberscopic examination of the stomach revealed diffuse polyposis. Barium enema through the artificial anus and small bowel series showed nothing abnormal.

　Removal of the lower abdominal tumor was performed. It was 5×4 cm in size and was located between the peritoneum and musculus rectus abdominus. The specimen showed a desmoid tumor.

　Hereditary evidence and chromosomal aberration were not noticed.