大腸腺腫症における大腸腺腫および随伴性病変の推移

Follow-up Study on Colonic Adenomas and Accompanied Tumors in Familial Polyposis Coli and Gardner's Syndrome

要旨　1962年～1984年1月までに，国立がんセンター病院にて26家系49例（男37例，女12例）の大腸腺腫症を経験した．死亡は10例で，生存39例中11例は年齢15歳未満の小児例である．大腸病変以外の随伴性腫瘍の頻度は，顎骨内骨腫86.8％（33/38），十二指腸ポリープ71.9％（23/32），胃ポリープ56.8％（25/44），顎骨以外の骨病変（骨腫，外骨腫，骨皮質肥厚）50.0％（16/32），体表の軟部組織腫瘍（異時性を含む）27.0％（10/37），腸間膜デスモイド腫瘍13.5％（5/37），歯牙腫13.2％（5/38）であった．なお大腸癌以外の悪性腫瘍としては，胃癌2例（53歳　男，41歳　女），甲状腺癌1例（27歳　男）であった．本症における大腸腺腫，および種々の随伴性腫瘍の経過を観察した結果，以下の知見と推測が得られた．①外・中・内胚葉に由来する良性の腫瘍性病変の多くは，学童期には既に発生しており，思春期～青年期までは数の増加と大きさの増大がみられたが，それ以後は一般的にみて腫瘍の発育は止まるものと推定された．②中胚葉由来の悪性腫瘍（肉腫）の合併はまれである．③外胚葉由来で被蓋上皮が主体である上皮組織の悪性化もまれである．④内胚葉由来の組織でも，大腸，小腸，胃，甲状腺のごとく，腺上皮としての機能が主体である上皮組織の悪性化（癌腫）は多い．以上の知見から，大腸腺腫症の経過観察に際しては，内胚葉由来でかつ腺上皮と吸収上皮の性格を有する上皮組織，およびこの組織を有する器官・臓器を中心に行うことが重要であると考える．

　We experienced 49 cases of polyposis of the colon in 26 families (37 males, 12 females) at the National Cancer Center Hospital between 1962 and January 1984. Ten cases of them had expired and the rest 39 cases are still alive. Among the latter cases, 11 are children and less than 15 years old. Incidence of their accompanied tumors other than colonic one was as follows: Intramandibular osteoma 86.8% (33/ 38), duodenal polyp 71.9% (23/32), gastric polyp 56.8 (25/44), bone lesions other than the mandibular (osteoma, exostosis, hypertrophy of the bone cortex) 50.0% (16/32), soft tissue tumors 27.0% (10/37), mesenteric desmoid tumor 13.5% (5/37), and odontoma 13.2% (5/38). Malignant tumor other than colon cancer included two cases of gastric cancer (53 year-old man and 41 year-old woman) and thyroid cancer (27 year-old man). The prognosis of those colonic adenomas and various accompanied tumors was studied and following results were obtained:

　(1) Benign tumors originated from ectoderm, mesoderm and endoderm were already developed at school age and they increased in size and number by the age of puberty or youth. However, it was suspected that the tumor growth generally stopped afterwards.

　(2) They seldom accompany malignant tumor (sarcoma) originated from mesoderm.

　(3) Epithelial tissue which originated from ectoderm seldom become malignant.

　(4) Among the tissue originated from endoderm, if it has function of glandular epithelium such as colon, small intestine, stomach and thyroid gland, its malignant change was common.

　Therefore, in follow-up study of adenomatosis of the colon, it is important to investigate mainly the epithelial tissue as well as its containing organs which are originated from endoderm and has had function of glandular epithelium.