いわゆる家族性大腸ポリポージスの随伴性病変―胃，骨・歯牙の病変について

Lesions Associated with on the So-called Familial Polyposis of the Colon: On lesions of the stomach, bones and teeth

　人に発生する腫瘍について，その遺伝的背景が重要視されているものがある．その中でも家族性大腸ポリポージスは，大腸に無数の腺腫性ポリープがびまん性に密生し，しかも高率に癌化する疾患でありメンデルの常染色体性優性遺伝法則に従って子孫に遺伝するものとされている．すなわち，家族性大腸ポリポージスは腫瘍の発生に遺伝が関与する典型的な疾患であり，腫瘍発生論上きわめて興味ある，またきわめて重要な疾患であるといえる．著者らは，家族性大腸ポリポージスとみなされていた症例について，全消化管および頭蓋骨撮影，下顎骨撮影，pantomographyなどの検査を行なった結果，上部消化管，とくに胃に67％の高率に隆起性病変を認め，また骨および歯牙に75％の高率に異常を発見した．これらの事実より，家族性大腸ポリポージスは多発性腫瘍素因を有する全身性疾患とみなすべきと老え，若干の文献的考察を加え報告する．

検索成績

　対象は，表1のごとく8家系17症例であり，男性13例，女性4例である．年齢分布は，7歳から48歳にわたっている．以下の検索成績については，要約して箇条書きにし，X線写真の提示を重点的に行なうこととする．

　Gardner's syndrome (a familial disease characterized by colonic polyposis, osteoma and soft tissue tumor) and familial polyposis of the colon have two theories for the pathogenesis. One is that they are caused by different genes ; and the other is that both originate from the same single gene. However, up to the present hardly any examination of the stomach, bones and teeth has been made in the sense of comparison between the two. Such being the case, we have attempted to examine along this line eight pedigrees (17 patients) apparently regarded as having familial polyposis of the colon. Examination of the entire gastrointestinal tract in most of the patients along with x-ray study of the skull and mandible and its pantomography revealed, in 10 out of 15 patients whose stomachs were examined either with x-ray or endoscopy, multiple sessile hemispheric protrusions as large as a grain of rice or a red bean, most often located in the upper segment of the stomach. Gastric biopsy was performed in 6 patients. Of 12 patients whose bones were examined we have found in 9 (75 per cent) minute changes in x-ray pictures of the bones and teeth otherwise utterly impossible to detect (small osteoma in 4 patients, exostosis in 2, multiple osteosclerotic areas in the mandible in 8, impacted teeth in 2, odontoma in 1 and a suspicion of adamantoblastoma in 1.) In addition, soft tissue tumor was found in 3 patients (a epidermal cyst in 1). These facts have shown us that there exists no distinct qualitative difference between the two diseases, although morphologically they may differ or less. Thus, we may conduct concluison that these two disease entities are substantially the same, and they both should be regarded as a systemic disease with a predisposition to multiple tumor formation.