横行結腸にみられた不完全型Peutz-Jeghers症候群の1例

Incomplete Type Peutz-Jeghers Syndrome with a Solitary Polyp in the Transverse Colon, Report of a Case

要旨　患者は52歳，女性で，腹痛を主訴に受診．家族歴に特記すべきことはなし．皮膚，口唇，口腔粘膜に色素沈着を認めなかった．注腸透視，大腸内視鏡および腹部CTで横行結腸に直径4cm大の山田Ⅳ型ポリープを認め，横行結腸部分切除を施行した．組織学的にはPeutz-Jeghers型の過誤腫であったが，家族歴と，色素沈着を伴わないことから不完全型Peutz-Jeghers症候群と診断された．本症の特徴は，比較的高齢にみられ，ポリープは単発であることが多いことであった．また，Peutz-Jeghers症候群の代表的な家系からその初発患者の特徴を推測するに，本症の特徴と非常に類似していた．

　A 52-year-old female was admitted to our hospital complaining of abdominal pain. Barium enema, endoscopic examination and computed tomography revealed a solitary polypoid lesion in the transverse colon. Partial colectomy was performed. The polyp was measured 4×4×3.5 cm in size. Although she had neither mucocutaneous pigmentation nor family history of polyposis, examination of the resected specimen showed a hamartomatous polyp pathologically characteristic to Peutz-Jeghers syndrome. Incomplete type Peutz-Jeghers syndrome, i.e., hamartomatous polyp without pigmentation, tends to occur in elderly people and has a single polyp rather than multiple ones as seen in complete type. We considered patients with incomplete type are carriers of Peutz-Jeghers syndrome.