von Recklinghausen病に十二指腸カルチノイドが合併しZollinger-Ellison症候群を呈した1例  [in Japanese] DUODENAL CARTINOID WITH ZOLLINGER-ELLISON SYNDROM IN A PATIENT WITH VON RECKLINGHAUSEN'S DISEASE  [in Japanese]

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53歳女性.2歳時に von Recklinghausen 病と診断された.41歳時に右側の,53歳時に左側の各々後腹膜神経線維腫摘出術を受け,51歳時に脊椎神経線維腫に対し椎弓切除術を受けた.50歳頃より下痢,難治性消化性潰瘍が出現し,同時に高ガストリン血症も認めたため,Zollinger-Elliso症候群が疑われた.平成11年8月20日心窩部痛,嘔吐にて緊急入院した.入院4日目に下血を認め,上部消化管内視鏡検査にて十二指腸下行脚に多発性潰瘍と乳頭部近傍の粘膜下腫瘍性病変を認めた.血中ガストジン値は1160pg/mlと著明に上昇していた.9月20日に膵頭十二指腸切除術を施行した.術中に幽門下リンパ節の転移を認めた.病理組織検査で乳頭開口部近位側こ径4mm大のカルチノイドを認め,免疫組織染色ではガストリンの陽性細胞像を呈した.術後血中ガストリン値は正常に復した。

A 53-year-old woman with von Recklinghausen's disease was admitted to the hospital because of abdominal pain and vomiting. She had been diagnosed as von Recklinghausen's disease at the age of two, and undergone removals of left and right retroperitoneal neurofibromas at the ages of 41 and 53respectively as well as spinal neurofibroma at the age of 41. Since 50 years of age, diarrea and intractable peptic ulcer appeared and at the same time, hypergastrinemia was noted. Zollinger-Ellison syndrome had been suspected. On the 4th hospital day, massive melena occurred. Endoscopic examination showed multiple duodenal ulcers and a submucosal tumor close to the Papilla Vater. As she had marked hypergastrinemia to 1160pg/ml, Zollinger-Ellison syndrome was suspected. Pancreaticoduodenectomy was performed, when a submucosal tumor close to the Papilla Vater and lymph node metatstasis were found. Pathological examination revealed doudenal cartinoid 4mm in diameter, which was positive for gastrin by immunohistochemical staining. Hypergastrinemia disappeared postoperatively.


  • Nihon Rinsho Geka Gakkai Zasshi (Journal of Japan Surgical Association)

    Nihon Rinsho Geka Gakkai Zasshi (Journal of Japan Surgical Association) 62(2), 400-405, 2001-02-25

    Japan Surgical Association

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