Treatment for gastrinoma has changed significantly since the syndrome was originally described in 1955. Initially, most patients developed severe symptoms and underwent emergency surgery for complications such as massive hemorrhage or perforation. Partial gastrectomy with or without vagotomy was an insufficient treatment, hence total gastrectomy became the standard operation for patients with gastrinoma. The development of effective antisecretory drugs has drastically changed the management of gastrinoma. In addition, recent advances in localization techniques, such as the selective arterial secretagogue injection test (SASI test) and somatostatin receptor scintigraphy (SRS) have promoted curative resection surgery for patients with gastrinoma. The goals of gastrinoma treatment are management of gastric acid hypersecretion and removal of the risk of distant metastasis and ultimately death of the patient by resecting tumors that are usually malignant. As the number of resection surgery procedures for gastrinoma has increased, it was noted that duodenal gastrinoma was detected more frequently than pancreatic gastrinoma in patients with Zollinger–Ellison syndrome. Curative resection surgery for gastrinoma has almost become standardized using the SASI test for localization of gastrinoma. The treatment strategy for gastrinoma for patients with multiple endocrine neoplasia type 1 (MEN1) has not been established, but resection surgery has been proved to contribute to the prolongation of survival time. The most important prognostic factor in patients with gastrinoma is hepatic metastases. The treatment strategy for hepatic metastases of gastrinoma has not been established and aggressive resection with chemotherapy and transarterial chemoembolization have been performed with significant benefit. Prospective studies of mammalian target of rapamycin (mTOR) inhibitors with or without octreotide and tyrosine kinase inhibitors have shown significant prolongation of survival time of patients with gastrinoma. New cytotoxic chemotherapy with temozolomide and capecitabine have also been reported to be effective, and these drugs are expected to among the new agents for gastrinoma.

Gastrinoma and insulinoma are the two most common functional pancreatic neuroendocrine tumors (PanNET), but there are also various kinds of rare PanNET, including glucagonomas, vasoactive intestinal peptide-releasing tumors (VIPomas) (Verner–Morrison syndrome, pancreatic cholera, watery diarrhea, hypokalemia, and achlorhydria [WDHA] syndrome), somatostatinomas, and other PanNET that secrete calcitonin, renin, luteinizing hormone, erythropoietin and insulin-like growth factor II. The diagnosis of rare PanNET requires the demonstration of an inappropriate elevation of the specific serum hormones combined with clinical and/or laboratory evidence of oversecretion of the appropriate hormone. Most rare PanNET present with metastatic disease and the survival time of patients is determined by the growth of the tumor rather than the hormone excess state. The best treatment for rare PanNET is curative surgical resection, which needs to be performed before liver metastasis develops. Indications for surgery depend on clinical symptom control, tumor size, location, extent, malignancy, and metastatic spread. Curative surgery should be indicated whenever possible, even in the presence of metastatic disease, including resectable hepatic metastases, when the patient can tolerate the surgery. In order to achieve successful surgical resection, a tumor localization study is important. Recent advances in localization techniques, such as the selective arterial secretagogue injection (SASI) test and somatostatin receptor scintigraphy (SRS), have promoted curative resection surgery. The types of surgery include pancreaticoduodenectomy, distal pancreatectomy, tumor enucleation, and enucleation in combination with pancreatic resection depending on the location of the primary tumor. Laparoscopic resection is currently not recommended because lymph node dissection and careful inspection for invasion and metastases are needed. Both somatostatin analogs and interferon have been shown to be effective in the control of symptoms in rare PanNET. Prospective studies of mammalian target of rapamycin (mTOR) inhibitors with or without octreotide and tyrosine kinase inhibitors have shown significant prolongation of survival time of the patients. New cytotoxic chemotherapy with temozolomide and capecitabine have also been reported to be effective.

CASE REPORT A 2-day-old girl, born to a healthy 34-year-old woman at the 36th week of gestation, was transferred to our hospital after she presented with bilious emesis. A gastrointestinal x-ray series with contrast revealed microcolon and ileal obstruction (Figure 1). Our differential diagnosis for the neonatal small bowel obstruction included ileal atresia, intestinal malrotation, meconium plug syndrome, and Hirschsprung disease.Figure 1.: Gastrointestinal x-ray series images with contrast at admission. (A) Contrast enema showing microcolon. (B) Contrast injected through the gastric tube shows ileal obstruction (arrows).She underwent exploratory laparoscopy, which revealed no anatomical anomaly, although the small intestine was filled with meconium. At 100 cm from the terminal ileum, the color of meconium, as seen through the intestinal wall, changed from white to green. The color change was even more distinct when the intestine was incised, creating a sharp contrast (Figure 2). Moreover, the proximal jejunum demonstrated a caliber change 35 cm distal to the ligament of Treitz, and its full-thickness biopsy revealed near total intestinal aganglionosis, highly suggestive of extensive type of Hirschsprung disease. Hence, we performed diverting jejunostomy 1 week after the previous operation, and she is currently gradually growing with parenteral and enteral nutrition support.Figure 2.: (A) The intestine showing an obvious change in color of its contents (forceps). (B) Color transition of meconium indicating the moment when bile began to flow into the duodenum.Hirschsprung disease is a congenital disorder characterized by complete absence of neuronal ganglion cells at the end of the large intestine, with continuous involvement of the proximal intestine. In less than 1% of patients, aganglionosis extends through nearly the entire intestine, and, although rare, such extensive aganglionosis can be a cause of neonatal small bowel obstruction.1 In the present case, the impressive color transition of meconium was representative of the precise moment when bile had begun to flow into the duodenum during the embryonic period. A hepatic diverticulum appears from the primitive midgut in the fourth week of gestation. The biliary system starts forming in the fifth week.2,3 The common bile duct elongates with its lumen plugged by epithelial cells. Recanalization of its lumen starts at the end of the fifth week.2,4 By the 13th week, bile flows through the extrahepatic biliary tree into the duodenum.2,5 It is presumed that the color transition in the present case was distinct because ileal aganglionosis and poor peristalsis resulted in inadequate mixing of meconium. DISCLOSURES Author contributions: R. Ataka wrote the manuscript. N. Takada and A. Sekiya revised the manuscript for intellectual content and approved the final manuscript. R. Doi is the article guarantor. Financial disclosure: None to report. Informed consent was obtained for this case report.

症例は59歳女性.人間ドックで嚢胞性膵病変を指摘され当院へ紹介された.腹部造影CTで膵尾部に50×30mmの多房性嚢胞性病変を認めた.中心部の嚢胞には早期相で濃染する充実成分を認め,周囲をより大きな嚢胞が取り囲む分葉状形態をとっていた.MRIではT2強調画像で高信号を呈する多嚢胞性病変として描出された.EUSでは嚢胞内結節,蜂巣状構造を認め,ERPにより主膵管と交通する嚢胞を認めた.膵液細胞診はClass IIであった.以上よりSCN(microcystic type)あるいはIPMN(gastric type),またその併存の術前診断のもと,膵体尾部切除術を施行した.病理組織学的検査はSCNとIPMNの併存であった.本例は異なった種類の嚢胞性腫瘍が隣接併存した非常に稀な症例と考えられた.嚢胞性膵腫瘍の診療では異なる腫瘍の併存も念頭におき,精度の高い診断と適切な治療を行うことが肝要である.

症例は39歳，女性．3年前に子宮頸癌に対して，広汎子宮全摘術および骨盤リンパ節郭清術，骨盤部放射線治療を施行された．突然の右下肢痛を主訴に当院救急外来を受診し，腹部造影CTで回腸にclosed loopがあり，絞扼性腸閉塞を疑った．腹部症状は乏しく非典型的であったが，手術既往を考慮し緊急手術を施行した．術中所見は，前回の手術で遊離された右外腸骨血管と右内腸骨血管の間に回腸が入り込んでいた．発症早期に整復できたため腸管切除は実施しなかった．術直後から右下肢痛は消失し，術後8日目に退院した．骨盤リンパ節郭清術後の絞扼性腸閉塞の症例は少数報告されているが，腹部症状が主訴であった．本症例の右下肢痛は拡張腸管による閉鎖神経の圧排が原因と考えられた．骨盤内リンパ節郭清術後の絞扼性腸閉塞においては，本症例のように下肢痛という非典型的な主訴で発症があることも念頭に置き，診察にあたる必要がある．

A 47 ― year ― old woman presented with a cystic lesion in the tail of the pancreas, and was referred for further investigation. Contrast ― enhanced computed tomography scan revealed a multilocular cyst with septa, measuring 46 mm in diameter. On magnetic resonance imaging, the cyst had heterogeneous hy-perintensity on T2 ― weighted images. Contrast ― enhanced endoscopic ultrasound revealed a cyst ― in ― cyst ― like structure with cystic portions giving a honeycomb appearance. The lesion was diagnosed as a mucinous cystic neoplasm in the tail of the pancreas and treated with laparoscopic distal pancreatectomy. Histopathologically, the cyst originated in the spleen and was invaginated into the tail of the pancreas. The cystic wall was lined by stratified squamous epithelium and the final diagnosis was an epidermoid cyst of the spleen. An epidermoid cyst of the spleen invaginating into the tail of the pancreas should be taken into consideration when a cystic lesion is located at the very distal end of the pancreas.

退形成性膵管癌は非常に頻度の少ない腫瘍でその悪性度は非常に高い.われわれは,2回の膵切除手術と化学療法で長期生存した退形成性膵管癌の症例を経験した.症例は70歳男性.糖尿病の悪化を契機に膵頭部癌と診断し手術を施行した.病理組織検査は腫瘍径9mm,TS1の破骨細胞型退形成性膵管癌の診断であった.術後2年5か月の腹部造影CT検査で残膵に22mmの腫瘤と尾側膵管の拡張を認めたため,腫瘍の再発を疑い残膵全摘術を施行した.病理組織検査は,初回と異なる亜型の紡錘細胞型退形成性膵管癌であり,残膵の新規病変と判断した.退形成性膵管癌が異時性に膵内に重複発生した例は本邦初である.残膵全摘術後に再発をきたしたものの,2回の手術を含む集学的治療で本患者は長期生存しており,積極的な手術加療が予後を改善する可能性が示唆された.

症例は64歳,男性.健診の腹部超音波検査で膵体部に腫瘤性病変を指摘された.術前に組織学的診断を得ることはできなかったが,画像検査では膵神経内分泌腫瘍を第一に疑い,また膵粘液癌などの悪性疾患を否定できなかったため,手術の方針とした.術式は腹腔鏡下膵体尾部切除術とした.病理診断は後腹膜神経鞘腫であった.本疾患の術前診断は困難であるが,CTで遅延性に造影される腫瘤を膵周囲に認めた際は,本疾患を鑑別の1つに挙げることが重要であると考えられた.

INTRODUCTION:Gastrointestinal (GI) involvement in hepatocellular carcinoma (HCC) is uncommon. In particular, HCC with duodenal invasion is known to be a rare condition. In such cases, surgical indication has been generally negative except in few reported cases. To our knowledge, this report describes the first case of HCC with duodenal invasion, resected by hepatectomy accompanied by pancreas-preserving partial duodenectomy (HPPD) following multimodal therapies including systemic sorafenib administration. CASE PRESENTATION:A 65-year-old man had been repeatedly treated for multiple HCCs by transarterial chemoembolization (TACE) and sorafenib. However, the main tumor formerly ruptured began to involve his duodenum, causing GI bleeding. The collateral vessels from the pancreatic and omental branches entered the tumor and nullified the transarterial hemostatic embolization. Hence, HPPD was performed to preserve the major Vater papilla. Histopathological examination revealed poorly-to -moderately differentiated HCC cells invading the duodenum. DISCUSSION AND CONCLUSION:HPPD treatment successfully removed HCC with duodenal invasion achieving viable tumor clearance status (R0). We underline the importance of achieving viable tumor clearance status at any time during the treatment course of patients with advanced HCC as this approach may be the only approach to enable HCC patients with duodenal invasion to resume a healthy life.

症例は66歳，男性．10年前から左鼠径部の膨隆を認めた．徐々に両側性となり，5年前から左右とも非還納性となった．陰嚢サイズはそれぞれ約20×10×10cmで，立位で陰嚢下端が大腿内側中点を越える両側巨大鼠径ヘルニアと診断した．還納容積が大きく，還納後の腹部コンパートメント症候群が懸念されたため，腹腔内圧に近似できる膀胱内圧を測定しつつ周術期管理を行った．全身麻酔導入後，気腹開始前に，両側ともヘルニア還納が可能であったが，膀胱内圧の上昇は認めなかったため，腹腔鏡手術で両側を一期的に修復した．術後の膀胱内圧上昇は軽度で，腹部コンパートメント症候群は認めず，術後7日目に退院した．術後6カ月を経過したが再発はない．今回，膀胱内圧を参照することで，腹部コンパートメント症候群の発症を危惧することなく，腹腔鏡下に一期的に手術しえた両側巨大鼠径ヘルニアの1例を経験したので，文献的考察を加えて報告する．

鉱質コルチコイド反応性低ナトリウム血症（mineralocorticoid responsive hyponatremia of the elderly:以下MRHE）は，著明な低ナトリウム血症をきたす疾患である．今回，膵体尾部切除術後に，MRHEを発症した症例を経験したので報告する．症例は70歳，男性．血糖コントロール悪化の精査で膵尾部に腫瘍を指摘され，当院へ紹介された．腹部造影CT検査，EUS-FNA検査で切除可能膵尾部癌と診断．腹腔鏡下膵体尾部切除術を施行した．術後15日目に著明な低ナトリウム血症（114mEq/l）を認めた．血清コルチゾールが正常で，脱水所見を認めたことより，MRHEと診断し，鉱質コルチコイド製剤の内服を開始した．術後24日目には，血清ナトリウム値が138mEq/lまで改善し退院した．その後，血清ナトリウム値は正常範囲で推移し，退院3カ月後には鉱質コルチコイド製剤は終了した．

Gastrointestinal Burkitt lymphoma (BL) is a highly aggressive malignancy in childhood, and early treatment is critical for its favorable prognosis. Ultrasonography is a widely accepted initial imaging workup; therefore, recognition of the sonographic features of BL should contribute to its early diagnosis and initiation of treatment. We present a 4-year-old boy with primary jejunal BL with intussusception mimicking presentation, in which initial abdominal US allowed sustainable detection and characterization of the intestinal lesion. Jejunotomy was performed and histopathological analysis revealed a 'starry sky' pattern and c-myc split signals characteristic of BL. The patient remains disease-free following chemotherapy.

It is evident that safe complete resection of the target tumor without excess or deficiency is the most important object in the oncological surgery, besides this is particularly true in the laparoscopic surgery for pancreatic neuroendocrine tumors (PanNETs).

症例は56歳の女性で，9年前に右浸潤性乳管癌に対して右乳房切除術および腋窩リンパ節郭清を施行され，術後3年目までに肝・骨転移を認めたが集学的加療を行うことでそれぞれ完全・部分奏効を得ていた．術後6年目に胆囊に小隆起性病変を認め，術後9年目にはそれ以前に認めなかった急峻なCEAの上昇（45.6 ng/ml）とPETで強い集積を認め，胆囊体部の2.5 cm大の広基性隆起性病変への増大が指摘された．穿刺生検は腫瘍播種の危険性を考慮し施行せず，胆囊ならびに胆囊床部切除を行い，病理組織学的検査では胆囊壁全層に胞巣状・索状の腺癌の病変と，肝浸潤・リンパ節転移を認めた．ホルモン受容体およびHER2サブタイプは乳癌病変と一致した．乳癌胆囊転移の症例は他に28例を認めるが，うち浸潤性乳管癌では6例とまれである．術後長期を経た浸潤性乳管癌であっても，胆囊の腫瘍性病変の鑑別には乳癌転移を考える必要がある．

急性上腸間膜動脈（SMA）閉塞症は予後不良として知られ，中でもSMAガス血症を伴うものは極めて稀で救命の報告例は無い．今回，SMAガス血症から救命しえた症例を報告する．65歳の女性で，急激な心窩部痛で発症し造影CT検査と血管造影にてSMAの空腸動脈第1枝直後での閉塞を指摘し，血栓吸引および溶解療法を施行した．SMA本幹に分節的な閉塞が残るものの，第1～4空腸動脈から辺縁動脈を介して回結腸動脈などSMA末梢枝が造影されるまでの改善をみたが，CT上わずかながら門脈ガス血症を認め厳重観察とした．2時間後のCTにてSMAガス血症を検出し緊急手術とした．術中ガス血症範囲に一致した腸管壊死を認め，上行結腸20cmまでの腸管切除を要したが術後1年4カ月現在も存命中である．わずかな門脈ガス血症から急速にSMAガス血症にまで進展した経過を観察しえ，時機を逸さず手術を施行し救命しえたのでこれを報告する．

症例は53歳男性.腹部造影CTで膵頭部に造影効果のない低濃度腫瘤を認め,膵頭部癌と診断した.腹腔動脈幹からは総肝動脈分岐はみられず,膵頭部膵内を上腸間膜動脈から分岐した膵アーケードを介し,右肝動脈へと続く脈管を認めた.血管造影を行い,まず上腸間膜動脈から膵頭部領域までカテーテルを選択的に誘導し,バルーンにて閉塞した.しばらく時間をおき腹腔動脈から造影を行うと,左胃動脈経由の左肝動脈から右肝動脈領域へのコミュニケーション血管が拡張し,右肝血流を認めたため,膵頭部域での血管塞栓術を施行した.塞栓術後も肝機能悪化は認めず,22日後に亜全胃温存膵頭十二指腸切除術を施行した.術後も肝不全なく退院した.膵癌の手術では,根治性の追求と臓器血流の温存が重要である.本症例では術前画像検査で膵内を通る肝動脈走行変異を認めたが,血管塞栓術にて肝血流を改変し,根治性を損なうことなく安全に手術を施行し得た.

Endoscopy revealed stomach protru-sion into the thoracic cavity. An upper gastrointestinal series showed an organoaxial gastric volvulus. Multidetector row computed tomography demonstrated migration of the stomach and part of the omentum into the left thoracic cavity. With the preoperative appearance of a Bochdalek hernia, we performed a laparotomy for hernia repair, with consideration of adhesions between the hernia sac and organs as well as compromised respiratory function. Intraoperative findings disclosed a typical parahiatal hernia with a diaphragmatic defect, 1 cm lateral to the left crus of the diaphragm. After restoring normal anatomy by dissecting the adhesions between the sac and involved organs, the diaphragmatic defect was then sutured. The patient resumed her usual daily life, without recurrence. We report this case because parahiatal hernia is very rare (0.2-3.3％ of diaphragmatic hernias). To our knowledge, only 10 cases have been reported in the literature in Japan, and cases associated with organoaxial gastric volvulus are particularly rare.

In the surgical treatment of pancreatic cancer, margin-negative status is one of the most important determinants of survival. We conducted this study to explore surgical margin status as well as other factors affecting the survival of borderline-resectable pancreatic cancer (BRPC) patients who received neoadjuvant chemotherapy with gemcitabine and S-1.

A 40-year-old man admitted to our hospital with diarrhea underwent abdominal computed tomography (CT) which showed multiple masses in the liver and pancreatic tail. Although there were no abnormal accumulations with fluorodeoxyglucose ((18)F) positron emission tomography (FDG-PET), (68)Ga-DOTATOC-PET/CT detected obvious abnormal accumulations for the both lobes of liver and pancreatic tail tumors. The serum gastrin was markedly high, and liver tumor biopsy demonstrated the presence of malignant cells with round nuclei that were positive for gastrin and somatostatin receptor. The patient was diagnosed with pancreatic tail gastrinoma with multiple liver metastases and treated with octreotide, everolimus, and a proton pump inhibitor which functionally controlled tumor growth. This case demonstrates (68)Ga-DOTATOC-PET/CT as a useful modality for the localization, qualitative diagnosis, and treatment of gastrinoma.