Suefumi Aosasa

症例は74歳の男性．貧血精査で近医を受診，上部消化管内視鏡検査では主乳頭よりやや口側の十二指腸下行脚左壁に可動性良好な15mm大の腫瘍を，超音波内視鏡では第3層主体の均一な低エコー腫瘤を認め，生検結果から副乳頭原発の神経内分泌腫瘍（NET）G1と診断された．造影CTでは動脈早期相から濃染される13mm大の腫瘤と膵内胆管背側および膵鉤状突起左側辺縁に計4個の造影効果を示す最大径5mmの結節影を認めた．その他遠隔転移を示唆する所見はなく，亜全胃温存膵頭十二指腸切除術を施行した．病理組織学的検査では腫瘍は副乳頭に限局し，Ki-67指数1.7％，核分裂像（4/10 HPF）からNET G2と診断され，術前CTで認めた膵鉤状突起周囲の結節影は転移リンパ節であった．副乳頭部NETは腫瘍径が小さくても高率にリンパ節転移をきたすため，安易な縮小手術をすべきではないと考えられた．

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Bochdalek hernia is a congenital diaphragmatic hernia, and adult cases are rare, with a reported frequency of 0.17%-6% among all diaphragmatic hernias. A 78-year-old man was referred to our hospital with a sudden onset of whole abdominal pain after playing with a blow gun. Chest radiography and computed tomography revealed diaphragmatic hernia with the small intestine. We therefore diagnosed him with an incarcerated Bochdalek hernia associated with increased intra-abdominal pressure during use of blow gun. Laparoscopic repair was performed. The omentum, transverse colon, and small intestine were located in the left thoracic cavity, without ischemic change. After placing the herniated organs into the abdominal cavity, we performed a primary closure of the diaphragmatic defect with interrupted non-absorbable sutures. It is generally recommended that all adult Bochdalek hernia patients undergo surgical repair to prevent life-threatening complications due to incarceration. Recently, laparoscopic techniques for repair the hernia have gained popularity, especially in elective cases. In our case, we could successfully perform emergency laparoscopic repair, as it is associated with a shorter inpatient hospitalization period. An incarcerated Bochdalek hernias associated with increased intra-abdominal pressure is an uncommon clinical finding in an adult, and laparoscopic repair of an incarcerated Bochdalek hernia is safe, feasible, and an excellent option as it is minimally invasive.

Although it is well-known that in multiple endocrine neoplasia type 1 (MEN 1) disease, multiple endocrine lesions frequently occur, synchronous or metachronous neuroendocrine tumors (NETs) in non-MEN 1 patients are extremely rare. An asymptomatic 72-year-old woman with an ileal NET was referred to our hospital. Abdominal computed tomography revealed another circular tumor within the pancreatic head. She was classified as a non-MEN 1 patient. An operative procedure was performed with a preoperative diagnosis of synchronous NET, which was confirmed by pathological examination. Both morphologic and immunophenotypic findings were different between in the ileum and pancreas. Therefore, it was reasonable to consider that both tumors were primary tumors. The synchronous occurrence of these tumors is unusual, and it may be considered as a chance occurrence. We here report the first case of synchronous pancreatic NET and ileal NET in a non-MEN 1 patient.

A carcinoma displaying undifferentiated features with dense lymphoplasmacytic infiltration is defined as lymphoepithelioma-like carcinoma (LELC). Intrahepatic cholangiocarcinoma (ICC) with LELC components is rare, and most LELCs are associated with Epstein-Barr virus (EBV). We report here on a case of ICC with LELC components not associated with EBV. A 65-year-old woman was incidentally found to have a hepatic tumor in the caudate lobe. An extended right hepatectomy with lymphadenectomy was performed. Histologically, the tumor was mainly composed of large undifferentiated epithelial cells with vesicular nuclei, prominent nucleoli, indistinct cell borders, and heavy small lymphocytic infiltration, which are the characteristic features of LELC. Immunohistochemical studies revealed that the tumor cells were positive for cytokeratin 19 but were negative for glypican 3. In situ hybridization using EBV-encoded RNA was negative. Therefore, a diagnosis of ICC with LELC components not associated with EBV was made. Because there is limited information available regarding the prognosis and treatment of ICC with LELC components because of the limited number of reported cases, additional studies will be needed to clarify the clinicopathologic features of this disease.