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Background The clinical characteristics of IgG4-related sclerosing cholangitis (IgG4-SC) especially without autoimmune pancreatitis (AIP) have not been investigated in a large cohort. Aims To clarify the clinical characteristics of IgG4-SC and IgG4-SC without AIP. Methods We retrospectively reviewed imaging, serology, other organ involvement (OOI) and histology of 872 patients with IgG4-SC who participated in a Japanese nationwide survey in 2019, and compared these items between IgG4-SC with and without AIP. Results AIP was present in 83.7% (730/872) of IgG4-SC. In IgG4-SC, bile duct wall thickening was observed on ultrasound (528/650; 81.2%), computed tomography (375/525; 71.4%) and magnetic resonance imaging or cholangiopancreatography (290/440; 65.9%). An elevated serum IgG4 level (≥ 135 mg/dL) was found in 88.0% (322/366). IgG4-related OOI other than AIP was observed in 25.2% (211/836). The proportion of females was significantly higher in IgG4-SC without AIP (28.9% vs. 20.1%; p = 0.025). Hilar stricture was the most common cholangiographic type in IgG4-SC without AIP (39/107; 36.4%).There were no significant differences between IgG4-SC with and without AIP in the rates of bile duct wall thickening, elevated serum IgG4 level, or IgG4-related OOI. Conclusions The clinical characteristics of IgG4-SC was similar between IgG4-SC with and without AIP in a large cohort.

The aim of this study was to elucidate the clinicopathological features and prognosis of mucinous cystic neoplasms (MCNs).We performed a multi-institutional, retrospective study on a collected series of patients with MCN pathologically defined by ovarian-type stroma. Clinicopathological features and prognosis were investigated.Mucinous cystic neoplasm was confirmed in 156 cases, including 129 adenomas (82.7%) and 21 noninvasive (13.4%) and 6 invasive carcinomas (3.9%). Patients with MCN were exclusively women (98.1%) with the mean age of 48.1 years. All but 1 MCN were in the pancreatic body/tail region with a mean size of 65.3 mm. Communication between the cyst and the pancreatic duct was found in 18.1%. The 3-, 5-, and 10-year survival rates were 97.6%, 96.6%, and 96.6%, respectively. A significant difference in the survival rates was observed between adenomas and carcinomas and between minimally invasive carcinomas and invasive carcinomas. Cyst diameter and presence of mural nodule were predictive of malignant MCN.Mucinous cystic neoplasm is a rare but distinctive pancreatic cystic neoplasm with a favorable overall prognosis. All MCNs should be resected to prevent malignant changes but can be observed for an appropriate time when the lesion is small without the presence of mural nodules.

症例は58歳の男性で，検診で直腸Rbに粘膜下腫瘍を指摘された．直腸カルチノイドの診断でendoscopic submucosal dissection（ESD）を行った．病理組織学的検索では腫瘍径は4 mmで，垂直・水平断端はともに陰性であったが微小脈管侵襲が認められた．十分なインフォームドコンセントの後，追加手術を行ったところ，251番リンパ節に転移を認めた．直腸カルチノイドでは，径10 mm以上，深達度mp以深，中心陥凹，表面の凹凸不整像，脈管侵襲陽性などの所見があれば，追加切除や厳重な経過観察が必要とされている．腫瘍径5 mm以下では，リンパ節転移のない症例がほとんどであること，手術によりquality of lifeが損なわれる可能性があることから，追加切除の是非についてはいまだにコンセンサスが得られていない．しかし，本症例のごとく微小病変であっても脈管侵襲陽性例は追加手術，あるいは厳重な経過観察が必要と考えられた．

膵嚢胞性病変の臨床病理学的特徴と超音波診断を中心とした画像診断の要点について概説する．臨床と病理の両面からの研究の進展に伴い膵嚢胞性病変に関する疾患概念も統一化されてきており必ずしも理解困難な病態ではなくなってきた．腫瘍性嚢胞である漿液性嚢胞腫瘍，粘液性嚢胞腫瘍，膵管内乳頭粘液性腫瘍などは発生部位，性や年齢，肉眼形態や組織像にそれぞれ特徴のある腫瘍であり，その臨床病理学的特徴を理解して画像診断に役立てる必要がある．二次性嚢胞の中では膵管癌の発見契機として重要な意味を有する貯留嚢胞や，本来充実性腫瘍である内分泌腫瘍やsolid-pseudopapillary tumorなどに見られる嚢胞状壊死などを理解しておく必要がある．先天性嚢胞としては遭遇する機会は少ないがvon Hippel-Lindau disease，polycystic disease，リンパ上皮性嚢胞，類表皮嚢胞などの病理学的知識が画像診断をしていく上では必要である．画像診断は臨床病理学的特徴の理解の上でなされるべきものであり，切除標本の肉眼所見と画像所見の対比を繰り返していくことにより診断の精度向上が期待出来るし，正確な診断が可能になれば不必要な手術を回避出来ることにも繋がる．

The clinical indications for hepatectomy and extrahepatic bile duct resection (EBDR) for pT2 gallbladder carcinoma (GBC) remains controversial. The aim of this study is to elucidate the therapeutic effect of hepatectomy and extrahepatic bile duct resection on the surgical treatment of pT2 GBC. Ninety-four patients with pT2 GBC who underwent a potentially curative resection were retrospectively analyzed regarding their pathological findings, surgical procedures, and survival. The most powerful predicting factor for the survival is the nodal status. The 5-year survival rate was 87.1% for the pN0 patients and 55.7% for the pN1 patients. With respect to surgical procedures, the 5-year survival rate was 73.3% for the 51 patients with hepatectomy, and 87.2% for the 43 patients without hepatectomy. In addition, the 5-year survival rate was 66.7% for the 11 patients with EBDR, and 81.1% for the 83 patients without EBDR. When restricting the patients to those with pN1 disease, the 5-year survival rate of the patients who received these procedures did not surpass that of the patients who did not. There is no positive therapeutic effect besides providing surgical margins in hepatectomy and EBDR in the surgical treatment of pT2 GBC whereas lymph node dissection is most effective procedure for improving survival. Provided that the negative surgical margins are secured, a hepatectomy and an EBDR can therefore be withheld in the surgical treatment for the pT2 GBC.

症例は24 歳の女性で, 職場の健康診断で施行された腹部超音波検査にて6cm 大の肝右葉の腫瘤性病変を指摘され当院に紹介された. CT にて腫瘤は中心性瘢痕の部分を除いて強い早期濃染を示し, Superparamagnetic iron oxide (SPIO)-enhanced MRI では取込みを認めず著明な高信号を呈しfibrolamellar carcinoma (FLC) が疑われた. 肝右葉切除が行われ, 病理組織学的に強い好酸性を示す大型の腫瘍細胞が層状の膠原線維により区画されておりFLCと診断された. FLC は通常型肝細胞癌とは異なる臨床病理学的特徴を有し, 肝硬変のない若年者に好発する. しばしばリンパ節転移を伴う事から治療にあたっては肝切除に加えリンパ節郭清を行う必要がある. FLC に関する本邦報告例の集計52例を含め文献的考察を加えて報告する.

Intraductal tubulopapillary neoplasm (ITPN) is a rare pancreatic tumor belonging to a newly recognized entity that is coined in the 2010 WHO classification. We present a case of ITPN-associated microinvasive adenocarcinoma that developed in an asymptomatic 78-year-old patient. The tumor demonstrated all the clinical, radiological, and pathological characteristics of ITPN, but it differs from other reported cases of ITPN in molecular analysis, which revealed a somatic mutation in BRAF (c.1799T>A; p.V600E) but no mutation in PIK3CA. Post-operative recurrence was discovered 34 months after tumor resection with negative margins and a 6-month course of adjuvant chemotherapy. To the best of our knowledge, this is the first case of ITPN with BRAF mutation. This case suggests that an activation of RAS-mitogen-activated protein kinase signaling pathway may play a role in development of some of ITPNs. A possible mechanism of tumor recurrence in ITPN is also discussed. Further case series with molecular study are awaited to delineate the clinicopathological and molecular characteristics of ITPN.

For superficial esophageal cancer, conventional endoscopic mucosal resection with cap (EMRC) has been widely prevailed for reliable and safety treatment1. Recent innovations of diagnostic endoscopy including high-resolution endoscopy, narrow band imaging (NBI), and magnified endoscopy allow detect and identify more small lesions of the GI tract, and endoscopist are forced to develop their skills for endocopic treatment. Recently, endoscopic submucosal dissection (ESD) has been developed for en bloc resection of larger superficial tumor in the GI tract than conventional EMR. In this decade, ESD has been widely accepted as a more reliable therapeutic procedure than EMR in Japan, and various devices are developed. Of the esophageal cancers, squamous cell carcinoma is the most common carcinoma in Japan, and iodine staining endoscopy has been widely used to detect esophageal SCC. Conventional EMRC has been performed for these 10 years, however, since conventional EMRC were carried out with small size cap, 12mm in diameter, there has been the risk to be piecemeal and residual lesions. Recently, newly classification of intrapapillary capillary loop pattern (IPCL) has developed for diagnose the early esophageal neoplasm, and because of these diagnostic innovation, therefore, many endoscopist are forced to remove larger en bloc resection for reliable ESD as well as gastric or colorectal lesions. Therefore, in Japan, ESD for large superficial esophageal neoplasm has been applied; however, esophageal ESD is considered as more difficult and challenging than gastric ESD, and only few studies have elucidated the technical feasibility of ESD in the esophagus. Needle knife has been used for early gastric and colorectal ESD, and it has been considered causing high complications for esophageal ESD because of its sharpness. For this reason, using other knives, some clinical case series are reported for esophageal ESD, and the safety of esophageal ESD using needle knife is still unclear. In this study, we conducted consecutive esophageal ESD with needle knife and compared EMR to evaluate safety and reliability of needle knife as esophageal ESD device.

The patient was a woman, aged 69, diagnosed with acute pancreatitis by a local physician; simultaneously, with US, a low-echo tumor was indicated in the pancreas' uncinate process. Diagnosis was made of acute pancreatitis resulting from a pancreatic IPMN, and the patient was referred. Ultrasound showed hypoechoic tumor images accompanied by posterior echo enhancement. With radiography-CT, from the pancreas parenchymal phase, the peripheral portion was densely stained, while internally, images showed densely stained dendriforms towards the equilibrium phase. With MRI T1-weighted images, there was appearance at low intensity, and with T2-weighted images, there was appearance at high intensity; with MRCP, there was depiction at relatively high intensity. In the final pathological diagnosis, there was prominent formation of mucinous nodules, and mucinous carcinoma including large quantity of mucous.

症例は78歳女性．閉塞性黄疸及び十二指腸狭窄による嘔吐を主訴に来院．腹部造影CTでは，十二指腸と膵頭部の間に帯状の低吸収域を認め，十二指腸に接する膵内に10mm大の腫瘤性病変が認められた．十二指腸球部からのEUS走査では十二指腸に接する膵辺縁に低エコー腫瘤とこれに連続して，groove領域，十二指腸壁に不整に拡がる境界不明瞭な低エコー像を認めた．また十二指腸狭窄部からの内視鏡的生検にて腺癌が検出された．以上よりgroove領域に浸潤した膵癌と診断し亜全胃温存膵頭十二指腸切除術を施行した．病理組織学的には十二指腸に接する膵領域より発生しgroove領域及び十二指腸へ浸潤性増殖を示す25×20mmの膵癌（T3N0M0 pStageIII）であった．これまでgroove pancreatic carcinomaの報告においてEUS所見に言及した例は少なく貴重な症例と考え報告する．