The proportion of male cases was higher than that in previous studies, and there were statistically significant differences in the onset age and tumor diameter between male and female patients. Therefore, women seemed to have an early occurrence of SPNs, suggesting a difference in the developmental stage between men and women. Images and pathological findings of SPNs varied according to tumor size. Our findings indicated that SPN patients have excellent survival after margin-negative surgical resection.

The biopsy‐based diagnosis of autoimmune pancreatitis (AIP) is difficult but is becoming imperative for pathologists due to the increased amount of endoscopic ultrasound‐guided biopsy tissue. To cope with this challenge, we propose guidance for the biopsy diagnosis of type 1 AIP. This guidance is for pathologists and comprises three main parts. The first part includes basic issues on tissue acquisition, staining, and final diagnosis, and is intended for gastroenterologists as well. The second part is a practical guide for diagnosing type 1 AIP based on the AIP clinical diagnostic criteria 2018. Inconsistent histological findings, tips for evaluating IgG4 immunostaining and key histological features including the ductal lesion and others are explained. Storiform fibrosis and obliterative phlebitis are diagnostic hallmarks but are sometimes equivocal. Storiform fibrosis is defined as spindle‐shaped cells, inflammatory cells and fine collagen fibers forming a flowing arrangement. Obliterative phlebitis is defined as fibrous venous obliteration with inflammatory cells. Examples of each are provided. The third part describes the differentiation of AIP from pancreatic ductal adenocarcinoma (PDAC), focusing on histological features of acinar‐ductal metaplasia in AIP, which is an important mimicker of PDAC. This guidance will help standardize pathology reports of pancreatic biopsies for diagnosing type 1 AIP.

Background Current nodal staging (N-staging) of am-pullary carcinoma in the TNM staging system distinguishes between node-negative (N0) and node-positive (N1) disease but does not consider the metastatic lymph node (LN) number. Methods Overall, 313 patients who underwent pancreatoduodenectomy for ampullary adenocarcinoma were categorized as N0, N1 (1–2 metastatic LNs), or N2 (≥3 metastatic LNs), as proposed by Kang et al. Clinico-pathological features and overall survival (OS) of the three groups were compared. Results The median number of LNs examined was 11, and LN metastasis was present in 142 cases (45 %). When LN-positive cases were re-classified according to the proposed staging system, 82 were N1 (26 %) and 60 were N2 (19 %). There was a significant correlation between proposed N-stage and lymphovascular invasion, perineural invasion, increased tumor size (each p < 0.001), and surgical margin positivity (p = 0.001). The median OS in LN-negative cases was significantly longer than that in LN-positive cases (107.5 vs. 32 months; p < 0.001). Patients with N1 and N2 disease had median survivals of 40 and 24.5 months, respectively (p < 0.0001). In addition, 1-, 3-, and 5-year survivals were 88, 76, 62 %, respectively, for N0; 90, 55, 31.5 %, respectively, for N1; and 68, 34, 30 %, respectively for N2 (p < 0.001). Even with multivariate modeling, the association between higher proposed N stage and shorter survival persisted (hazard ratio 1.6 for N1 and 1.9 for N2; p = 0.018). Conclusions Classification of nodal status in ampullary carcinomas based on the number of metastatic LNs has a significant prognostic value. A revised N-staging classification system should be incorporated into the TNM staging of ampullary cancers.

Intracystic papillary neoplasm (ICPN) of gallbladder is a comparatively new concept and is described as pre-malignant lesions in Nakanuma et al. (In: Bosman et al. (eds) WHO Classification of Tumours of the Digestive System, World Health Organization of Tumours, IARC, Lyon, 2010). ICPN with high-grade intraepithelial neoplasia is understood to include intraepithelial carcinoma or noninvasive carcinoma. And lesions with invasive cancer components are classified as ICPN with an associated invasive carcinoma [1]. According to Adsay et al., more than half of patients diagnosed with ICPN have invasive cancer components (Adsay et al., Am J Surg Pathol 36:1279-1301, 2012).Polypoid masses in the gallbladder including benign, malignant, and non-neoplastic lesions have been called gallbladder polyps, and ICPN is also a polypoid lesion in the gallbladder. However, it is difficult to differentiate between them. In the literature, it is said that the possibility of malignancy is high in lesions exceeding 1 cm (Terzi et al., Surgery 127:622-627, 2000). And there are few reports on characteristic imaging findings of ICPN.We have experienced three cases (two females and one male) of ICPN and report our imaging findings. Contrast-enhanced computed tomography revealed large papillary polypoid lesions approximately 2-4 cm in size in the gallbladder. Findings suggestive of deformation of the gallbladder wall and extrinsic progression were absent in all cases. T2-weighted magnetic resonance imaging revealed intense signals and diffusion-weighted imaging showed high intensity. Expanding of the gallbladder was seen in case 1, and a tumor stalk-like appearance was seen in the papillary mass in cases 2 and 3. Surgery was performed in all three cases and ICPN was diagnosed pathologically. The cancer was localized to the mucosa, with no infiltration of surrounding tissue in all three cases.

Solid pseudopapillary neoplasm (SPN) of the pancreas has generally been regarded as a low-grade malignant tumour that preferentially develops in young women and can have a good prognosis with surgery. Among the few patients who have died from metastatic SPN are mostly those whose tumours harbour an undifferentiated component characterized by diffuse sheets of cells with increased nuclear atypia and proliferative index. We herein report a case of an aggressive, fatal, solid pseudopapillary neoplasm (SPN) of the pancreas in a 63-year-old woman complaining of epigastric pain. Despite having undergone surgical resection for a 10 cm pancreatic mass and multiple liver metastases, the patient later died due to uncontrollable metastases 36 months after the initial surgery. Histological examination showed that the tumour displayed unusual high-grade malignant features, showing diffuse sheets of cells with increased nuclear atypia and proliferative activity, along with conventional low-grade malignant features. The tumour was subsequently recognized as an SPN with foci of high-grade malignant transformation according to the 2010 World Health Organization classification. Immunohistochemical studies revealed that p16-RB pathway alterations contributed to the high-grade malignant transformation. The present case report suggests the necessity for developing diagnostic and treatment methods targeting p16 and RB for high-grade variants of SPN.