A synchronous case of small bowel adenocarcinoma(SAB) is reported, accompanied with gastrointestinal stromal tumor(GIST),and gangliocytomain in an elderly woman with neurofibromatosis type 1 (NF-1). A 67-year-old female was hospitalized with the chief complaint of abdominal pain, the computed tomography scan indicated a large bowel mass. Multiple tumors were found in the small intestine, through which two larger tumors (7 cm and 1.5 cm) were resected. A novel germline NF1 mutation and a PMS2 mutation were identified after genetic testing, followed by the exploration of possible relationship between them in promoting tumorigenesis. Our results suggest multiple gastrointestinal tumors emerging in NF1 patients, and genetic testing can better guide postoperative treatment in a more efficient way.

We report a father and his daughter who both had multiple somatostatinomas in the duodenal bulb without a known syndrome. The father, at age 68 years, was incidentally found to harbor 4 approximately 1.5-cm well-differentiated neuroendocrine tumors in the duodenal bulb. His preoperative somatostatin level was elevated. He underwent partial duodenectomy and regional lymph node dissection; one lymph node was positive for metastasis. One year postoperatively, a recurrence was found in the surgical bed; he was treated with octreotide for 2 years, which stabilized the recurrent tumor. Ten years postoperatively, the mucosa of his remaining duodenum was normal. His daughter, at age 53 years, was found to harbor multiple small neuroendocrine tumors in the duodenal bulb. Immunostaining of available specimens showed that the neuroendocrine tumors from the father and daughter both were strongly positive for somatostatin. Micronodules of somatostatin-expressing neuroendocrine cells were found in the parts of the specimens uninvolved with the tumors. Both patients exhibited no evidence of known syndromes associated with somatostatinoma. The daughter did not harbor mutations in 93 genes commonly found in genetic tumor syndromes. The 2 cases thus suggest a novel, autosomal dominant, genetic syndrome of familial duodenal somatostatinomatosis.

An improved understanding of the CT characteristics for histologically confirmed primary intestinal tumors would be helpful for guiding prognosis and treatment plans in affected dogs. This retrospective, multi-center, analytical study aimed to evaluate the CT characteristics for the differentiation of adenocarcinoma, lymphoma, and spindle cell sarcoma (SCS) in dogs. Thirty-seven dogs who underwent contrast CT and histopathological examinations were included (adenocarcinomas, n = 11; lymphomas, n = 12; SCS, n = 14). Quantitative and qualitative CT parameters, including tumor morphology, contrast enhancement pattern, Hounsfield unit (HU) value, and presence or absence of intraabdominal lymphadenopathy, were evaluated for each included small intestine tumor CT case. Adenocarcinomas tended to show endophytic growth, intestinal obstruction, and a heterogeneous enhancement pattern. Lymphomas tended to show exophytic growth, contrast enhancement of the intestinal tumor mucosal layer, a homogeneous enhancement pattern, and the presence of lymphadenopathies in the abdominal cavity. SCSs tended to show lobulated growth, a large cystic portion within the tumor, a heterogeneous enhancement pattern, a large size with fat stranding sign, and lower HU values in postcontrast images. Cut-off values of the minimum diameter/fifth lumbar vertebral mid-body height (≥5.80; area under the curve [AUC] = 0.97, P < 0.001) and minimum HU value/HU value of the aorta (≤0.26; AUC = 0.96, P < 0.001) were derived to discriminate SCS from the two other tumor types. In conclusion, contrast CT characteristics may be useful in differentiating small intestinal adenocarcinomas, lymphomas, and SCSs in dogs.

In this case report is discussed the diagnosis of papillary carcinoma in the case of a patient in whose age the occurrence of the disease is very rare. The patient underwent a series of non-invasive and invasive examinations, the results of which eventually led to a surgical solution of the suspected lesion (Wipples procedure). Histological examination of the resection confirmed mixed type ampular adenocarcinoma. In the context of this finding, the results of the examination, their information value, interpretation and mutual interaction are discussed. In the postoperative period, the patient suffered from gastrointestinal bleeding, subileum and poor postoperative healing. After hospitalization, the patient was transferred to oncology care and indicated for genetic testing.

Small intestinal neuroendocrine tumor SI-NETs are serotonin-secreting well-differentiated neuroendocrine tumors of putative enterochromaffin (EC) cell origin. However, EC cell-derived tumorigenesis remains poorly understood. Here we examined whether the gain of Myc and the loss of RB1 and Trp53 function in EC cells result in SI-NET using tryptophan hydroxylase 1 (TPH1) Cre-ERT2-driven RB1fl Trp53fl MycLSL (RPM) mice. TPH1-Cre induced gain of Myc and loss of RB1 and Trp53 function resulted in endocrine or neuronal tumors in pancreas, lung, enteric neurons, and brain. Lineage tracing indicated that the cellular origin for these tumors was TPH1-expressing neuroendocrine, neuronal or their precursor cells in these organs. However, despite that TPH1 is most highly expressed in EC cells, we observed no incidence of EC cell tumors. Instead, the tumor of epithelial cell-origin in the intestine was exclusively non-endocrine adenocarcinoma, suggesting dedifferentiation of EC cells into intestinal stem cells (ISC). Further, ex-vivo organoid studies indicated that loss of functions of Rb1 and Trp53 accelerated dedifferentiation of EC cells that were susceptible to apoptosis with expression of activated MycT58A, suggesting that the rare dedifferentiating cells escaping cell death went on to develop adenocarcinomas. Lineage tracing demonstrated that EC cells were short-lived compared to neuroendocrine or neuronal cells in other organs. In contrast, EC cell-derived ISCs were long-lasting and actively cycling and thus susceptible to transformation. These results suggest that tissue- and cell-specific properties of EC cells affect the fate and rate of tumorigenesis induced by genetic alterations and provide important insights into EC cell-derived tumorigenesis.

Various complications of colorectal endoscopic submucosal dissection (ESD) have been reported, including bleeding, penetration, perforation, and coagulation syndrome. However, the occurrence of bowel obstruction after ESD is rare. We report a case of adhesive bowel obstruction after ESD for a laterally spreading tumor in the sigmoid colon. The 35-mm tumor was successfully removed by ESD without intraoperative complications. The patient had a fever, lower abdominal pain, and a small amount of bloody stool the day after ESD. Endoscopy revealed minor bleeding from the ESD scar, which was treated by hemostatic clips. Pathological analysis showed adenocarcinoma was exposed to the vertical margin; therefore, the resection was non-curative. At 39 days after ESD and 36 days after discharge, the patient had abdominal pain and nausea. She was readmitted with a diagnosis of adhesive bowel obstruction. Conservative treatment was ineffective; therefore, she underwent sigmoidectomy combined with partial resection of the small intestine because of small intestinal stenosis caused by inflammation. The pathological examination showed localized peritonitis around the sigmoid colon where ESD was performed. There was more fibrosis along the serous surface of the small intestine than on the sigmoid colon. We concluded that there was a micro-perforation that could not be detected by endoscopy or physical examination. This case indicates that adhesive bowel obstruction may occur as a complication of ESD.

Small bowel cancer encompasses a series of malignant lesions that may be identified throughout the small intestine (SI). The small bowel lies between the stomach and the large intestine (LI/colon) and is comprised of three different sections, the duodenum, jejunum, and ileum to the level of the ileocecal valve which provides the terminal transition point between the SI and the LI. While there are both benign and malignant lesions that can be identified throughout the SI, the overall incidence of small bowel neoplasms is extremely low when compared to lesions noted in other portions of the gastrointestinal tract. This article will focus on the overall characteristics, diagnostics, treatment and prognosis of malignant lesions. The majority of these lesions cause multiple nonspecific symptoms which very often lead to delay in diagnosis and therefore delay in early intervention with available treatment strategies. Common clinical features include abdominal pain, anorexia, gastrointestinal bleeding, and weight loss. More advanced processes can present with perforation, small bowel obstruction, or obstructive jaundice. Diagnosis can be variable based on location of the lesion under investigation and generally consists of laboratory studies, radiographic imaging and endoscopic evaluation. Malignant lesions overall include lymphomas, neuroendocrine tumors (carcinoids), adenocarcinomas, and stromal tumors.[1]

The frequency of primary small intestinal adenocarcinoma is increasing but is still low. Its frequency is approximately 3% of that of colorectal adenocarcinoma. Considering that the small intestine occupies 90% of the surface area of the gastrointestinal tract, small intestinal adenocarcinoma is very rare. The main site of small intestinal adenocarcinoma is the proximal small intestine. Based on this characteristic, dietary animal proteins/lipids and bile concentrations are implicated and reported to be involved in carcinogenesis. Since most nutrients are absorbed in the proximal small intestine, the effect of absorbable intestinal content is a suitable explanation for why small intestinal adenocarcinoma is more common in the proximal small intestine. The proportion of aerobic bacteria is high in the proximal small intestine, but the absolute number of bacteria is low. In addition, the length and density of villi are greater in the proximal small intestine. However, the involvement of villi is considered to be low because the number of small intestinal adenocarcinomas is much smaller than that of colorectal adenocarcinomas. On the other hand, the reason for the low incidence of small intestinal adenocarcinoma in the distal small intestine may be that immune organs reside there. Genetic and disease factors increase the likelihood of small intestinal adenocarcinoma. In carcinogenesis experiments in which the positions of the small and large intestines were exchanged, tumors still occurred in the large intestinal mucosa more often. In other words, the influence of the intestinal contents is small, and there is a large difference in epithelial properties between the small intestine and the large intestine. In conclusion, small intestinal adenocarcinoma is rare compared to large intestinal adenocarcinoma due to the nature of the epithelium. It is reasonable to assume that diet is a trigger for small intestinal adenocarcinoma.

Pyloric gland adenoma (PGA) in the duodenum is a rare gastric phenotype duodenal neoplasm. Although heterotopic gastric mucosa in the duodenum has been recognized as a benign lesion, it is a potential precursor of PGA and gastric phenotype adenocarcinoma. Herein, we present a case follow-up of endoscopic and histological changes in the PGA in the duodenum from low-grade to high-grade dysplasia. PGA was considered to arise from the heterotopic gastric mucosa, because the heterotopic gastric mucosa was observed in the initial examination. It is difficult to distinguish heterotopic gastric mucosa from PGAs, both endoscopically and histologically. This increase in size may be useful for their differentiation. Therefore, endoscopists should not underestimate the growth of the heterotopic gastric mucosa compared to that in the previous examination.

An ampullary carcinoma (AC) is defined as cancer that arises in the ampulla of Vater (AV). It is a tiny opening in the beginning segment of the small intestine called the duodenum. Pancreatic and bile duct fluids are pumped into the intestines via the AV. There are various factors listed as the causes of AC. A 45-year-old male presented to the emergency department with complaints of pain in the abdomen for two months, vomiting for two days, and a history of fever for four days, which was persistent in nature. He had been operated on for endoscopic retrograde cholangiopancreatography (ERCP) stenting two months back due to pain in the abdomen. The patient underwent investigations such as blood tests, histopathology, ultrasonography, abdominal contrast-enhanced computed tomography (CECT), and ampullary mass biopsy, based on which a final diagnosis was made. The patient was operated on by the Whipple surgical procedure and was later treated with antibiotics and analgesics. Surgical management for AC is a novel treatment chosen instead of chemotherapy. Although early-stage AC can be cured with radical surgery, around half of the patients experience tumor recurrence. The prognosis of our patient was good.

With the recent availability and upgrading of many emerging intestinal microbes sequencing technologies, our research on intestinal microbes is changing rapidly. A variety of investigations have found that intestinal microbes are essential for immune system regulation and energy metabolism homeostasis, which impacts many critical organs. The liver is the first organ to be traversed by the intestinal portal vein, and there is a strong bidirectional link between the liver and intestine. Many intestinal factors, such as intestinal microbes, bacterial composition, and intestinal bacterial metabolites, are deeply involved in liver homeostasis. Intestinal microbial dysbiosis and increased intestinal permeability are associated with the pathogenesis of many chronic liver diseases, such as alcoholic fatty liver disease (AFLD), non-alcoholic fatty liver disease (NAFLD), non-alcoholic steatohepatitis (NASH), chronic hepatitis B (CHB), chronic hepatitis C (CHC), autoimmune liver disease (AIH) and the development of hepatocellular carcinoma (HCC). Intestinal permeability and dysbacteriosis often lead to Lipopolysaccharide (LPS) and metabolites entering in serum. Then, Toll-like receptors activation in the liver induces the exposure of the intestine and liver to many small molecules with pro-inflammatory properties. And all of these eventually result in various liver diseases. In this paper, we have discussed the current evidence on the role of various intestinal microbes in different chronic liver diseases. As well as potential new therapeutic approaches are proposed in this review, such as antibiotics, probiotics, and prebiotics, which may have an improvement in liver diseases.

(1) Background: Small bowel adenocarcinoma (SBA) is one of the predominant primary small bowel cancers that has a dismal outcome. We aim to report 10 years of experience in SBA management at a regional cancer centre in Canada.; (2) Methods: We retrospectively analysed clinical and pathological data of patients diagnosed with an SBA between 2011 and 2021 at the Ottawa Hospital (TOH), Ottawa, Canada. We describe the clinicopathological features and outcomes, including survival. Potential prognostic factors were analysed using the Cox proportional hazard model for multivariate analysis.; (3) Results: We identified 115 patients with SBA. The duodenum was the most common SBA location representing 61% (70) of the total patients, followed by the jejunum (17%) and ileum (10%). Around 24% (27) of cases presented with bowel obstructions. The majority of patients (56%, 64) had stage IV disease on presentation. Seven patients had MSI-high tumours, while 24% (27) were MS-stable. In terms of management, 48 patients underwent curative surgical resection, 17 of whom received adjuvant chemotherapy. On the other hand, 57 patients (49.5%) with the advanced disease received palliative systemic therapy, and 18 patients (16%) had supportive care only. Over a median follow-up of 21.5 months (range 0-122), the median overall survival was 94, 61, and 34 months for stages II, III, and IV, respectively (p < 0.05). The median recurrence-free survival was 93 and 23 months for stages II and III, respectively. However, there was no statistically significant difference between TNM stages in RFS, p = 0.069. Multivariate Cox regression analysis showed only poor performance status at diagnosis as a predictor for shorter overall survival (p < 0.05). The univariate analysis didn't show any significant correlation between RFS and covariants.; (4) Conclusions: SBA remains one of the most aggressive tumours with a dismal prognosis even after surgical resection. The optimal chemotherapy regimen has not been established. Further studies are needed to explore the role of adjuvant chemotherapy for stages I-III SBA.

Small bowel and ileocecal diseases remain a diagnostic and therapeutic challenge, despite the introduction of various modalities for deep enteroscopy. Novel Motorized Spiral Enteroscopy is an innovative technology that uses an overtube with a raised spiral at the distal end to pleat the small intestine. It consumes less time and meets both the diagnostic and therapeutic needs of small bowel diseases. The objective of this article is to highlight the possibility of using NMSE as an alternative technique when a target lesion is inaccessible during conventional colonoscopy or cecal intubation cannot be achieved. We report the case of a 61-year-old man who presented with pain in the right lower abdominal segment, diarrhea, and rapid weight loss for more than 3 months. An initial ultrasound showed a suspicious liver metastasis. Computerized tomography scans showed an extensive ileocecal tumor mass with liver metastasis. The colonoscopy was unsuccessful and incomplete due to dolichocolon and intestinal tortuosity. Later, endoscopy was performed using a Novel Motorized Spiral Enteroscope in a retrograde approach, passing the scope through the anus and colon up to the ileocecal segment, where a tumor biopsy was performed and adenocarcinoma was pathohistologically confirmed.

Multiple cancer patients who achieve long-term survival are sometimes encountered. Multiple carcinogenesis may occur due to genetic or environmental characteristics. We report the case of a 74-year-old woman who was diagnosed with synchronous double carcinoma of the duodenal papilla and gall bladder without clinical symptoms, who underwent postoperative follow-up for familiar adenomatous polyposis coli with multiple colonic adenocarcinomas, ileal adenocarcinoma, left urinary tract neoplasm, and left upper lobe lung adenocarcinoma. Computed tomography also showed an intraductal papillary mucinous neoplasm of the pancreas and a lesion that was suspected to be uterine body carcinoma; however, they did not show any clear malignant behavior. No metastatic lesions of either of these biliary cancers were observed. Based on preoperative diagnostic imaging, subtotal stomach preserving pancreaticoduodenectomy with gall bladder bed liver resection with D2 lymphadenectomy was planned and R0 resection was achieved. The postoperative histological diagnosis showed early carcinoma of the duodenal papilla and gall bladder pyloric gland adenoma without node metastasis. The postoperative course was uneventful and the patient had a good prognosis without tumor recurrence or new malignant lesions for two years after the last operation, without adjuvant therapy. Six metachronous carcinomas of eight different organ neoplasms were diagnosed, which is rare. This represents the first reported case of ampullary carcinoma in a patient with sextuple cancer.

Double tract reconstruction (DTR) is the main digestive tract reconstruction method after proximal gastrectomy (PG). Single tract jejunal interposition (STJI) derived from the double tract reconstruction is also increasingly used in clinical practice. However, there is still a great controversy as to which of the two reconstruction methods can achieve better results. In this study, we systematically reviewed studies on DTR and STJI after PG and performed a meta-analysis. We searched PubMed, Embase, and Cochrane Library databases for clinical studies comparing DTR and STJI after PG to December 2021 without language restriction. Review Manager (version5.4) software was used to perform meta-analysis on operative outcomes, postoperative complications and nutritional outcomes. The protocol for this meta-analysis was registered with PROSPERO (CRD42022301455). Five randomized controlled trials involving 453 patients were included in the meta-analysis. There were no significant differences between DTR and STJI in terms of intraoperative blood loss, postoperative hospital stay, incidence of reflux esophagitis, anastomotic complications and total complications. The operation time of STJI group was longer than that of DTR group [WMD - 0.79; 95% CI (- 1.55, - 0.03)] [heterogeneity: χ2 = 4.94, df = 3 (P = 0.18); I2 = 39%, test for overall effect: Z = 2.04 (P = 0.04)]. The body weight of STJI group was significantly higher than that of DTR group at 6 months after surgery [WMD 3.90; 95% CI (0.56, 7.23)] [heterogeneity: τ2 = 7.67, χ2 = 19.76, df = 2 (P < 0.0001); I2 = 90%, test for overall effect: Z = 2.29 (P = 0.02)]. To the best of our knowledge, this is the first systematic review and meta-analysis to compare the outcomes of DTR and STJI after PG. There were no significant differences in operative outcomes and postoperative complications between DTR and STJI after PG. Although STJI prolonged the operation time compared to DTR, postoperative nutritional outcomes of patients in the STJI group was significantly better than that in the DTR group. Therefore, compared to DTR, STJI may be more suitable for the vast majority of patients undergoing PG due to its better postoperative nutritional status.

Many environmental risk factors for hepatobiliary cancers are known but whether they are associated with specific cancer types is unclear. We present here a novel approach of assessing standardized incidence ratios (SIRs) of previously diagnosed comorbidities for hepatocellular carcinoma (HCC), gallbladder cancer (GBC), cholangiocarcinoma (CCA) and ampullary cancer. The 13 comorbidities included alcohol and nonalcohol related liver disease, chronic obstructive pulmonary disease, gallstone disease, viral and other kinds of hepatitis, infection of bile ducts, hepatic and other autoimmune diseases, obesity and diabetes. Patients were identified from the Swedish Inpatient Register from 1987 to 2018, and their cancers were followed from 1997 onwards. SIRs for HCC were 80 to 100 in men and women diagnosed with hepatitis C virus and they were also >10 in patients diagnosed with hepatitis B virus, other kind of hepatitis, hepatic autoimmune disease and nonalcohol related liver disease. Many of these risks, as well as alcohol related liver disease, were either specific to HCC or were shared with intrahepatic CCA. For GBC, CCA and ampullary cancer infection of bile ducts was the main risk factor. Gallstone disease, nonhepatic autoimmune diseases and diabetes were associated with all hepatobiliary cancers. The limitations of the study include inability to cover some rare risk factors and limited follow-up time. Many of the considered comorbidities are characterized by chronic inflammation and/or overt immune disturbance in autoimmune diseases. The results suggest that local chronic inflammation and a related immune disturbance is the carcinogenic trigger for all these cancers.