Small Bowel Tumors Research Articles

Myosin Vb as a tumor suppressor gene in intestinal cancer.

Colorectal cancer causes >900,000 deaths every year and a deeper understanding of the molecular mechanisms underlying this disease will contribute to improve its clinical management and survival. Myosin Vb (MYO5B) regulates intracellular vesicle trafficking, and inactivation of this myosin disrupts the polarization and differentiation of intestinal epithelial cells causing microvillous inclusion disease (MVID), a rare congenital disorder characterized by intractable life-threatening diarrhea. Here, we show that the loss Myosin Vb interfered with the differentiation/polarization of colorectal cancer cells. Although modulation of Myosin Vb expression did not affect the proliferation of colon cancer cells, MYO5B inactivation increased their migration, invasion, and metastatic potential. Moreover, Myo5b inactivation in an intestine-specific knockout mouse model caused a >15-fold increase in the number of azoxymethane-initiated small intestinal tumors. Consistently, reduced expression of Myosin Vb in a cohort of 155 primary colorectal tumors was associated with shorter patient survival. In conclusion, we show here that loss of Myosin Vb reduces polarization/differentiation of colon cancer cells while enhancing their metastatic potential, demonstrating a tumor suppressor function for this myosin. Moreover, reduced expression of Myosin Vb in primary tumors identifies a subset of poor prognosis colorectal cancer patients that could benefit from more aggressive therapeutic regimens.

Small and large intestinal cancer in patients with Crohn's disease studied by surgeons.

The current study summarized the clinical course and treatment outcomes of intestinal cancer in CD seen in our department and explored the steps to take in the future. Subjects were patients who had been diagnosed with CD at our hospital and who underwent surgery in our department from 1985 to 2020. Thirty-one patients had CD and intestinal cancer, including 6 with cancer of the small intestine and 25 with cancer of the large intestine. In all six patients with cancer of the small intestine, the site where cancer or a tumor developed was at or near the site of the anastomosis made at a previous surgery. Of the 25 patients with cancer of the large intestine, 22 developed cancer in the rectum or anal region. Many of the patients with cancer of the small intestine had previously undergone surgery, and the cancer developed at or near the site of the anastomosis. In patients who have previously undergone resection of the small intestine, the small intestine needs to be examined regularly. Cancer of the large intestine often developed in the rectum or anal region of our patients, so a detailed examination of the same site needs to be performed.

The state of cancer care in Russia: cancer of the small intestine (C17). Survival of patients, median survival, survival of patients according to tumor localization, histological pattern and stage of the disease (Population study at the federal district level). Part II

Background. Small intestine cancer is extremely rare cancer worldwide with an incidence of less than 1.0 per 100,000 population. In 2020, 1,711 cases of small intestine cancer were recorded in Russia, including 781 cases among the male population, and 930 among the female population. It should be noted that in Russia, despite the decrease in the total number of new cancer cases associated with the coronavirus epidemic, the number of patients with small intestine cancer increased by 4.14 % from 2019 to 2020. In the Northwestern Federal district of the Russian Federation, 216 patients with newly diagnosed small intestine cancer were registered in 2020, (29 more patients than in the previous year). The purpose of the study was to analyze the efficiency of small intestine cancer care provision based on of the database of the population cancer Registry (db pcr) of the Northwestern Federal district of the Russian Federation, with an assessment of one- and five-year survival rates. Material and methods. To calculate the survival rates for patients with small intestine cancer, we selected 1922 patients from the database of the pcr of the Northwestern Federal district of the Russian Federation, for the period from 2000 to 2018. Standard methods for calculating survival rates according to the eurocare program were used. Results. The one-year survival rate of small intestine cancer patients increased from 50.0 % to 61.1 % from 2000 to 2018, and the five-year survival rate remained almost unchanged. The relative one-year survival rate of patients was 2.0 % higher. The five-year survival rate for five-year cohorts indicates defects in staging of small intestine cancer rather than an improvement in patient care; although this rate increased from 31.5 to 32.9 %. The histological detail of small intestine cancer according to the icd-10 was investigated. Conclusion. The study confirmed the high mortality rates and modest survival benefits in survival rates in patients with small intestine cancer. Defects in the distribution of patients by disease stages were revealed. The most common histological types of small intestine cancer with calculations of patient survival were identified.

Concurrent loss of MLH1, PMS2 and MSH6 immunoexpression in digestive system cancers indicating a widespread dysregulation in DNA repair processes.

Immunohistochemical analysis of mismatch repair (MMR) protein expression is widely used to identify tumors with a deficient MMR (dMMR). MMR proteins (MLH1/PMS2 and MSH2/MSH6) work as functional heterodimers, which usually leads to the loss of expression in only one functional MMR heterodimer. Recently, there have been studies showing the simultaneous loss of immunoexpression in proteins of both heterodimers. Yet, this phenomenon has been rarely investigated. In this study, we retrospectively considered cases of different digestive system cancers (gastric cancer, ampullary cancer, small bowel cancer, colorectal cancer), which were immunohistochemically tested for dMMR within a 4-year period at our university hospital (n=352). Of the 103 cases showing dMMR, 5 cases (1.4% of all, 5.1% of dMMR cases) showed a concurrent loss of MLH1, PMS2 and MSH6 immunoexpression, whereas in the other 98 dMMR cases only one MMR heterodimer was affected. MLH1-/PMS2-/MSH6- cancer cases almost arose throughout the entire digestive tract: from the gastric antrum to the left colic flexur. To provide a comprehensive molecular characterization of this MLH1-/PMS2-/MSH6- immunophenotype, tumors were analyzed for microsatellite instability, MLH1 promotor hypermethylation and BRAF exon 15 status. Furthermore, we performed next-generation sequencing focusing on genes related to DNA repair. Here, we could detect pathogenic germline variants as well as multiple sporadic mutations in different genes involved in MMR and homologous recombination repair (HRR) respectively. The affected MMR/HRR-related genes were: ATM, BARD1, BRCA1, CDK12, CHEK1, CHEK2, FANCA, MLH1, MSH6, PALB2, TP53. Considering the biologic function of HRR/MMR proteins as potential drug targets and the low frequency of most of these mutations in digestive system cancers in general, their common occurrence in our MLH1-/PMS2-/MSH6- cases seems to be even more noteworthy, highlighting the need for recognition, awareness and further investigation of this unusual IHC staining pattern.

Predicting Total Hospitalizations and Emergency Visits in Radiotherapy Patients: A Claims-Based Model

<h3>Purpose/Objective(s)</h3> This study aimed to develop a claims-based prediction model to evaluate the risk of total hospitalizations (TH) in patients undergoing radiation therapy (RT). <h3>Materials/Methods</h3> This retrospective cohort study included lung, head and neck, rectal, esophageal, gastrointestinal (GI)-other, anal, pancreatic, colorectal and small bowel cancer patients who underwent RT and were insured by a large health plan (fully insured (FI) and Medicare (M)) in the United States from Jan 1, 2018, to Sep 30, 2021. We defined TH as either an emergency department (ED) or inpatient (IP) visit within 30 days of RT. Multivariate logistic regression was used to predict TH, overall and by cancer type, while adjusting for confounders. The model was trained on 66% of the data; performance was tested on 33% to determine accuracy, F1-score, precision and recall. <h3>Results</h3> 4,996 of 15,874 RT patients (31.5%) had a TH. The most common primary diagnoses for TH were sepsis, pneumonia and fluid and electrolyte disorder. In unadjusted analysis, type of insurer (FI vs. M), concurrent chemotherapy, hydration, malnutrition, pharmacy visits and past hospitalizations (defined as an ED or IP visit 30 days before the first RT visit) were significantly different (p<.001) between TH and non-TH patients. After adjusting for variables, our regression model's performance against the test set had an accuracy of 76%, an F-1 score of 55%, precision of 43% and recall of 74%. Cancer types with the highest F-1 scores were esophageal, head and neck, GI-other and anal cancer. Past hospitalizations (odds ratio (OR) = 9.0, 95% confidence interval (CI):7.96-10.17), chemotherapy (OR = 1.92, 95% CI:1.73-2.13), hydration (OR = 1.46; 95% CI:1.30-1.63), malnutrition (OR = 1.41; 95% CI:1.17-1.69) and Medicare insurance (OR =1.20; 95% CI:1.00-1.44) (Table 1) increased the risk of TH. Having a dietician visit (OR = 0.63; 95% CI:0.13-3.06) reduced the risk of TH, but not significantly. <h3>Conclusion</h3> Herein, we describe a claims-based predictive model for TH in patients undergoing RT. The model demonstrates practical utility as it predicts TH events with good precision and recall. Although occurring in a small percentage of patients, past hospitalizations had the highest OR of TH. Using this claims-based model to identify at-risk patients and including high-touch interventions such as nutritional support may mitigate TH.

Clinical Characteristics and Outcomes of Young Adult Patients with Small Intestinal Neuroendocrine Tumors Differ from Those of Older Adults

INTRODUCTION: Small intestinal neuroendocrine tumors (siNETs) are most commonly diagnosed in the sixth and seventh decades of life. Little is known about the characteristics and outcomes of patients diagnosed at younger ages. METHODS: The National Cancer Database was queried for patients diagnosed with siNETs from 2004 to 2018. Young adults (18 to 39 years) and older adults (40+ years) were compared using Wilcoxon and Chi-square tests and Kaplan-Meier estimates. RESULTS: A total of 26,902 adults with siNETs were identified. Median age was 62 years (interquartile range [IQR] 53 to 71). Young adults (1,130; 4.2%) were more likely to be of non-white race (21.2% vs 15.0%, p < 0.001) and Hispanic ethnicity (8.4% vs 4.5%, p < 0.001). Of young adults, 51.9% had stage 3 disease and 24.7% had stage 4 disease, compared with 54.4% and 29.2%, respectively, of older adults (p < 0.001); 91.5% of young adults and 91.6% of older adults underwent resection (p = 0.89). Young adults were more likely to have negative margins (90.6% vs 84.7%, p < 0.001) and more regional nodes examined (median 11.0 (IQR 1.0 to 20.0) vs 9.0 (IQR 1.0 to 16.0), p < 0.001). Ninety-day mortality was 0.9% for young adults and 3.5% for older adults (p < 0.001). Across all stages, young adults had more favorable 5-year survival (Table). Table. - Clinical Characteristics and Survival Rates of Young vs Older Adults with siNETs Variable Young adults (n = 1,130) Older adults (n = 25,772) p Value Grade 1 76.9% 79.9% 0.046 Grade 2 20.7% 18.1% Grade 3 2.4% 2.0% Stage 1-2 23.4% 16.4% <0.001 Stage 3 51.9% 54.4% Stage 4 24.7% 29.2% 5-year survival: stage 1–2 93.2% (95% CI 88.0–98.5) 80.3% (95% CI 78.6–82.0) <0.001 5-year survival: stage 3 97.0% (95% CI 94.7–99.1) 86.6% (95% CI 85.8–87.5) 5-year survival: stage 4 84.1% (95% CI 78.3–90.3) 71.3% (95% CI 69.8–72.9) CONCLUSION: Young adults with siNETs are demographically different from older patients, with a higher percentage of non-white and Hispanic patients. Young adults present with less advanced disease and are more likely to undergo resection with negative margins and have more regional nodes examined. Young adults have more favorable short- and long-term survival regardless of stage, which warrants separate evaluation for prognostication.

Current standards in the surgical treatment of pancreatic and small intestinal neuroendocrine tumors

Current standards in the surgical treatment of pancreatic and small intestinal neuroendocrine tumors

Small Bowel Adenocarcinoma: 10-Year Experience in a Cancer Center-The Ottawa Hospital (TOH).

(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.

S3368 Bleeding From a Rare Omental Gastrointestinal Stromal Tumor Found on Capsule Endoscopy

Introduction: Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors involving the gastrointestinal tract compromising approximately 1 -2 % of gastrointestinal cancers. They can arise from the bowel wall as a subepithelial neoplasm in the stomach and small intestine, and less than 5% of them arise as extragastrointestinal tumors in the retroperitoneum, mesentery and omentum. They occur in older adults aged 65 to 69 years old, and vary in their presentation depending on their location. We present a unusual case of small bowel gastrointestinal bleeding from a GIST adherent to the omentum. Case Description/Methods: 58 year old male with a history of diabetes mellitus type II, hypertension, recent prolonged hospitalization for disseminated nocardia infection on long term antibiotics and a Decadron taper presented with several days of epigastric pain, nausea and non-bloody diarrhea. He denied any signs of gastrointestinal bleeding such as melena or hematochezia. His labs were notable for a hemoglobin of 4.2 g/dL with a mean corpuscular volume of 78.5 fL that was previously noted to be within normal limits. Computed tomography of the abdomen and pelvis with contrast showed a 4 cm circumscribed, hyperdense enhancing exophytic solid mass arising from the small bowel in the left lower quadrant with adjacent hyperenhancement. The patient subsequently underwent capsule endoscopy which showed fresh blood in the mid jejunum with more active bleeding surrounding a possible small bowel tumor. Due to ongoing bleeding and anemia, the patient underwent laparoscopic small bowel resection and lymphadenectomy of a small bowel tumor adherent to the omentum with about 10 cm of proximal and distal margins. Pathology was consistent with a low-grade gastrointestinal stromal tumor approximately 3.0 x 3.0 x 2.3 cms with tumor free margins and no evidence of lymphovascular spread. Thereafter, the patient remained hemodynamically stable with improvement of his blood counts and was discharged home. (Figure) Discussion: While GISTS are common findings in the gastrointestinal tract, only a small percentage of them can cause gastrointestinal bleeding needing surgical intervention. Surgical resection remains the mainstay of treatment for localized, non-metastatic GISTs that present with bleeding as it can both stop the bleeding and resect the lesion. However, other modalities of treatment include endoscopic intervention depending on the location or transcatheter arterial embolization.Figure 1.: Top: CT Abdomen and Pelvis with contrast demonstrating a 4 cm circumscribed hyperdense enhancing exophytic solid mass arising from the small bowel in the left lower quadrant. Bottom: Capsule endoscopy showing a small bowel tumor.

S3496 You Shall Not Pass: Rare Duodenal Bulb Adenocarcinoma Causing Gastric Outlet Obstruction

Introduction: Duodenal carcinoma accounts for only 1-2% of diagnosed gastrointestinal cancers in the United States. Due to its insidious onset, advanced disease is often established at the time of diagnosis resulting in poor outcomes for patients. Although duodenal adenocarcinomas have been found to comprise around half of all small bowel adenocarcinomas, disease arising from within the duodenal bulb in the D1 segment is a vanishingly rare phenomenon. Case Description/Methods: A 52-year old female with a past medical history of morbid obesity with placement of laparoscopic adjustable gastric band 14 years prior presented with frequent emesis, inability to tolerate oral diet and a 22kg weight loss. Recent esophagogastroduodenoscopy during a previous admission revealed a cratered ulcer in the duodenal bulb. An upper GI series was obtained, showing a 2cm mass in the bulb apex which was confirmed by computed tomography. A repeat esophagogastroduodenoscopy was performed which identified a firm and friable ulcer with contact bleeding with complete obstruction of the duodenal bulb. Subsequent histopathology confirmed the presence of invasive, moderately differentiated adenocarcinoma. (Figure) Discussion: Small bowel tumors comprise about 2% of all GI tumors. Adenocarcinoma accounts for about 30% of all small bowel tumors. The duodenum comprises 53% of all small bowel neoplasms. Of duodenal tumors, about 57% occur in the D2 segment of the duodenum. One 2014 meta-analysis of small bowel cancers between 1990-2012 from an institutional registry (excluding ampullary cancers arising from the bile duct mucosa) identified only 30 cases of primary duodenal adenocarcinoma with none arising from D1. A previous 1991 literature review cites 47 cases of primary duodenal adenocarcinoma arising from D1, however, anatomic distinction between the duodenal bulb and the distal D1 segments was not made. Although small bowel tumors are the third leading cause of gastric outlet obstruction, lesions are typically asymptomatic. In a study of 217 primary duodenal adenocarcinoma, the median survival was 20 months with 35% presenting with metastatic disease (39% present with stage III disease). Although it is difficult to distinguish the prevalence of duodenal bulb neoplasms, it has been clearly demonstrated that neoplasms arising from the D1 segment of the duodenum are extremely rare. By presenting this case, we hope to increase the index of suspicion for small bowel neoplasms as part of the differential for gastric outlet obstruction.Figure 1.: CT demonstration of duodenal bulb mass causing gastric outlet obstruction; Figure 2: Endoscopic image of duodenal bulb tumor.

S953 The Influence of Inflammatory Bowel Disease on Upper Gastrointestinal Malignancy

Introduction: It is well known that Inflammatory Bowel Disease (IBD) increases the risk of developing Colorectal cancer. However, older works from the 1990’s examined how Ulcerative Colitis and Crohn’s Disease separately influence gastric and small bowel cancers as well. The aim of this nationwide investigation is to explore the current relationship between upper gastrointestinal malignancies and both Crohn’s Disease and Ulcerative Colitis. Methods: The National Inpatient Sample was queried for patients with a primary diagnosis of upper gastrointestinal malignancy and a secondary diagnosis of either Ulcerative Colitis or Crohn’s Disease in 2014. Upper gastrointestinal malignancies were defined as esophageal cancer, gastric cancer, small bowel malignancy, or cholangiocarcinoma. The primary outcome is the relationship between Ulcerative Colitis, Crohn’s Disease, and various forms of upper gastrointestinal cancer. Multivariate logistic regression was conducted using Stata v.13. Results: In 2014, there were 175 patients with IBD, 105 of which had Crohn’s Disease. Regression analyses demonstrated an increased association with upper gastrointestinal malignancies in both Crohn’s Disease (aOR 2.08, p< 0.00) and Ulcerative Colitis (aOR 1.91, p=0.05). When stratified, there was a greater association between cholangiocarcinoma and Ulcerative Colitis (aOR 4.30, p< 0.00), as compared to Crohn’s Disease (aOR 2.14, p=0.04). However, there was a strong relationship between small bowel cancer and Crohn’s disease (aOR 11.88, p< 0.00), but no significant association with Ulcerative Colitis. With regard to esophageal and gastric malignancies, no significant relationship with either form of IBD was established. Conclusion: There have been limited studies evaluating the relationship between upper gastrointestinal malignancies and either Crohn’s Disease or Ulcerative Colitis. This study demonstrated that both conditions have an increased association with development of cholangiocarcinoma. Ulcerative Colitis poses a two-fold greater risk, likely due to its relationship with Primary Sclerosing Cholangitis. Moreover, Crohn’s Disease offers a nearly six-fold increased risk of developing small bowel malignancy. Given its propensity to cause ileitis and intestinal strictures, dysplasia and cancer are worrisome possibilities. This is the first study in recent years to highlight the ability of both forms of IBD to influence development of extracolonic malignancies, which can be used to improve clinical evaluation in this population.

S3497 Tubulo-Villous Adenoma of the Distal Ileum Presenting With Small Bowel Obstruction-Like Symptoms

Introduction: Small bowel tumors (SBT) are rare and account for < 3% of all gastrointestinal tumors in the United States. Given their insidious presentation with non-specific symptoms, they can evade detection and cause delays in diagnosis. Case Description/Methods: A 74-year-old lady came to the ER with abdominal pain, nausea, vomiting and abdominal distention since two days. Past medical history included esophageal spasm, GERD, achalasia, hypertension, CKD with anemia and fibromyalgia and had undergone total abdominal hysterectomy. At her last colonoscopy 10 years ago a 5 cm tubular adenoma was excised and a repeat was advised after 5 years, which she did not follow up with. EGD 8 years ago showed grade 1 reflux esophagitis, normal stomach and small bowel. Pulse was 100/minute and BP was 132/77. Abdomen was soft and distended, with tenderness in the right upper quadrant and epigastrium. Hb was 9.2 g/dL, consistent with her baseline. Though abdominal CT suggested SBO with a transition point just proximal to the ileocecal valve, a small bowel series ruled out an SBO. The patient was made NPO and NGT was placed. Colonoscopy revealed a partially obstructing, medium-sized, fungating, friable, infiltrative, circumferential, villous mass, 8-10 cm away from the ileocecal valve. Histopathology confirmed tubulovillous (TV) adenoma with no signs of dysplasia or malignancy. The patient underwent robotic-assisted ileocecectomy and recovered well. Follow up colonoscopy 4 months later showed healing ileocolonic anastomosis, with no stricture or ulceration. (Figure) Discussion: Though the small bowel constitutes 90% of the surface area of the GI tract, it contributes to < 2% of GI malignancies. Reasons for this include the more liquid contents causing less mucosal irritation than solid colonic contents and rapid transit. Adenomatous polyps, the most common benign SBTs, have an epithelial origin. Histological types include tubular, villous and tubulovillous. Villous components, atypia or large size increase the risk for malignancy. Presenting symptoms are nonspecific and they are commonly found unexpectedly during surgery in patients with SBO. Evaluation in symptomatic patients includes endoscopy with biopsies. Push, double-balloon, or video capsule endoscopy may be needed. Diagnostic laparoscopy or surgical exploration can help establish a definitive diagnosis. Given their potential for malignant transformation, TV adenomas must be removed, and patients should be monitored for recurrence.Figure 1.: A: XR small bowel with oral contrast suggestive of mucosal irregularity with narrowing around distal ileum. B: Colonoscopy showing partially obstructing, circumferential villous mass, 8-10 cm from the ileocecal valve. C: Histo-pathology showing epithelial finger-like projections away from the muscularis mucosae, lined by dysplastic epithelium.

Cardiac Metastasis of Neuroendocrine Tumor Without Cardiovascular Symptoms

A 72-year-old man with a stage IV small intestinal neuroendocrine tumor presented to our cardiology clinic as a referral for an abnormal positron emission tomography–computed tomography scan with an intense gallium uptake in the heart. Follow-up cardiac magnetic resonance was suggestive of myocardium infiltration by the neuroendocrine tumor with late gadolinium enhancement and T1 time elevation. (Level of Difficulty: Intermediate.)

S3378 Bleeding GIST Treated Successfully With Hemospray in a Patient With Neurofibromatosis Type 1

Introduction: Gastrointestinal stromal tumors (GISTs) are rare subepithelial mesenchymal neoplasms of the gastrointestinal tract that are typically KIT (CD117) positive. GISTs can present with a wide range of symptoms including gastrointestinal hemorrhage. Small bowel bleeding from a GIST can be difficult to treat endoscopically and there is a paucity of data on the use of TC-325 (hemospray) for these tumors. Case Description/Methods: A 47-year-old man with a past medical history significant for neurofibromatosis type 1 and multifocal small bowel GISTs presented to our center with symptomatic anemia. The patient described previous episodes of melena. The patient's initial hemoglobin was 2.2 g/dl. He was initially treated with a transfusion of 4 units packed red blood cells. CT abdomen and pelvis with IV contrast was obtained which revealed a centrally located 6 x 5cm small bowel mass with heterogeneous enhancement and intraluminal signal concerning for active gastrointestinal hemorrhage. A push enteroscopy was performed using a pediatric colonoscope via the mouth and advanced deeply into the small bowel. A large ulcerated submucosal mass with active bleeding was found at the junction of the fourth portion of the duodenum and proximal jejunum. Submucosal injection of 1:10,000 diluted epinephrine around the ulcer base failed to achieve hemostasis. Next, the hemospray catheter was advanced to several centimeters proximal to the bleeding ulcer. Hemospray was deployed and immediate hemostasis was achieved after coating the area. Discussion: The majority of GISTs are sporadic and found in the stomach. Patients with neurofibromatosis type 1 however, have a higher incidence of GISTs, are often multifocal, and most commonly are found in the small intestine as demonstrated here. Endoscopic treatment of small bowel tumor bleeding is challenging because of the location and nature of the bleeding. Conventional treatments such a hemoclips or bipolar electrocautery usually fail to stop tumor bleeding. TC-325 or hemospray is a novel inert powder which accelerates the coagulation cascade leading to rapid hemostasis of actively bleeding lesions. Hemospray should be considered first line treatment for malignant bleeding because of its ease of use, wide field of application and demonstrated success.

S3438 Malignant Gastrointestinal Neuroectodermal Tumor of the Small Intestine: A Rare Cause of Iron Deficiency Anemia

Introduction: Gastrointestinal neuroectodermal tumors (GNET) are a type of high-grade sarcoma of the gastrointestinal (GI) tract that share features with clear cell carcinoma of tendon sheaths and aponeuroses. Patients typically present with clinical signs and symptoms of small bowel obstruction, intussusception or abdominal mass. These tumors can arise in the stomach, colon, as well as metastasis to liver or regional lymph nodes. We present a case of ileal GNET in an otherwise asymptomatic patient with Iron-deficiency Anemia (IDA) diagnosed using video capsule endoscopy (VCE). Case Description/Methods: A 58-year-old Hispanic male with a past medical history of GERD and obesity presented for evaluation of chronic microcytic anemia. Workup revealed: hemoglobin 8.6 g/dl, MCV 62.2, iron saturation 2.6%, transferrin 307 mg/dl, ferritin < 5 ng/ml, reticulocyte index of 1.34; serum folate, vitamin B12 and TSH levels were within normal limits. He was taking oral ferrous sulfate and vitamin C for IDA. He reported heartburn but denied melena, hematochezia, hematemesis, weight loss and had an unremarkable colonoscopy four years prior. Upper endoscopy revealed mild erosive esophagitis and mild chronic active gastritis with negative biopsies. Colonoscopy was unremarkable in the setting of optimal bowel preparation. VCE revealed an ulcerated mass occupying 35% of the ileal lumen. CT of the abdomen noted asymmetric wall thickening with enhancement of the small bowel. The patient underwent laparoscopy-assisted enteroscopy with rendezvous technique allowing direct visualization of a 3 cm proximal ileum mass requiring open segmental resection with primary anastomosis. Pathology suggested malignant GNET or clear cell sarcoma-like tumor of the GI tract. Immunohistochemistry was positive for neural crest markers (S100, SOX10) and Vimentin; negative for CD117, DOG1, HMB45, MART1, MAA, chromogranin, and synaptophysin. Fluorescence in-situ hybridization was positive for EWSR1 (22q12) rearrangement. Given the clear surgical margins, absent regional lymph node spread and lack of standard guidelines for this rare pT1N0M0 tumor, surveillance imaging every three months without adjuvant radiation or systemic chemotherapy was recommended. (Figure) Discussion: This case highlights the importance of VCE for small bowel assessment after negative bidirectional endoscopy to identify the etiology of IDA per American Gastroenterology Association guidelines. In this case, an ulcerated GNET was the etiology of IDA persisting despite iron supplementation.Figure 1.: A-Luminal view of small bowel tumor on Video Capsule Endoscopy (VCE) B–Close up view of ulceration of small bowel tumor on VCE C–Histopathology slide showing epithelioid cells with eosinophilic cytoplasm, pleomorphic nuclei with prominent nucleoli and vesicular nucleoli. Scattered mitotic figures identified.

Clinical profile associated with bleeding small bowel tumors in patients undergoing capsule endoscopy

Clinical profile associated with bleeding small bowel tumors in patients undergoing capsule endoscopy

Characteristics and outcome of patients with small bowel adenocarcinoma (SBA)

BackgroundSmall bowel adenocarcinoma (SBA) remains a rare malignancy accounting for less than 5% of all the gastrointestinal tract cancers. However, only limited data and expert guidelines are available for this entity. As a result, treatment concepts are predominantly derived from colorectal cancer.MethodsTo substantiate data on the course of disease, diagnosis and treatment of SBA, we performed a population-based analysis from a Bavarian population of 2.2 million people.ResultsWe identified 223 patients with SBA. Mean age at diagnosis was 67.8 years and patients were diagnosed rather late (34.5% UICC stage IV). Largest proportion of these patients were diagnosed with adenocarcinoma of the duodenum (132 patients, 59.2%) and most patients were diagnosed with late stage cancer, stage IV (70 patients, 31.4%). With respect to treatment, most patients underwent primary surgery (187 patients, 84.6%). Systemic therapy seemed to have an impact in UICC stage IV patients but not in UICC stage IIB or III. The 5-year survival rate was 29.0%. This was significantly less compared to colon cancer in the same cohort, which was 50.0%. Furthermore, median survival of patients with small bowel cancer was only 2.0 years (95% CI 1.4–2.5) compared to 4.9 years (95% CI 4.8–5.1) of patients with colon cancer.ConclusionSBA showed a distinct epidemiology compared to colon cancer. Thus, data acquisition particularly on systemic treatment are paramount, with the objective to complement the available guidelines.

Unexpected Etiology of Ascites in Disseminated Small Bowel Neuroendocrine Tumor Patient Diagnosed and Treated by Interventional Radiological Techniques

A case of ascites developed in disseminated neuroendocrine small bowel tumor G1 (Ki-67 2 %) patient due to the unlikeliest of the possible reasons, i.e. iatrogenic mesenteric arterioportal fistula, is presented in the paper. The patient after palliative small bowel resection was preparing for the liver transarterial chemoembolization (TACE) when the fluid accumulation was revealed in the peritoneal cavity at ultrasound and MR imaging. The probable ascites etiologies (peritoneal carcinomatosis, carcinoid right heart failure, portal hypertension due to tumor compression or vascular elastic fibrosis) were consistently ruled out. The fistulation between the superior mesenteric arterial branch and the superior mesenteric vein developed shot after small bowel surgery and maintaining portal ascites was occasionally found at superior mesentericography. The ascites vanished after fistula occlusion with the metal coils, and the patient proceeded to the planned liver TACE therapies with good effect.

Detachment of an enteroscope spiral fragment during anterograde motorized spiral enteroscopy.

Motorized spiral enteroscopy is a recent technology for deep enteroscopy that uses a 3 cm diameter spiral attached to the tip of an endoscope to facilitate examination of the small bowel. Introduced a few years ago, it has since proved its efficacy and safety in prospective studies [1]. We report the detachment of a spiral ring during motorized spiral enteroscopy during small-bowel tumor examination.

Cancer risk associated with cytomegalovirus infection among solid organ transplant recipients in the United States.

Cytomegalovirus (CMV) is among the most common viral infections after solid organ transplantation (SOT). Associations of CMV with cancer risk among SOT recipients have been incompletely evaluated. The authors used linked data from the US SOT registry and 32 cancer registries. Poisson regression was used to compare cancer incidence across CMV risk groups based on donor (D) and recipient (R) immunoglobulin G (IgG) serostatus: high risk (R-negative/D-positive), moderate risk (R-positive), and low risk (R-negative/D-negative). In total, 247,318 SOT recipients were evaluated during 2000-2017 (R-negative/D-positive, 20.3%; R-positive, 62.9%; R-negative/D-negative, 16.8%). CMV-seropositive recipients were older, more racially/ethnically diverse, and had lower socioeconomic status than CMV-seronegative recipients. Compared with R-negative/D-negative recipients, recipients in the R-negative/D-positive and R-positive groups had a lower incidence of diffuse large B-cell lymphoma (DLBCL; R-negative/D-positive: adjusted incidence rate ratio [aIRR], 0.74; 95% confidence interval [CI], 0.59-0.91; R-positive: aIRR, 0.83; 95% CI, 0.69-1.00). CMV serostatus modified the association between Epstein-Barr virus (EBV) status and DLBCL (p=.0006): DLBCL incidence was increased for EBV R-negative/D-positive recipients (aIRR, 3.46; 95% CI, 1.50-7.95) among CMV R-negative/D-negative recipients but not among the other CMV risk groups. Compared with recipients who were CMV R-negative/D-negative, those who were R-negative/D-positive had a lower incidence of small intestine cancer (aIRR, 0.23; 95% CI, 0.09-0.63), and R-positive recipients had a higher incidence of lung cancer (aIRR, 1.24; 95% CI, 1.05-1.46). CMV status was not associated with risk for other cancers. CMV status was not associated with risk for most cancers among SOT recipients. The inverse association with DLBCL may reflect the protective effects of CMV prophylaxis or treatment with off-target efficacy against EBV infection (the major cause of lymphoma in SOT recipients).