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Small Bowel Cancer

Information for Patients and the Public
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Latest Research Publications
Colorectal (Bowel) Cancer
Gastrointestinal System Cancers

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Information for Health Professionals / Researchers (3 links)

Latest Research Publications

This list of publications is regularly updated (Source: PubMed).

Lovasik BP, Wang VL, Point du Jour KS, et al.
Visceral Kaposi Sarcoma Presenting as Small Bowel Intussusception: A Rare Presentation and Call to Action.
Am Surg. 2019; 85(7):778-780 [PubMed] Related Publications
Surgical emergencies related to visceral involvement of Kaposi sarcoma (KS) are rare complications of the disease. In this report, we describe a case of visceral KS causing small bowel intussusception in a young, previously undiagnosed human immunodeficiency virus (HIV)-positive patient. Southern surgeons should be particularly attentive to HIV/AIDS-related disease as a cause of surgical pathology, particularly in the southeast, and can play a significant advocacy role for improved access to HIV/AIDS diagnostic and treatment services.

Taghipour Zahir S, Heidarymeybodi Z, AleSaeidi S
Prognostic Factors and Survival Time in Patients with Small Bowel Tumors: A Retrospective Observational Study.
Int J Surg Oncol. 2019; 2019:2912361 [PubMed] Free Access to Full Article Related Publications
This study examines survival time in patients with small bowel tumors and determines its contributing factors. In this retrospective analytical study, the medical records of 106 patients with small bowel cancer (from 2006 to 2011) were investigated. The patients' data were extracted, including age, gender, clinical presentation, location of tumor, histological type, grade of tumor, site of metastasis, and type of treatment. The Kaplan-Meier test was used to estimate the overall survival time and the Log-rank test to compare the survival curves. The Cox regression was also used to evaluate the effect of the confounding variables on survival time. This study was conducted on 106 patients with a median age of 60 years (Min: 7, Max: 87). The tumor types included adenocarcinoma (n=78, 73.6%), MALToma (n=22, 20.8%), neuroendocrine tumors (n=4, 3.8%), and sarcoma (n=2. 1.8%). Grade 3 adenocarcinomas had a significantly lower survival time (HR: 1.48, 95% CI: 0.46-2.86; P=.001). Combined therapy (chemotherapy and surgery) vs. single-therapy (only surgery) had no significant effects on the survival of the patients with MALToma (5 vs. 3 months, 95% CI: 1.89-5.26; P=.06). There were no significant differences between the survival time in adenocarcinoma and MALToma (12 vs. 20 months, 95% CI: 6.24-24.76; P=.49). Tumor grade was the only independent prognostic factor that affected survival in adenocarcinoma. The patients diagnosed with MALToma in the study also had a poor prognosis, and the type of treatment had no significant effect on their survival.

Fu H, Li Y, Bai G, et al.
Persistent cholestasis resulting from duodenal papillary carcinoma in an adolescent male: A case report.
Medicine (Baltimore). 2019; 98(22):e15708 [PubMed] Related Publications
RATIONALE: Cholestasis in pediatric patients has diverse etiologies and can be broadly classified as intrahepatic or extrahepatic. The common causes of extrahepatic cholestasis are bile duct calculus, inflammation, or pancreatitis. Malignant tumor is a rare cause of bile ducts obstruction in adolescent. Here we report a 14-year-old male patient with cholestasis due to poorly differentiated adenocarcinoma.
PATIENT CONCERNS: A 14-year-old male patient with cholestasis was admitted because of jaundice, weakness, weight loss, and stomach pain for 2 months. The patient had been diagnosed with epilepsy 4 years previously and was being treated with sodium valproate and oxcarbazepine. On admission, laboratory studies showed elevated levels of aspartate aminotransferase (271 IU/L), alanine aminotransferase (224 IU/l), γ-glutamyltransferase (1668.9 IU/L), total bilirubin (66.4 μmol/L), and direct bilirubin (52.6 μmol/L). Additional laboratory tests eliminated common causes of cholestasis such as bacterial/viral infection, autoimmune liver disease, Wilson disease, Alagille syndrome, or progressive familial intrahepatic cholestasis type 3. The results of laboratory investigations showed no improvement after 10 days of treatment with ursodeoxycholic acid and vitamins A, D, and K1. Enhanced magnetic resonance imaging demonstrated a tumor of 22 mm diameter in the duodenal lumen and dilatation of the common bile duct. Endoscopic retrograde cholangiopancreatography detected a tumor in the duodenal lumen.
DIAGNOSIS: Considering the clinical features, imaging manifestation, endoscopic findings, and pathologic characteristic, the patient was diagnosed with poorly differentiated adenocarcinoma.
INTERVENTIONS: The patient underwent pancreaticoduodenectomy and chemotherapy.
OUTCOME: The patient recovered well. Elevated levels of tumor biomarkers or abnormal liver function tests have not occurred during the 2-year follow-up.
CONCLUSION: Cholestasis resulting from primary duodenal papillary carcinoma is rare in pediatric patients but should be considered in the differential diagnosis.

Caio G, Volta U, Ursini F, et al.
Small bowel adenocarcinoma as a complication of celiac disease: clinical and diagnostic features.
BMC Gastroenterol. 2019; 19(1):45 [PubMed] Free Access to Full Article Related Publications
BACKGROUND: Small bowel adenocarcinoma (SBA) is a rare neoplasm, which can occur in a sporadic form or can be associated with a number of predisposing conditions such as hereditary syndromes and immune-mediated intestinal disorders, e.g. celiac disease (CD). However, the features of SBA in the context of CD remain only partly understood. This study was aimed to show the main clinical features, diagnostic procedures and management options of SBA cases detected in a large cohort of celiac patients diagnosed in a single tertiary care center.
METHODS: We retrospectively reviewed all the SBA cases detected in a cohort of 770 CD patients (599 females; F / M ratio: 3.5:1; median age at diagnosis 36 years, range 18-80 years), diagnosed at the Celiac Disease Referral Center of our University Hospital (Bologna, Italy) from January 1995 to December 2014.
RESULTS: Five (0.65%) out of our 770 CD patients developed SBA. All of them were female with a mean age of 53 years (range 38-72 years). SBA, diagnosed at the same time of the CD diagnosis in three cases, was localized in the jejunum in four cases and in the duodenum in one case. The clinical presentation of SBA was characterized by intestinal sub-occlusion in two cases, while the predominant manifestation of the remaining three cases was iron deficiency anaemia, abdominal pain and acute intestinal obstruction, respectively. All the patients were referred to surgery, and three cases with advanced stage neoplasia were also treated with chemotherapy. The overall survival rate at 5 years was 80%.
CONCLUSIONS: Although in a limited series, herein presented CD-related SBA cases were characterized by a younger age of onset, a higher prevalence in female gender and a better overall survival compared to sporadic, Crohn- and hereditary syndrome-related SBA.

Dapri G, Bascombe NA
Three trocars laparoscopic right ileocolectomy for advanced small bowel neuroendocrine tumor.
Surg Oncol. 2019; 28:76-77 [PubMed] Related Publications
BACKGROUND: In the last decade Reduced Port Laparoscopy (RPL) has been introduced to reduce the risks related to the trocars and abdominal wall trauma, with enhanced cosmetic outcomes. The authors report a 59 year old man with a small bowel neuroendocrine tumor, submitted to three trocars right ileocolectomy.
VIDEO: Preoperative work-up, including endoscopic ultrasound, octreo-PET-CT and FDG PET-CT, showed a 15 mm small bowel low grade well differentiated neuroendocrine tumor with mesenteric and transverse mesocolic extension, until the muscularis propria of the 3rd duodenum. The procedure was performed using three trocars: 12-mm in the umbilicus, 5-mm in the right and left flanks. After mobilization of the right colon, the 2nd and 3rd duodenal segments were exposed, showing tumor extension to the anterior duodenal wall. After encircling the anterior aspect of the duodenal wall with a piece of cotton tape, a linear stapler was inserted through the umbilical trocar under a 5-mm scope in the left flank, and it was fired. The specimen was removed through a suprapubic access. Frozen section biopsy showed free duodenal margin, hence the procedure was finished with handsewn intracorporeal ileocolic anastomosis.
RESULTS: Operative time was 4 hours. No added trocars were necessary. Patient was discharged on 4th day. Pathology showed a grade I, well differentiated small bowel neuroendocrine tumor, with lymphovascular emboli and perinervous infiltration; 1/20 metastatic nodes, free margins; stage (8 UICC edition): pT3N1. At 12 months of follow-up the patient is free of disease.
CONCLUSIONS: RPL offers all MIS advantages, including reduced trocar complications and enhanced cosmetic outcomes.

Baiu I, Visser BC
Minimally Invasive Small Bowel Cancer Surgery.
Surg Oncol Clin N Am. 2019; 28(2):273-283 [PubMed] Related Publications
Small bowel malignancies are extremely rare. Surgical resection is often the mainstay of treatment with the extent of the operation depending on the type of tumor. Whereas neuroendocrine tumors and adenocarcinoma require lymph node resection, gastrointestinal stromal tumors do not typically metastasize to regional nodes and therefore need resection only. Minimally invasive approaches are applicable to small tumors that require a limited resection and reconstruction and have been shown to have equal survival benefits with decreased risk of postoperative complications.

Vasilakakis M, Koulaouzidis A, Yung DE, et al.
Follow-up on: optimizing lesion detection in small bowel capsule endoscopy and beyond: from present problems to future solutions.
Expert Rev Gastroenterol Hepatol. 2019; 13(2):129-141 [PubMed] Related Publications
INTRODUCTION: This review presents noteworthy advances in clinical and experimental Capsule Endoscopy (CE), focusing on the progress that has been reported over the last 5 years since our previous review on the subject. Areas covered: This study presents the commercially available CE platforms, as well as the advances made in optimizing the diagnostic capabilities of CE. The latter includes recent concept and prototype capsule endoscopes, medical approaches to improve diagnostic yield, and progress in software for enhancing visualization, abnormality detection, and lesion localization. Expert commentary: Currently, moving through the second decade of CE evolution, there are still several open issues and remarkable challenges to overcome.

Xu C, Wang K, Ding YH, et al.
World J Gastroenterol. 2019; 25(5):584-599 [PubMed] Free Access to Full Article Related Publications
BACKGROUND: Claudin-7, one of the important components of cellular tight junctions, is currently considered to be expressed abnormally in colorectal inflammation and colorectal cancer. However, there is currently no effective animal model to study its specific mechanism. Therefore, we constructed three lines of
AIM: To determine the function of the tumor suppressor gene
METHODS: We crossed
RESULTS: We generated
CONCLUSION: The knockout of

Clark M, Nathan M, Broski SM
Multifocal Small Bowel Carcinoid: Evaluation by 68Ga-DOTATATE PET.
Clin Nucl Med. 2019; 44(4):e308-e310 [PubMed] Related Publications
Carcinoid is a subtype of neuroendocrine tumor, a rare group of tumors that are known to express somatostatin receptor 2. Ga-DOTATATE is a somatostatin analog that is specific for somatostatin receptor 2 and therefore allows visualization of neuroendocrine tumors. We present 2 cases of primary multifocal small bowel carcinoid evaluated using Ga-DOTATATE PET/CT, along with contrast-enhanced CT corollary findings. Given the increased sensitivity of Ga-DOTATATE PET/CT compared with CT and In-octreotide scintigraphy, we expect multifocal carcinoid to become increasingly recognized.

Manguso N, Gangi A, Nissen N, et al.
Long-Term Outcomes after Elective
Am Surg. 2018; 84(10):1570-1574 [PubMed] Related Publications
Small bowel neuroendocrine tumors (SBNETs) are often indolent, but occasionally, patients present with acute symptoms requiring emergent operative intervention. Our aim was to determine whether emergency surgery for SBNETs affects long-term outcomes. An institutional database was reviewed to identify patients with SBNET diagnosed between 1990 and 2015. Need for emergency resection (ER) was compared with elective resection (ELR). One hundred and thirty-four patients met inclusion criteria. Median age was 59 years (range, 21-91), and median tumor size was 1.5 cm (range, 0.1-5). Median follow-up time was 5.5 years. One hundred (74.6%) patients had ELR, whereas 34 (25.4%) required ER. ELR had a higher number of lymph nodes resected (median 12.5

Quaas A, Heydt C, Waldschmidt D, et al.
Alterations in ERBB2 and BRCA and microsatellite instability as new personalized treatment options in small bowel carcinoma.
BMC Gastroenterol. 2019; 19(1):21 [PubMed] Free Access to Full Article Related Publications
BACKGROUND: Carcinomas of the small bowel are rare tumors usually with dismal prognosis. Most recently, some potentially treatable molecular alterations were described. We emphasize the growing evidence of individualized treatment options in small bowel carcinoma.
METHODS: We performed a DNA- based multi-gene panel using ultra-deep sequencing analysis (including 14 genes with up to 452 amplicons in total; KRAS, NRAS, HRAS, BRAF, DDR2, ERBB2, KEAP1, NFE2L2, PIK3CA, PTEN, RHOA, BRCA1, BRCA2 and TP53) as well as an RNA-based gene fusion panel including ALK, BRAF, FGFR1, FGFR2, FGFR3, MET, NRG1, NTRK1, NTRK2, NTRK3, RET and ROS1 on eleven formalin fixed and paraffin embedded small bowel carcinomas. Additionally, mismatch-repair-deficiency was analyzed by checking the microsatellite status using the five different mononucleotide markers BAT25, BAT26, NR-21, NR-22 and NR-27 and loss of mismatch repair proteins using four different markers (MLH1, MSH6, MSH2, PMS2).
RESULTS: In five out of eleven small bowel carcinomas we found potentially treatable genetic alterations. Three patients demonstrated pathogenic (class 5) BRCA1 or BRCA2 mutations - one germline-related in a mixed neuroendocrine-non neuroendocrine neoplasm (MiNEN). Two additional patients revealed an activating ERBB2 mutation or PIK3CA mutation. Furthermore two tumors were highly microsatellite-instable (MSI-high), in one case associated to Lynch-syndrome. We did not find any gene fusions.
CONCLUSION: Our results underscore, in particular, the relevance of potentially treatable molecular alterations (like ERBB2, BRCA and MSI) in small bowel carcinomas. Further studies are needed to proof the efficacy of these targeted therapies in small bowel carcinomas.

Cumberledge JD, Anaka I, Kupec JT
Rare case of small intestine bleeding.
BMJ Case Rep. 2019; 12(1) [PubMed] Related Publications
We present a case of a healthy 59-year-old woman who presented for a capsule endoscopy to evaluate melaena and iron deficiency anaemia. She had previously underwent an oesophagogastroduodenoscopy and colonoscopy at an outside institution which were unremarkable. Capsule endoscopy showed an ulcerated, bleeding lesion likely in the duodenum. Differential diagnosis included adenocarcinoma, carcinoid tumour, lymphoma, gastrointestinal stromal tumour and metastatic disease. A push enteroscopy was performed after which showed an ulcerated mass in the third portion of the duodenum. Biopsies confirmed adenocarcinoma. Computed tomography of the abdomen showed no signs of distant metastasis and the patient was referred to surgery for evaluation. The patient underwent a pancreaticoduodenectomy, with resection of the mass and negative lymph nodes in all nine that were removed (T3N0). The patient was classified as stage II duodenal adenocarcinoma. Duodenal adenocarcinoma is a rare but clinically significant cause of small bowel bleeding.

Kundu R, Gupta S, Kaushik R, et al.
Isolated giant ileal neurofibroma sans neurofibromatosis.
Indian J Cancer. 2018 Jul-Sep; 55(3):301-303 [PubMed] Related Publications
Neurofibromas are benign neoplasms that are usually seen in hereditary disorders such as von Recklinghausen's disease [neurofibromatosis type 1 (NF1)]. The occurrence of isolated ileal neurofibroma in patients without the classic manifestations of NF1 or multiple endocrine neoplasia (MEN) syndromes is an extremely rare entity . We report one such case of isolated ileal neurofibroma in a 60 year old woman without any other stigmata of NF. It may be the initial manifestation of NF1 or MEN 2b or malignant transformation, all of which necessitate further follow-up of these patients.

Orbion A, Mouman A, Behr J, et al.
Correlation between a continent ileocecal valve and CT signs of severity in patients presenting with obstructive colonic cancer.
Emerg Radiol. 2019; 26(3):277-282 [PubMed] Related Publications
OBJECTIVE: To study the association of a continent ileocecal valve and the degree of severity of the CT signs in patients presenting with large bowel obstruction due to colonic cancer.
PATIENTS AND METHODS: Sixty-six patients undergoing emergency surgery for confirmed obstructive colonic cancer were included. The CT examinations were analyzed without consultation of the surgical results. For each patient, the diameter of the cecum at its widest point and that of the last ileal loop were measured. The ileocecal valve was considered incontinent when there was a distension of the last ileal loop greater than or equal to 25 mm. Below 25 mm, the ileocecal valve was considered continent. The presence of CT signs of severity of the LBO was noted, i.e., intestinal pneumatosis, absence of contrast enhancement of the large bowel wall, defect in the large bowel wall, and presence of extra-digestive air and ascites.
RESULTS: Among the 66 patients included, 42 had an incontinent ileocecal valve and 24 had a continental ileocecal valve. There was a statistically significant difference between the two groups in the diametrical measurements of the cecum's widest point (mean diameter measured at 10.3 cm in patients with continent ileocecal valve vs 8.4 cm in patients with incontinent ileocecal valve, P = 0.0023). Patients with a continent valve had statistically higher rates of CT severity (79% vs 40%, P < 0.005). Perforation of the cecum remained rare (8%) and was only observed in patients with continent ileocecal valve in our series.
CONCLUSION: Continence of the ileocecal valve appears to be statistically correlated both with cecum distension and the presence of CT signs of severity in patients with obstructive colonic cancer. As such, its presence must be retained as a risk factor for a pejorative evolution of this type of LBO and must be specified in the CT report of these patients.

Xi S, Tong W
Pleomorphic rhabdomyosarcoma metastasis to small intestine causing intussusception: A case report.
Medicine (Baltimore). 2018; 97(51):e13648 [PubMed] Free Access to Full Article Related Publications
RATIONALE: Pleomorphic rhabdomyosarcoma (PRMS) is a rare soft tissue malignancy which is frequently misdiagnosed and associated with metastasis to the lungs, lymph nodes, and bone marrow. Case studies are needed to improve the awareness of the disease and our understanding of it.
PATIENT CONCERNS: In this study, we present a case of a 36-year-old man with a lesion on the right back shoulder. Lesion was confirmed by magnetic resonance imaging (MRI) around the right armpit, subscapularis, deltoid, and infraspinatus muscle, with oozing surrounding soft tissues.
DIAGNOSIS: The tumor was diagnosed as PRMS which metastasized to the intestine, where it caused intussusception.
INTERVENTIONS: The patient was treated by complete surgery in combination with neo-adjuvant chemotherapy including ifosfamide and epirubicin.
OUTCOME: The patient remained alive 6 months after the treatment with no recurrence and metastasis.
LESSON: PRMS can be aggressive, and surgical treatment in combination with multidrug chemotherapy can be used in the management.

Iemoto T, Sanuki T, Ose T, et al.
Acute Pancreatitis Caused by Ampullary Duodenum Adenoma in a Patient with Adenomatous Polyposis Coli with Billroth II Reconstruction After Distal Gastrectomy.
Am J Case Rep. 2018; 19:1495-1498 [PubMed] Free Access to Full Article Related Publications
BACKGROUND Adenomatous polyposis coli is an autosomal dominant hereditary disorder. Duodenal adenocarcinoma and adenoma, which are extracolonic lesions, not only affect the prognosis of patients but also cause acute pancreatitis. CASE REPORT We present the case of a 73-year-old male. He had undergone proctocolectomy for familial adenomatous polyposis and distal gastrectomy (Billroth II reconstruction with Braun anastomosis) for gastric ulcer; he presented with acute pancreatitis caused by ampullary duodenum adenoma. Double-balloon endoscopy showed 2 adenomatous polyps in the major papilla and descending limb of the duodenum. Based on the findings of endoscopy and biopsy, the duodenal polyps were diagnosed as adenomas and classified as Spigelman stage II. CONCLUSIONS Our case report suggests that duodenal surveillance is necessary for patients with adenomatous polyposis coli. In addition, surveillance using double-balloon endoscopy is useful for patients with an altered gastrointestinal anatomy.

Fotouhi O, Zedenius J, Höög A, Juhlin CC
Regional differences in somatostatin receptor 2 (SSTR2) immunoreactivity is coupled to level of bowel invasion in small intestinal neuroendocrine tumors.
Neuro Endocrinol Lett. 2018; 39(4):305-309 [PubMed] Related Publications
OBJECTIVE: Somatostatin receptor (SSTR) expression constitutes a pivotal cornerstone for accurate radiological detection and medical treatment of small intestinal neuroendocrine tumors (SI-NETs), and the development of somatostatin analogues for these purposes have revolutionized the clinical work-up. Previous assessments of SSTR isoform expression in SI-NETs have found correlations to overall prognosis and treatment response, however these analyses usually report overall tumoral immunoreactivity, and little is reported regarding histo-regional differences in expressional patterns.
METHODS: Thirty-seven primary SI-NETs (WHO grade I, n=32 and WHO grade II, n=5) were collected and assessed for SSTR2 immunohistochemistry. Samples were stratified with regards to histological level of bowel infiltration and spread (mucosal region, muscularis propria region, subserosal region) and each of these tumoral regions was separately scored by SSTR2 staining localization (membrane, cytoplasmic), overall staining intensity and local staining differences within each region.
RESULTS: SSTR2 immunoreactivity was progressively weaker as the tumor cells advanced through the small intestinal layers. This was exemplified by a reduction in the amount of tumor samples with strong SSTR2 expression in the deeper histological levels of the section; 56% of tumors displayed strong SSTR2 expression in the mucosal region, as compared to 29% and 30% of tumors within muscularis propria and subserosal layers, respectively.
CONCLUSIONS: This observation indicates a down-regulation of SSTR2 expression as the tumors progress through the intestinal wall, which might signify underlying biological processes of importance for SI-NET invasion behavior.

Feng C, Lu F, Shen Y, et al.
Tumor heterogeneity in gastrointestinal stromal tumors of the small bowel: volumetric CT texture analysis as a potential biomarker for risk stratification.
Cancer Imaging. 2018; 18(1):46 [PubMed] Free Access to Full Article Related Publications
BACKGROUND: To explore whether volumetric CT texture analysis (CTTA) can serve as a potential imaging biomarker for risk stratification of small bowel gastrointestinal stromal tumors (small bowel-GISTs).
METHODS: A total of 90 patients with small bowel-GISTs were retrospectively reviewed, of these, 26 were rated as high risk, 13 as intermediate risk, and 51 as low or very low risk. Histogram parameters extracted from CT images were compared among small bowel-GISTs with different risk levels by using one-way analysis of variance. Receiver operating characteristics (ROCs) and areas under the curve (AUCs) were analyzed to determine optimal histogram parameters for stratifying tumor risk.
RESULTS: Significant differences in mean attenuation, 10th, 25th, 50th, 75th and 90th percentile attenuation, and entropy were found among high, intermediate, and low risk small bowel-GISTs (p ≤ 0.001). Mean attenuation, 10th, 25th, 50th, 75th and 90th percentile attenuation, and entropy derived from arterial phase and venous phase images correlated significantly with risk levels (r = 0.403-0.594, r = 0.386-0.593, respectively). Entropy in venous phase reached the highest accuracy (AUC = 0.830, p < 0.001) for differentiating low risk from intermediate to high risk small bowel-GISTs, with a cut-off value of 5.98, and the corresponding sensitivity and specificity were 82.4 and 74.4%, respectively.
CONCLUSIONS: Volumetric CT texture features, especially entropy, may potentially serve as biomarkers for risk stratification of small bowel-GISTs.

Rompteaux P, Gagnière J, Gornet JM, et al.
Resection of small bowel adenocarcinoma metastases: Results of the ARCAD-NADEGE cohort study.
Eur J Surg Oncol. 2019; 45(3):331-335 [PubMed] Related Publications
INTRODUCTION: Data are lacking with regard to curative resection of metastasis from small bowel adenocarcinoma (SBA). This study evaluated outcomes and prognostic factors in patients with curatively resected metastatic SBA.
METHODS: A series of 34 patients undergoing resection of metastatic SBA from January 2009 to November 2014 at French centers were included into this cohort study. The primary endpoint was overall survival (OS). Secondary endpoints were recurrence-free survival (RFS) and prognostic factors. Univariate analyses were performed to determine prognostic risk factors.
RESULTS: The sites of SBA metastases were peritoneal (29.4%), liver (26.5%), lymph nodes (11.8%), lung (2.9%), multiple (14.7%), and other (14.7%). Thirty (88.2%) patients received adjuvant or perioperative chemotherapy, mainly was oxaliplatin-based (76.5%). The median OS was 28.6 months and RFS was 18.7 months. Fourteen (41.2%) patients survived for more than 36 months. In univariate analysis, poor differentiation (P = 0.006), invaded margins (P = 0.003), and lymphatic invasion in the primary tumor (P = 0.039) were associated with decreased OS.
CONCLUSION: Overall survival of patients after resection of metastatic SBA remains poor, but long-term survivors are observed. Resection of metastatic SBA should be consider if patients are expected to be operated on with curative intent and have moderately or well-differentiated tumors.

Pan ST, Ko YH, Tan SY, Chuang SS
Primary cutaneous peripheral T-cell lymphoma with a late relapse solely in the ileum mimicking monomorphic epitheliotropic intestinal T-cell lymphoma.
Pathol Res Pract. 2018; 214(12):2106-2109 [PubMed] Related Publications
BACKGROUND: Primary cutaneous peripheral T-cell lymphomas (PC-PTCLs) are classified into mycosis fungoides (MF) and other rare specific types; and those do not fit into any specific entities are designated as PTCL, not otherwise specified (NOS), an aggressive neoplasm. Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is an aggressive primary intestinal T-cell lymphoma with enteropathy in the non-neoplastic mucosa. We report a rare case of PC-PTCL-NOS with a late relapse solely in the ileum after complete remission. We discuss the importance of evaluating enteropathy, megakaryocyte-associated tyrosine kinase (MATK) immunostaining, and the implication of clonal relationship of metachronous lymphomas.
CASE REPORT: We reviewed the histopathology and immunohistochemistry of the skin tumor from a 68-year-old female and the relapsed intestinal T-cell lymphoma. The tumor cells "trans-regressed" from large and pleomorphic in the skin to small/medium-sized cells with clear cytoplasm in the ileum; and furthermore, there was immunophenotypic alteration. However, there was no enteropathy in the non-tumoral ileal mucosa adjacent to the tumor proper and both the cutaneous and ileal tumors were negative for MATK. Clonality study showed clonal TRG and TRB rearrangement with identical band sizes of the amplicons, confirming primary cutaneous tumor with a late relapse in the ileum.
CONCLUSIONS: Although PC-PTCL-NOS is an aggressive neoplasm, rare cases such as this might have a long-term survival. Furthermore, the late relapse mimicking MEITL is intriguing and exceptional, in spite the fact that MEITL is a primary intestinal T-cell lymphoma with a typical histopathology and immunophenotype. Detailed clinicopathological and molecular studies are mandatory to elucidate the clonal relationship of metachronous lymphomas, as this has important clinical implication for treatment. Evaluation of the non-tumoral intestinal mucosa for enteropathy and immunostaining for MATK might help to differentiate a mimicker from a true MEITL.

Hammoudi N, Dhooge M, Coriat R, et al.
Duodenal tumor risk in Lynch syndrome.
Dig Liver Dis. 2019; 51(2):299-303 [PubMed] Related Publications
BACKGROUND AND AIMS: Lynch syndrome (LS) is associated with an increased risk of small bowel tumors but routine screening is not recommended in international guidelines. The aim of our study was to determinate the prevalence of duodenal tumors in a French cohort of LS patients.
METHODS: Patients carrying a germline pathogenic variant in a MMR gene, supported by our local network, in which at least one upper endoscopy had been performed, were included. We registered the occurrence of duodenal lesions in those patients.
RESULTS: 154 LS patients were identified including respectively 85 MSH2 and 41 MLH1 mutated patients respectively. Seven out of 154 (4.5%) had at least one duodenal lesion. Median age at diagnosis was 58 years (range: 49-73). The twelve lesions locations were: descending duodenum (n = 7), genu inferius (n = 2), duodenal bulb (n = 1), ampulla (n = 1), fourth duodenum (n = 1). Three lesions were invasive adenocarcinomas. The incidence rate of duodenal lesions in patients with MSH2 or MLH1 pathogenic variants was respectively 7.1% (6 out of 85) and 2.4% (1 out of 41) emphasizing a trend toward increased risk of developing duodenal lesion in MSH2 mutated patients: OR: 5.17, IC95% (0.8-60.07), p = 0.1307.
CONCLUSION: Regarding this high prevalence rate, especially in MSH2 patients, regular duodenal screening during upper endoscopy should be considered in routine in LS patients.

Seo YK, Choi JS
Endoscopic Papillectomy for Synchronous Major and Minor Duodenal Papilla Neuroendocrine Tumors.
Korean J Gastroenterol. 2018; 72(4):217-221 [PubMed] Related Publications
Neuroendocrine tumor (NET) of the major duodenal papilla is a rare occurrence. However, that of the minor duodenal papilla is even rarer. To date, only a few cases have been reported. Herein, we present a rare case of NETs detected at the major and minor duodenal papilla synchronously, which were successfully treated with endoscopic papillectomy without procedure-related complication. To the best of our knowledge, this is the first report of this kind in the world. Photomicrograph of the biopsy specimen stained immunohistochemically for synaptophysin showed a positive reaction of tumor cells. All resection margins were negative. Further experience with more cases will be needed to establish the exact indication of endoscopic papillectomy for duodenal papillary NETs.

Melnitchouk N, Saadat LV, Bleday R, Goldberg JE
A Decision Analysis for Rectal-Sparing Familial Adenomatous Polyposis: Total Colectomy With Ileorectal Anastomosis Versus Proctocolectomy With IPAA.
Dis Colon Rectum. 2019; 62(1):27-32 [PubMed] Related Publications
BACKGROUND: There are different approaches for the surgical management of rectal-sparing familial adenomatous polyposis with variable impacts on both quality of life and survival.
OBJECTIVE: The aim of this study was to quantify the trade-offs between total proctocolectomy with IPAA versus total colectomy with ileorectal anastomosis using decision analysis.
DESIGN: We created a disease simulation Markov model to simulate the clinical events after IPAA and ileorectal anastomosis for rectal-sparing familial adenomatous polyposis in a cohort of individuals at the age 30 years. We used available literature to obtain different transition probabilities and health-states utilities. The output parameters were quality-adjusted life-years and life-years. Deterministic and probabilistic sensitivity analyses were performed.
SETTINGS: A decision analysis using a Markov model was conducted at a single center.
PATIENTS: Patients with rectal-sparing familial adenomatous polyposis at age 30 years were included. Rectal-sparing familial adenomatous polyposis is defined as the presence of 0 to 20 polyps that can be removed endoscopically.
MAIN OUTCOME MEASURES: Quality-adjusted life-years were measured.
RESULTS: Our model showed that the mean quality-adjusted life-years for IPAA was 25.12 and for ileorectal anastomosis was 27.12 in base-case analysis. Mean life-years for IPAA were 28.81 and 28.28 for ileorectal anastomosis. A 1-way sensitivity analysis was performed for all of the parameters in the model. None of the deterministic sensitivity analyses changed the model results across the range of plausible values. Probabilistic analysis identified that, in 86.9% of scenarios, ileorectal anastomosis had improved quality-adjusted life-years compared with IPAA.
LIMITATIONS: The study was limited by characteristics inherent to modeling studies.
CONCLUSIONS: Ileorectal anastomosis was found to be preferable for patients with rectal-sparing familial adenomatous polyposis when quality of life is taken into consideration. This model was robust based on both deterministic and probabilistic sensitivity analyses. These data should be taken into consideration when counseling patients regarding a surgical approach in rectal-sparing familial adenomatous polyposis. See Video Abstract at http://links.lww.com/DCR/A715.

Song JS, Yi JM, Cho H, et al.
Dual loss of USP10 and p14ARF protein expression is associated with poor prognosis in patients with small intestinal adenocarcinoma.
Tumour Biol. 2018; 40(10):1010428318808678 [PubMed] Related Publications
Oncogene-induced senescence occurs following oncogene activation in normal cells and is considered as a critical tumor-suppressing mechanism. Ubiquitin-specific protease 10 (USP10) has been reported to play a vital role in oncogene-induced senescence via the deubiquitination-dependent stabilization of p14ARF. However, knowledge of the clinical significance of USP10 and p14ARF expression in patients with small intestinal adenocarcinoma is limited. To study the clinical significance of USP10 and p14ARF expression, we performed immunohistochemistry for USP10 and p14ARF on 195 surgically resected small intestinal adenocarcinoma specimens. Furthermore, we performed methylation analysis on five small intestinal adenocarcinoma samples and matched adjacent normal intestinal tissue samples. UPS10 ( p = 0.023) and p14ARF ( p = 0.007) expression were significantly decreased in adenocarcinoma in comparison with normal tissue. The loss of USP10 was observed in 124/194 (63.9%) of small intestinal adenocarcinoma samples and was correlated with a higher pT stage ( p = 0.044), lymphatic invasion ( p = 0.033), and the absence of sporadic adenoma ( p = 0.024) and peritumoral dysplasia ( p = 0.019). p14ARF expression was downregulated in 75/195 (38.5%) of small intestinal adenocarcinoma samples and was associated with vascular ( p = 0.011) and lymphatic ( p = 0.013) invasions. The loss of USP10 expression was associated with the loss of p14ARF expression ( r = 0.342, p < 0.001). Multivariate survival analysis revealed that the combined loss of USP10 and p14ARF expression could be an independent prognostic factor for overall survival in small intestinal adenocarcinoma. Furthermore, the aberrant hypermethylation of the USP10 and p14ARF promoter could be a key mechanism for the downregulation of USP10 and p14ARF proteins in small intestinal adenocarcinoma. These findings suggest that the dual loss of USP10 and p14ARF could be used as a prognostic indicator of small intestinal adenocarcinoma.

Tang L, Zhao P, Kong D
The risk factors for benign small bowel obstruction following curative resection in patients with rectal cancer.
World J Surg Oncol. 2018; 16(1):212 [PubMed] Free Access to Full Article Related Publications
BACKGROUND: So far there have been limited studies about the risk factors for benign small bowel obstruction (SBO) after colorectal cancer surgery. This study aimed to determine the factors affecting the development of benign SBO following curative resection in patients with rectal cancer.
METHODS: Patients (3472) receiving curative resection of rectal cancer at the Department of Colorectal Cancer, Tianjin Medical University Cancer Institute and Hospital, between January 2003 and December 2012 were retrospectively studied. The incidence of benign SBO and its risk factors were then determined.
RESULTS: The incidence of benign SBO was 7.3% (253/3472) in follow-up studies with an average time of 68 months. Further, 27% (68/253) of the patients received operative treatment because of the signs of strangulation or the lack of clinical improvement with conservative management. Open surgery and radiotherapy were defined as the risk factors for benign SBO after curative resection in patients with rectal cancer (P <  0.001).
CONCLUSION: Open surgery plus radiotherapy led to an increased risk of benign SBO in rectal cancer patients receiving curative resection.

Ekici U, Yilmaz S, Tatli F
Ileocecal invagination due to an inflammatory fibroid polyp. A case report.
Ann Ital Chir. 2018; 89:347-349 [PubMed] Related Publications
The invagination develops as a result of the dislocation of proximal bowel segment within the following distal portion. It is rarely seen in adult population. It generally develops as a result of intestinal wall neoplasia in the adult patients. Only 3-6% of gastrointestinal system tumors are seen to be localized to small bowels. Inflammatory fibroid polyp is a rarely seen benign tumor in gastrointestinal system. We present the case of 47 year-old female with ileo-cecal invagination caused by Inflammatory fibroid polyp in ileum. KEY WORDS: Invagination, Intusseption, Inflamaotry Fibroid Polyp.

Aden K, Bartsch K, Dahl J, et al.
Epithelial RNase H2 Maintains Genome Integrity and Prevents Intestinal Tumorigenesis in Mice.
Gastroenterology. 2019; 156(1):145-159.e19 [PubMed] Free Access to Full Article Related Publications
BACKGROUND & AIMS: RNase H2 is a holoenzyme, composed of 3 subunits (ribonuclease H2 subunits A, B, and C), that cleaves RNA:DNA hybrids and removes mis-incorporated ribonucleotides from genomic DNA through ribonucleotide excision repair. Ribonucleotide incorporation by eukaryotic DNA polymerases occurs during every round of genome duplication and produces the most frequent type of naturally occurring DNA lesion. We investigated whether intestinal epithelial proliferation requires RNase H2 function and whether RNase H2 activity is disrupted during intestinal carcinogenesis.
METHODS: We generated mice with epithelial-specific deletion of ribonuclease H2 subunit B (H2b
CONCLUSIONS: In analyses of mice with disruption of the ribonuclease H2 subunit B gene and colorectal tumors from patients, we provide evidence that RNase H2 functions as a colorectal tumor suppressor. H2b/p53

Schmuck RB, Brokat C, Andreou A, et al.
Clinicopathological Stratification and Long-term Follow-up of Patients with Periampullary Carcinomas.
Anticancer Res. 2018; 38(9):5379-5386 [PubMed] Related Publications
BACKGROUND: Periampullary carcinomas generally confer a poor outcome. Choosing the most effective treatment regimen for each sub-entity proves challenging and is usually based on experience from pancreatic adenocarcinoma (PDAC).
PATIENTS AND METHODS: The long-term follow-up is presented of 472 patients with periampullary tumors [PDAC, distal cholangiocarcinoma (dCC) and ampullary carcinomas (AC)] who underwent radical resection considering clinical characteristics, paraclinical findings and histopathological features in order to define factors of prognostic relevance.
RESULTS: Patients with PDACs presented with larger tumor sizes, more frequent R1 resection, higher rate of nodal and perineural invasion, higher tumor stage according to the classification of tumors of the Union Internationale contre le Cancer when compared to those with dCCs and ACs. In a multivariate analysis, age >65 years, postoperative complications and higher grading of the tumor proved to be independent prognostic factors for survival.
CONCLUSION: Patients suffering from PDAC have the worst prognosis and greatest benefit from radical resection of all patients with periampullary tumors. More detailed studies are warranted to better distinguish between the different entities.

Nicolas G, Nasser H, Haddad J, et al.
Periampullary Neuroendocrine Tumor as a Cause of Acute Pancreatitis.
Am J Case Rep. 2018; 19:1063-1067 [PubMed] Free Access to Full Article Related Publications
BACKGROUND Duodenal and ampullary carcinoids are very rare tumors accounting respectively for 2% and 0.03% of all carcinoid tumors. Clinical findings vary according to the location of the tumor within the periampullary region; with epigastric pain being the most common presenting symptom in duodenal carcinoids and jaundice the most common clinical finding in ampullary carcinoids. Treatment options include pancreaticoduodenectomy, local excision, and endoscopic excision. CASE REPORT In this case report, we present a 60-year-old male who presented with a one-week history of intractable epigastric pain. He was diagnosed with duodenal periampullary carcinoid tumor and treated with local excision. CONCLUSIONS Although duodenal and ampullary carcinoid tumors may have different clinical presentations, as well as histochemistry characteristics and metastatic potential, they appear to benefit from the same surgical treatment.

Kim J, Yoon CJ, Seong NJ
Transjejunostomy Stent Placement in Patients With Malignant Small-Bowel Obstructions.
AJR Am J Roentgenol. 2018; 211(5):1148-1154 [PubMed] Related Publications
OBJECTIVE: The objective of our study was to evaluate the clinical effectiveness and safety of transjejunostomy stent placement in patients with malignant small-bowel obstructions (SBOs).
MATERIALS AND METHODS: Twenty-three patients (age range, 20-81 years) who underwent transjejunostomy stent placement between March 2009 and December 2016 for one (n = 20) or two (n = 3) malignant SBOs from advanced abdominal and pelvic malignancies were included in our study. Percutaneous jejunostomy was created 30-100 cm upstream to malignant SBOs and was immediately followed by stent placement through the jejunostomy stoma during the same session. A retrospective analysis was conducted for technical success, bowel decompression, improvement of obstructive symptoms (3-point scale), improvement of food intake capacity (4-point scale), and procedure-related complications.
RESULTS: Stent placement was technically successful in 22 of 23 patients (95.7%). Bowel decompression was confirmed by enterography (n = 21) and CT (n = 16). Obstructive symptoms improved partially (n = 9) or completely (n = 13) within 2 weeks after the procedure. Food intake capacity improved by 3 points in one patient, 2 points in seven patients, and 1 point in 14 patients (p < 0.0001). Major complications (n = 3, 13.0%), including localized peritonitis (n = 2) and bowel perforation (n = 1), were successfully treated conservatively.
CONCLUSION: Transjejunostomy stent placement is an effective treatment in patients with malignant SBOs. It is technically feasible in most patients (95.7%) and provides substantial symptomatic improvement. Procedure-related complications are not rare but can be managed conservatively.

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