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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
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
Persistent cholestasis resulting from duodenal papillary carcinoma in an adolescent male: A case report.
Medicine (Baltimore). 2019; 98(22):e15708 [PubMed] Related Publications
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.
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
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.
Three trocars laparoscopic right ileocolectomy for advanced small bowel neuroendocrine tumor.
Surg Oncol. 2019; 28:76-77 [PubMed] Related Publications
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.
Minimally Invasive Small Bowel Cancer Surgery.
Surg Oncol Clin N Am. 2019; 28(2):273-283 [PubMed] Related Publications
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
World J Gastroenterol. 2019; 25(5):584-599 [PubMed] Free Access to Full Article Related Publications
AIM: To determine the function of the tumor suppressor gene
METHODS: We crossed
RESULTS: We generated
CONCLUSION: The knockout of
Multifocal Small Bowel Carcinoid: Evaluation by 68Ga-DOTATATE PET.
Clin Nucl Med. 2019; 44(4):e308-e310 [PubMed] Related Publications
Long-Term Outcomes after Elective
Am Surg. 2018; 84(10):1570-1574 [PubMed] Related Publications
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
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.
Rare case of small intestine bleeding.
BMJ Case Rep. 2019; 12(1) [PubMed] Related Publications
Isolated giant ileal neurofibroma sans neurofibromatosis.
Indian J Cancer. 2018 Jul-Sep; 55(3):301-303 [PubMed] Related Publications
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
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.
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
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.
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
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
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.
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
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.
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
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.
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
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.
Duodenal tumor risk in Lynch syndrome.
Dig Liver Dis. 2019; 51(2):299-303 [PubMed] Related Publications
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.
Endoscopic Papillectomy for Synchronous Major and Minor Duodenal Papilla Neuroendocrine Tumors.
Korean J Gastroenterol. 2018; 72(4):217-221 [PubMed] Related Publications
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
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.
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
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
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.
Ileocecal invagination due to an inflammatory fibroid polyp. A case report.
Ann Ital Chir. 2018; 89:347-349 [PubMed] Related Publications
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
METHODS: We generated mice with epithelial-specific deletion of ribonuclease H2 subunit B (H2b
RESULTS: The 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
Clinicopathological Stratification and Long-term Follow-up of Patients with Periampullary Carcinomas.
Anticancer Res. 2018; 38(9):5379-5386 [PubMed] Related Publications
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.
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
Transjejunostomy Stent Placement in Patients With Malignant Small-Bowel Obstructions.
AJR Am J Roentgenol. 2018; 211(5):1148-1154 [PubMed] Related Publications
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.