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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).

Hashimoto D, Arima K, Chikamoto A, et al.
Limited Resection of the Duodenum for Nonampullary Duodenal Tumors, with Review of the Literature.
Am Surg. 2016; 82(11):1126-1132 [PubMed] Related Publications
The surgical management of duodenal pathology is challenging because of its retroperitoneal position and shared blood supply with the pancreas. We present three types of limited resection of the duodenum for the removal of superficial or small nonampullary duodenal (NADL) lesions, and also a review of the English literature regarding management, such as endoscopic resection and limited duodenal resection. Ten cases underwent limited resections of the duodenum for superficial or small NADL lesions from 2011 to 2015. Pancreas-preserving segmental duodenectomy was performed in three cases, local full-thickness resection was performed in three and transduodenal submucosal dissection was performed in four. One patient experienced pancreatic fistula as a postoperative complication. Postoperative pathological diagnosis were adenoma (n = 2), mucosal adenocarcinomas (n = 5), and neuroendocrine tumor (n = 3). Surgical margin was negative in all cases, and no patient has experienced postoperative recurrence or metastasis. Limited resections of the duodenum were feasible and safe procedures for patients with superficial or small NADL lesions. Laparoscopic surgery may be considered in treatment for these tumors. However, the optimal surgical management for superficial or small nonampullary duodenal lesions remains controversial.

Yang J, Chen S, Chen L, et al.
Chronic diarrhea associated with high serum level of immunoglobulin A and diffuse infiltration of plasma cell in small intestine: A case report.
Medicine (Baltimore). 2017; 96(5):e6057 [PubMed] Free Access to Full Article Related Publications
RATIONALE: Chronic diarrhea in adult patients due to various causes is very common in clinic, but patient suffering with mal-absorption due to immunoproliferative small intestinal disease was rarely reported in China.
PATIENT CONCERNS AND DIAGNOSES: A 35-year-old female presented with more than three years history of chronic diarrhea, rickets, high serum value of immunoglobulin A protein, and anemia. Bone marrow aspiration suggested that the patient was in a sideropenic and megalobastic anemia stage. Duodenal and ileac biopsies revealed atrophy and blunting villi. The bowel lamina propria was infiltrated with slightly increased intraepithelial lymphocytes and mainly with diffuse plasma cells. The following enzyme labeling immunohistochemistry results were strongly positive to alpha-heavy-chain. Computed tomography manifested she had diffuse thickening of small intestine wall. At last a diagnosis of immunoproliferative small intestinal disease was made.
INTERVENTIONS AND OUTCOMES: On the first month, the patient was treated with vitamin D supplements, calcium, magnesium, potassium, iron, folic acid, mecobalamin replacements and microflora probiotics. The patient frequency of water diarrhea alleviated slightly, but her weight loss, anxiety neurosis and other disorders were still severe. After taking with prednisone (40 mg per day, and gradually reduced to the lowest dose) for another month, the symptoms was gradually subsided.
LESSONS: The study shows that immunohistochemical staining for alpha-heavy chain proteins should be completed on small intestine biopsy specimens if the patient is suspected a diagnosis of immunoproliferative small intestinal disease.

Moris D, Spanou E, Sougioultzis S, et al.
Duodenal plexiform fibromyxoma as a cause of obscure upper gastrointestinal bleeding: A case report.
Medicine (Baltimore). 2017; 96(1):e5883 [PubMed] Free Access to Full Article Related Publications
RATIONALE: We are reporting the first-to our knowledge-case of duodenal Plexiform Fibromyxoma causing obscure upper gastrointestinal bleeding.
PATIENT CONCERNS: Plexiform fibromyxoma triggered recurrent upper gastrointestinal bleeding episodes in a 63-year-old man who remained undiagnosed, despite multiple hospitalizations, extensive diagnostic workups and surgical interventions (including gastrectomies), for almost 17 years.
DIAGNOSES-INTERVENTIONS: During hospitalization for the last bleeding episode, an upper gastrointestinal endoscopy revealed an intestinal hemorrhagic nodule. The lesion was deemed unresectable by endoscopic means. An abdominal computerized tomography disclosed no further lesions and surgery was decided. The lesion at operation was found near the edge of the duodenal stump and treated with pancreas-preserving duodenectomy (1st and 2nd portion).
OUTCOMES: Postoperative recovery was mainly uneventful and a 20-month follow-up finds the patient in good health with no need for blood transfusions.Plexiform fibromyxomas stand for a rare and widely unknown mesenchymal entity. Despite the fact that they closely resemble other gastrointestinal tumors, they distinctly vary in clinical management as well as the histopathology. Clinical awareness and further research are compulsory to elucidate its clinical course and prognosis.

Husarić E, Hasukić Š, Hotić N, et al.
Risk factors for post-colectomy adhesive small bowel obstruction.
Acta Med Acad. 2016; 45(2):121-127 [PubMed] Related Publications
OBJECTIVE: The purpose of this study was to assess the risk factors for adhesive small bowel obstruction (SBO) following colectomy for colorectal cancer.
PATIENTS AND METHODS: In this retrospective study we analyzed 284 patients who underwent surgery for colorectal cancer at the Department of Surgery University Clinical Center Tuzla in the period from 1st January 2009 until 31st December 2014. All patients underwent open colectomy. The length of follow up was from 6 months to 6 years (median follow up 3 years and 6 months). The study included all patients who underwent surgery due to colon cancer. The study excluded patients with postoperative small bowel obstruction after colon cancer surgery with different comorbidities.
RESULTS: In the analyzed sample of 284 patients, a small bowel obstruction occurred in 13.7% patients after surgery for colon cancer. The highest correlation of risk factors and the occurrence of postoperative small bowel obstruction after colectomy for colorectal cancer in multivariate regression analysis was found to be for Tumor-Node-Metastasis ≥3 (or =3.680), and postoperative complications (or =30.683).
CONCLUSIONS: Postoperative SBO have many causes, but in this study the highest risk factors were the Tumor-Node-Metastasis ≥3, and postoperative complications.

Ethun CG, Postlewait LM, Baptiste GG, et al.
Small bowel neuroendocrine tumors: A critical analysis of diagnostic work-up and operative approach.
J Surg Oncol. 2016; 114(6):671-676 [PubMed] Related Publications
BACKGROUND: Small bowel neuroendocrine tumors (SB-NETs) are often small, multifocal, difficult to localize preoperatively, and can be overlooked during operative exploration. The optimal work-up and operative approach is unknown.
METHODS: Patients who underwent resection of SB-NETs at a single-institution from 2000 to 2014 were included. Primary aim was to describe the diagnostic work-up and compare minimally invasive (MIS) to open resection.
RESULTS: Ninety-three patients underwent resection for SB-NETs. About 71% were symptomatic and on average underwent three diagnostic tests: 45% had octreoscans (85% diagnostic yield); 11% had SB-enteroscopy (10% yield); 19% had capsule endoscopy (83% yield, but identified the correct tumor number in only 21%). About 27 pts underwent MIS versus 66 open. MIS pts were younger (56 vs. 61 yrs; P = 0.035), and less likely to have obstruction (4% vs. 24%; P = 0.019) and metastases (19% vs. 44%; P = 0.038). Compared to open, MIS had smaller (1.7 vs. 2.4 cm; P = 0.03) and fewer tumors resected (2 vs. 5; P = 0.049), but similar LN yield (13 vs. 12; P = 0.7). In non-metastatic, curative-intent resections, MIS still resected fewer tumors compared to open (1.5 vs. 4; P = 0.034).
CONCLUSION: Capsule endoscopy may be better than small bowel enteroscopy at identifying occult SB-NETs, but may underestimate tumor burden. While MIS may be appropriate in select patients, recognizing the limitations of preoperative evaluation is critical for these tumors, as heightened operative vigilance is often required. J. Surg. Oncol. 2016;114:671-676. © 2016 Wiley Periodicals, Inc.

Fazio N, Milione M
Heterogeneity of grade 3 gastroenteropancreatic neuroendocrine carcinomas: New insights and treatment implications.
Cancer Treat Rev. 2016; 50:61-67 [PubMed] Related Publications
Gastroenteropancreatic (GEP) neuroendocrine neoplasms (NENs) are currently classified as grade (G) 1, G2 and G3, in accordance with the 2010 WHO classification. G1 and G2 are named neuroendocrine tumors (NETs) whereas G3 neuroendocrine carcinomas (NECs). While advanced G1 and G2 are usually treated with several different therapies, including somatostatin analogs, chemotherapy, interferon, molecular targeted agents, peptide receptor radionuclide therapy (PRRT) and liver-directed treatments, advanced G3 NECs are usually treated with a platinum-etoposide chemotherapy, trusting their clinical homogeneity is similar to that of small cell lung cancer. However, over the last years a number of reports suggested that 2010 WHO G3 GEP NECs are more heterogeneous than expected. Therefore, we critically reviewed the literature about this topic and reported pathological and clinical considerations on 2010 WHO G3 GEP NEC category proposing new sub-categories. Over the last five years, six studies specifically investigating large series of G3 GEP NECs have been published, including around 800 patients. Tumor morphology and Ki-67 Labeling Index (that will be mentioned as Ki-67 in this manuscript) combination has been reported as a tool to define two or even three subgroups of this category with different prognosis and potentially different therapeutic approach. Prospective trials are warranted to investigate if several types of therapy other than the platinum/etoposide chemotherapy can be effective in well differentiated GEP NEN with 21-55% Ki-67 and alkylating-based chemotherapy in poorly differentiated GEP NEN with 21-55% Ki-67.

Takahashi T, Maruyama Y, Saitoh M, et al.
Synchronous Occurrence of Diffuse Large B-cell Lymphoma of the Duodenum and Gastrointestinal Stromal Tumor of the Ileum in a Patient with Immune Thrombocytopenic Purpura.
Intern Med. 2016; 55(20):2951-2956 [PubMed] Free Access to Full Article Related Publications
A 64 year-old woman with steroid-dependent immune thrombocytopenia developed anemia. Esophagogastroduodenoscopy revealed the presence of a tumor, which was diagnosed to be diffuse large B-cell lymphoma, in the second portion of the duodenum. (18)F-fluorodeoxy glucose positron emission tomography showed an increased uptake mass in the pelvic cavity as well as in the duodenum. Though the duodenal tumor disappeared after 4 cycles of chemotherapy, the pelvic mass did not shrink in size. As a result, laparoscopic resection of the pelvic tumor was performed and the tumor was histologically diagnosed to be a gastrointestinal stromal tumor. Subsequently, the patient was treated with 2 more cycles of the chemotherapy. Eventually, thrombocytopenia completely resolved.

McCusker S, Trangucci J, Frederick W, et al.
Primary Myeloid Sarcoma of the Small Intestine: Case Report and Literature Review.
Conn Med. 2016 Jun-Jul; 80(6):349-52 [PubMed] Related Publications
Primary myeloid sarcoma is a rare extramedullary manifestation of acute myeloid leukemia (AML). Typically, myeloid sarcoma presents after a diagnosis of AML or other myeloproliferative disorder. However, primary myeloid sarcoma may present without any preexisting condition, thereby making it extremely difficult to diagnose. We discuss a case of a 22-year-old female who was misdiagnosed initially with acute appendicitis and underwent an appendectomy. Postoperatively, she continued to be symptomatic and eventually developed small bowel obstruction. Diagnostic laparoscopy revealed multiple small bowel masses as well as diffuse abdominal and pelvic lymphadenopathy. After extensive pathological review and additional workup consisting of immunohistochemistry and molecular studies, the correct diagnosis of myeloid sarcoma was made. This review will discuss the presentation, diagnosis, management, and prognosis of primary myeloid sarcoma.

Chabowski M, Szymanska-Chabowska A, Dorobisz T, et al.
A massive bleeding from a gastrointestinal stromal tumor of a Meckel's diverticulum.
Srp Arh Celok Lek. 2016 Mar-Apr; 144(3-4):219-21 [PubMed] Related Publications
INTRODUCTION: Meckel's diverticulum is the most common congenital anomaly of the gastro intestinal tract, present in about 2% of population.
CASE OUTLINE: The article presents the case of a 44-year-old otherwise healthy man with anemia, who was diagnosed lower gastrointestinal bleeding. An abdominal CT scan revealed a clearly demarcated solid tumor in hypogastric region, measuring 65 x 45 mm. A laparotomy through lower midline incision was performed. A surgical resection of a lesion of a Meckel's diverticulum was carried out and a final diagnosis of gastrointestinal stromal tumor was made.The patient made an uneventful recovery.
CONCLUSION: The preoperative diagnosis of a complicated Meckel's diverticulum may be challenging. CT is usually an adequate method to diagnose tumors arising from Meckel's diverticulum.

Shirai Y, Ohki T, Yamamoto M
Application of the clip method, using thread, for duodenal endoscopic mucosal resection.
BMJ Case Rep. 2016; 2016 [PubMed] Related Publications
Endoscopic mucosal resection (EMR) of a duodenal tumour is associated with a risk of loss of the resected specimen resulting from air introduction, peristaltic motion of the intestines, insertion of the retrieval device or endoscopic operation, or in cases where the specimen is too large to pass the pylorus. There is a high possibility of losing the tumour if the resected tumour is passed through the second portion of the duodenum. Retrieving a specimen after EMR is essential to obtain a pathological finding. The clip method using thread has been useful for endoscopic submucosal dissection of the oesophagus and stomach. We report the effectiveness of the clip method using thread during duodenal EMR.

Konishi T, Ishida H, Ueno H, et al.
Feasibility of laparoscopic total proctocolectomy with ileal pouch-anal anastomosis and total colectomy with ileorectal anastomosis for familial adenomatous polyposis: results of a nationwide multicenter study.
Int J Clin Oncol. 2016; 21(5):953-961 [PubMed] Related Publications
BACKGROUND: Data supporting the safety and feasibility of laparoscopic total proctocolectomy with ileal pouch-anal anastomosis (TPC-IPAA) and total colectomy with ileorectal anastomosis (TC-IRA) for patients with familial adenomatous polyposis (FAP) are limited. The aim of this study was to clarify the feasibility and morbidity of laparoscopic TPC-IPAA and TC-IRA for patients with FAP, using a large Japanese multicenter dataset.
METHODS: Data on 256 patients with FAP who underwent TPC-IPAA (n = 171) or TC-IRA (n = 85) at 23 institutions between the years 2000 and 2012 were collected. Short- and long-term clinical outcomes were compared between laparoscopic and open approaches for each procedure.
RESULTS: Among the 256 patients with FAP, a total of 126 patients underwent laparoscopic surgery, consisting of 74 laparoscopic TPC-IPAAs and 52 laparoscopic TC-IRAs. The proportion of the FAP patients who underwent laparoscopic surgery increased during the study period, reaching 79 % of all TPC-IPAAs and 82 % of all TC-IRAs in the final two years covered by the data. In both TPC-IPAA and TC-IRA, the laparoscopic approach was associated with a longer operative duration but a similarly low postoperative morbidity and comparably adequate anal function compared with the open approach. The overall survival and the incidence of desmoid tumor were also comparable between the laparoscopic and open approaches in both procedures.
CONCLUSIONS: Laparoscopic TPC-IPAA and TC-IRA are both feasible options-with low rates of morbidity, good functional outcomes, and excellent overall survival rates-in patients with FAP. Since the data indicate that laparoscopic TPC-IPAA and TC-IRA are feasible, they also support the recent increase in laparoscopic surgery for patients with FAP in Japan.

Oeconomopoulou A, de Verney Y, Kanavaki K, et al.
Inflammatory myofibroblastic tumor of the small intestine mimicking acute appendicitis: a case report and review of the literature.
J Med Case Rep. 2016; 10:100 [PubMed] Free Access to Full Article Related Publications
BACKGROUND: Inflammatory myofibroblastic tumor is a rare tumor of a borderline malignancy. Although it is commonly seen in children, the number of childhood cases in the current literature is limited. The lung is the most commonly affected location. However, cases that have been documented in the mesentery-omentum have mostly been located in the mesentery of the small bowel and not in the antimesenteric edge as in our patient.
CASE PRESENTATION: A 6-year-old Greek boy was referred to our hospital with acute abdominal pain mimicking appendicitis. Ultrasound and computed tomography revealed a solid mass in the abdomen. The patient underwent laparoscopic resection of the tumor, and histopathology and immunohistochemical analysis favored an omental-mesenteric myxoid hamartoma, which is a variant of an inflammatory myofibroblastic tumor. The patient's postoperative course was uneventful, and he has been asymptomatic during follow-up.
CONCLUSIONS: Inflammatory myofibroblastic tumor of the small intestine is a rare, benign neoplasm in children that should be considered as a possible cause of acute abdomen. A precise diagnosis can be made on the basis of histologic findings. Surgical excision is the treatment of choice.

Tan QT, Teo JY, Ahmed SS, Chung AY
A case of small bowel metastasis from spinal Ewing sarcoma causing intussusception in an adult female.
World J Surg Oncol. 2016; 14:109 [PubMed] Free Access to Full Article Related Publications
BACKGROUND: Ewing sarcomas are highly aggressive malignant tumours occurring predominantly in the long bones of the extremities in children and young adults. About 20 % of patients will present with metastases at diagnosis with the commonest sites being the lungs, bone and bone marrow. Cases of primary small bowel Ewing sarcomas have been described but are nonetheless exceedingly rare, even more so cases of metastasis to the small bowel.
CASE PRESENTATION: We describe a case of vertebral Ewing sarcoma in a 44 year-old female which metastasized to the jejunum causing intussusception.
CONCLUSIONS: Ewing's sarcoma is highly aggressive and presence of metastases, overt or subclinical, is thought to be present in almost all patients at diagnosis. As evidenced by our patient, metastatic disease can progress rapidly to cause further complications and confer a poorer survival. The possibility of metastasis, no matter how rare or unlikely the site is, should be considered and actively investigated to expedite treatment of the primary disease.

Amr B, Shahtahmassebi G, Briggs CD, et al.
Assessment of the effect of interval from presentation to surgery on outcome in patients with peri-ampullary malignancy.
HPB (Oxford). 2016; 18(4):354-9 [PubMed] Free Access to Full Article Related Publications
BACKGROUND: Delay between diagnosis of peri-ampullary cancer (PC) and surgery may allow tumour progression and affect outcome. The aim of this study was to explore associations of interval to surgery (IS) with pathological outcomes and survival in patients with PC.
METHOD: A database review of all patients undergoing surgery between 2006 and 2014 was undertaken. IS was measured from diagnosis by imaging. Potential association between IS and survival was measured using Cox regression analysis, and between IS and pathological outcome with multivariate logistic analysis.
RESULTS: 388 patients underwent surgery. The median IS was 49 days (1-551 days), and was not associated with any of the evaluated outcomes in patients with pancreatic (149) or distal bile duct (46) cancer. For patients with ampullary cancer (71) longer IS was associated with improved survival, with median survival of 27.5 months for patients waiting ≤ median IS (35) and 38.3 months for patients waiting > median IS (36) for surgery (p = 0.041). A higher rate of margin positivity (31.4%) was also noted among patients who waited less than the median IS compared to those waiting longer than this interval (11.4%) (p = 0.032).
CONCLUSION: For patients with ampullary cancer there is a paradoxical improvement in outcome among those with a longer IS, which may be explained by progression to inoperability of more aggressive lesions.

Di Leo A, Nesi G, Principi M, et al.
Epithelial turnover in duodenal familial adenomatous polyposis: A possible role for estrogen receptors?
World J Gastroenterol. 2016; 22(11):3202-11 [PubMed] Free Access to Full Article Related Publications
AIM: To investigate estrogen receptors expression in duodenal familial adenomatous polyposis (FAP) and any relationship with epithelial proliferation/apoptosis markers.
METHODS: Twenty-two patients affected by FAP undergoing duodenal resection for malignancies were recruited. Controls were 15 healthy subjects undergoing endoscopy for dyspeptic symptoms. ER-α, ER-α, Ki-67, TUNEL and caspase 3 expression (labeling index: percentage of positive cells) were evaluated by immunohistochemistry or immunofluorescence and examined by light or confocal microscopy. Samples were assigned to four groups: normal tissue, low (LGD) and high-grade dysplasia (HGD), adenocarcinoma (AC). One-way analysis of variance, corrected by Bonferroni's test, and Pearson's correlation test were applied for statistical analysis.
RESULTS: ER-beta showed a progressive decline: normal tissue (23.5 ± 4.9), LGD (21.1 ± 4.8), HGD (9.3 ± 3.5), AC (7.1 ± 3.1). The normal tissue of FAP subjects expressed ER-beta like the controls (23.9 ± 6.2). Conversely, ER-α showed a progressive increase from normal tissue (24.8 ± 5.6) to AC (52.0 ± 8.2); the expression in normal tissue was similar to controls (22.5 ± 5.3). Ki67 demonstrated a statistically significant progressive increase at each disease stage up to AC. TUNEL did not reveal differences between controls and normal tissue of FAP subjects, but progressive decreases were observed in LGD, through HGD to AC. Pearson's correlation test showed a direct relationship between ER-β and TUNEL LI (r = 0.8088, P < 0.0001). Conversely, ER-α was inversely correlated with TUNEL LI (r = - 0.7257, P < 0.0001). The co-expression of ER-β and caspase 3 declined progressively from normal to neoplastic tissue.
CONCLUSION: This study confirmed that ER-β is strongly decreased in duodenal FAP carcinomas, declining in a multiple step fashion, thereby suggesting a putative anti-carcinogenic effect. ER-α showed the opposite trend. ER-β/caspase 3 co-expression suggests this hormone's possible involvement in apoptosis. Hormonal influences in FAP duodenal tumorigenesis, and modulation of these as a possible chemoprevention strategy, may be a promising approach.

Giannetti A, Randisi P, Stumpo M, Coratti F
Diagnosis of one small bowel tumor: the role of conventional ultrasound and elastography.
J Ultrasound. 2016; 19(1):57-60 [PubMed] Free Access to Full Article Related Publications
Neuroendocrine tumors are the most common primary tumors of the small bowel, but diagnosis is usually delayed as the lesions are small and symptoms are mild or nonspecific. Diagnosis of this disease is currently based on radiologic or endoscopic findings. Ultrasound (US) is used as a first-line examination in patients with abdominal symptoms, as this technique permits an overall view of the gastrointestinal tract providing diagnosis of inflammatory and/or neoplastic disorders in a large proportion of patients. The aim of this case report is to describe the feasibility and diagnostic accuracy of elastography in the diagnosis of a tumor of the ileum. The patient underwent conventional gray-scale US and elastography before surgery and these examinations were repeated postoperatively on the surgical specimen confirming preoperative outcome.

Komori S, Kawai M, Nitta T, et al.
A case of carcinoma of the papilla of Vater in a young man after subtotal colectomy for familial adenomatous polyposis.
World J Surg Oncol. 2016; 14(1):47 [PubMed] Free Access to Full Article Related Publications
BACKGROUND: Carcinoma and adenoma of the duodenum, including the papilla of Vater, are problematic diseases in patients with familial adenomatous polyposis (FAP).
CASE PRESENTATION: A 36-year-old man underwent a periodic medical examination for early colon cancer originating from FAP for which laparoscopic-assisted subtotal colectomy with a J-shaped ileal pouch-rectal anastomosis was performed 3 years earlier. A tumor was detected at the papilla of Vater along with elevation of total bilirubin and hepatobiliary enzymes. Although cytology did not determine the tumor to be an adenocarcinoma, we suspected adenocarcinoma due to its hypervascularity shown by contrast-enhanced computed tomography. Pylorus-preserving pancreaticoduodenectomy with modified Imanaga reconstruction and regional lymph node dissection (D2) was performed. The pathological study showed that the tumor was a papillary and moderately differentiated tubular adenocarcinoma. The patient is currently in good health without recurrence, weight loss, or severe diarrhea at 12 months after surgery.
CONCLUSIONS: Awareness of biliary-pancreatic symptoms and periodic gastroduodenoscopy might contribute both to the early detection of duodenal or periampullary polyps and cancer and to the radical treatment of FAP. Modified Imanaga reconstruction has the potential to become one of the more effective procedures for providing good quality of life to FAP patients with duodenal or periampullary cancer.

Li ZR, Li DJ, Xie XP, Liu Y
Segmental Duodenectomy in Surgical Therapy of Tumor of the Third and Fourth Portions of Duodenum.
Hepatogastroenterology. 2015; 62(140):913-8 [PubMed] Related Publications
BACKGROUND/AIMS: To analyze our experience of segmental duodenectomy for tumors located at the third and fourth portion of the duodenum and attempt to explore the security and feasibility of this surgical procedure.
METHODOLOGY: A retrospective cohort study of five patients who underwent segmental duodenectomy in our hospital, medical records were analyzed in this study.
RESULTS: The initial symptoms in five patients are not specific. Five were surgically treated by segmental resection. All patients without postoperative anastomotic leakage, the gastroparesis and anastomotic stenosis each appeared in a case and all recovered after supportive care. Pathological examination showed: 3 cases of stromal tumor, 1 :ases of lymphangioma, diffuse large B-cell lymphoma. Postoperative gastrointestinal bleeding does not appear in the lymphangioma,two cases of high risk group of stromal tumor patients received targeting therapy with Imatinib Mesylate for 2 years after resection, the patient with lymphoma administer postoperative adjuvant chemotherapy. All patients are still alive and the lymphoma patient developed postoperative local recurrence after approximately six months.
CONCLUSIONS: Segmental duodenectomy is a reliable and curative option for most duodenal benign tumor and stromal tumor located at the third and fourth portion. It is also applicable to some malignant tumor.

Jun SY, Kim M, Jin Gu M, et al.
Clinicopathologic and prognostic associations of KRAS and BRAF mutations in small intestinal adenocarcinoma.
Mod Pathol. 2016; 29(4):402-15 [PubMed] Related Publications
Activating KRAS and/or BRAF mutations have been identified as predictors of resistance to anti-epidermal growth factor receptor (EGFR) chemotherapy in colorectal cancer. But the status of KRAS and BRAF mutations and their clinicopathologic and prognostic significance has not been extensively evaluated in small intestinal adenocarcinomas. In this work, the KRAS and BRAF genes in 190 surgically resected small intestinal adenocarcinoma cases were sequenced and their association with various clinicopathologic variables, including survival of the patients, was analyzed. KRAS or BRAF mutations were observed in 63 (33%) cases. Sixty-one cases had KRAS mutations and 2 had BRAF mutations and the two types of mutation were mutually exclusive. The majority of KRAS mutations were G>A transition (43/61 cases, 71%) or p.G12D (31/61 cases, 51%). The patients with mutant KRAS tended to have higher pT classifications (P=0.034) and more frequent pancreatic invasion (P=0.020) than those with wild-type KRAS. Multivariate logistic regression analysis showed that certain mutated KRAS subtypes (G>A transitions and G12D mutations) were significantly correlated with higher pT classification (P=0.015 and 0.004, respectively) than wild-type KRAS and other KRAS mutations. The patients with KRAS or BRAF mutation had a tendency to shorter overall survival than those with wild-type KRAS and BRAF (P=0.148), but subgroup analysis demonstrated the patients with KRAS mutations showed worse survival (median, 46.0 months; P=0.046) than those with wild-type KRAS (85.4 months) in lower pT classification (pT1-pT3) group. In summary, KRAS and, infrequently, BRAF mutations are observed in a subset of small intestinal adenocarcinomas, and are associated with higher pT classification and more frequent pancreatic invasion. KRAS mutation is a poor prognostic predictor in patients with lower pT classification tumors. Anti-EGFR targeted therapy could be applied to about two-thirds of small intestinal adenocarcinoma patients, namely those with wild-type KRAS and BRAF if they have metastatic disease, similar to colorectal cancer patients.

Meshikhes AW, Joudeh AA
Late metastatic colon cancer masquerading as primary jejunal carcinoma.
Ann R Coll Surg Engl. 2016; 98(3):e49-51 [PubMed] Free Access to Full Article Related Publications
Metastasis to the small bowel from a previously resected colorectal cancer is rare and may erroneously be diagnosed as a primary small bowel carcinoma. It usually occurs several years after the primary resection. We present the case of a 67-year-old man who had undergone left hemicolectomy for colon cancer 3 years earlier and returned with subacute small bowel obstruction. This was initially thought, based on preoperative radiological findings and normal colonoscopic examination, to be due a primary jejunal cancer. Even at surgery, the lesion convincingly appeared as an obstructing primary small bowel carcinoma. However, the histology of the resected small bowel revealed metastatic colon cancer. This rare and an unusual metastatic occurrence some years after the primary resection is described and reviewed.

Mendonça EQ, Bernardo WM, Moura EG, et al.
Endoscopic versus surgical treatment of ampullary adenomas: a systematic review and meta-analysis.
Clinics (Sao Paulo). 2016; 71(1):28-35 [PubMed] Free Access to Full Article Related Publications
The aim of this study is to address the outcomes of endoscopic resection compared with surgery in the treatment of ampullary adenomas. A systematic review and meta-analysis were performed according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) recommendations. For this purpose, the Medline, Embase, Cochrane, Literatura Latino-Americana e do Caribe em Ciências da Saúde (LILACS), Scopus and Cumulative Index to Nursing and Allied Health Literature (CINAHL) databases were scanned. Studies included patients with ampullary adenomas and data considering endoscopic treatment compared with surgery. The entire analysis was based on a fixed-effects model. Five retrospective cohort studies were selected (466 patients). All five studies (466 patients) had complete primary resection data available and showed a difference that favored surgical treatment (risk difference [RD] = -0.24, 95% confidence interval [CI] = -0.44 to -0.04). Primary success data were identified in all five studies as well. Analysis showed that the surgical approach outperformed endoscopic treatment for this outcome (RD = -0.37, 95% CI = -0.50 to -0.24). Recurrence data were found in all studies (466 patients), with a benefit indicated for surgical treatment (RD = 0.10, 95% CI = -0.01 to 0.19). Three studies (252 patients) presented complication data, but analysis showed no difference between the approaches for this parameter (RD = -0.15, 95% CI = -0.53 to 0.23). Considering complete primary resection, primary success and recurrence outcomes, the surgical approach achieves significantly better results. Regarding complication data, this systematic review concludes that rates are not significantly different.

Jahagirdar V, Kamal A, Steeds R, Ayuk J
Metastatic small bowel neuroendocrine tumour with bilateral carcinoid heart disease.
BMJ Case Rep. 2016; 2016 [PubMed] Related Publications
A 63-year-old woman was admitted with a year's history of exertional breathlessness, anxiety attacks, syncopal episodes, diarrhoea, fatigue, reduced appetite, 2 stones weight loss, and flushing affecting her face and trunk. Investigations revealed raised urine 5-hydroxy indole acetic acid (5-HIAA) and chromogranin A. CT scan demonstrated extensive soft tissue encasing the major vessels intra-abdominally, and a retroperitoneal mass. (111)In-octreotide single-photon emission CT (SPECT CT) showed increased focal activity in the mediastinum, retroperitoneum and mesenteric lymph nodes. Para aortic lymph node biopsy confirmed the diagnosis of metastatic well-differentiated grade 1 gastrointestinal neuroendocrine tumour (NET). Extensive cardiac investigations confirmed severe mitral regurgitation, moderate aortic and tricuspid regurgitation, and mild pulmonary regurgitation. The patient's symptoms of flushing and diarrhoea were controlled with octreotide LAR, and she underwent mechanical aortic and mitral valve replacement. Following discharge from surgery, she went on to develop hydronephrosis and urosepsis, followed by infective endocarditis, resulting in recurrent admissions, and eventually passed away in her sleep nearly 14 months after her initial diagnosis.

Liu G, Yan G, Kuang S, Wang Y
Detection of small bowel tumor based on multi-scale curvelet analysis and fractal technology in capsule endoscopy.
Comput Biol Med. 2016; 70:131-8 [PubMed] Related Publications
Wireless capsule endoscopy (WCE) has been a revolutionary technique to noninvasively inspect gastrointestinal (GI) tract diseases, especially small bowel tumor. However, it is a tedious task for physicians to examine captured images. To develop a computer-aid diagnosis tool for relieving the huge burden of physicians, the intestinal video data from 89 clinical patients with the indications of potential tumors was analyzed. Out of the 89 patients, 15(16.8%) were diagnosed with small bowel tumor. A novel set of textural features that integrate multi-scale curvelet and fractal technology were proposed to distinguish normal images from tumor images. The second order textural descriptors as well as higher order moments between different color channels were computed from images synthesized by the inverse curvelet transform of the selected scales. Then, a classification approach based on support vector machine (SVM) and genetic algorithm (GA) was further employed to select the optimal feature set and classify the real small bowel images. Extensive comparison experiments validate that the proposed automatic diagnosis scheme achieves a promising tumor classification performance of 97.8% sensitivity and 96.7% specificity in the selected images from our clinical data.

Weaver JL, Barnett RE, Patterson DE, et al.
Large-bowel disease presenting as small-bowel obstruction is associated with a poor prognosis.
Am J Emerg Med. 2016; 34(3):477-9 [PubMed] Related Publications
INTRODUCTION: Small-bowel obstruction (SBO) is a common cause of admission to the surgical service. On rare occasions, a diagnosed SBO is actually due to large-bowel pathology combined with an incompetent ileocecal valve. The purpose of this study was to investigate this phenomenon.
METHODS: We performed a retrospective medical record review of patients that were admitted with a diagnosis of SBO at University of Louisville hospital and the Veterans Affairs hospitals in Louisville, KY, from 2006 until 2014.
RESULTS: A total of 498 patients were admitted with SBO during this time period. Forty-one patients were found to have an underlying large-bowel disease. The most common large-bowel pathologies included malignancy (51%), inflammation (15%), and infection (15%). Fifteen (43%) of these patients died during admission; 93% of these were due to either their bowel obstruction or the underlying disease state. This was significantly higher than the general population (9.4% mortality, 6% due to underlying disease).
CONCLUSIONS: Patients that present with SBO due to a large-bowel source have a much higher mortality rate than those that present with other causes. Rapid identification of these patients will allow for more timely and appropriate treatment.

Williamsson C, Wennerblom J, Tingstedt B, Jönsson C
A wait-and-see strategy with subsequent self-expanding metal stent on demand is superior to prophylactic bypass surgery for unresectable periampullary cancer.
HPB (Oxford). 2016; 18(1):107-12 [PubMed] Free Access to Full Article Related Publications
BACKGROUND: A patient with unresectable periampullary malignancy found at laparotomy has traditionally received a prophylactic double bypass (biliary and duodenal), associated with considerable morbidity. With modern endoscopic treatments, surgical bypass has become questionable. This study aims to compare the two strategies. Sahlgrenska University Hospital (SU) performs a double bypass (DoB) routinely, and Skåne University Hospital Lund (SUL) secures biliary drainage endoscopically and treats only symptomatic duodenal obstruction (Wait and See, WaS).
METHOD: Between 2004 and 2013, 73 patients from SU and 70 from SUL were retrospectively identified. Demographics, tumour-related factors and postoperative outcomes during the remaining lifetime were noted.
RESULTS: The DoB group had significantly more complications (67% vs. 31%, p = 0.00002) and longer hospital stay (14 vs. 8 days, p = 0.001) than the WaS-group. The two groups had similar proportion of patients in need of readmission. The DoB patients and the WaS patients with metallic biliary stents were comparable regarding their need of re-interventions and hospitalisation due to biliary obstruction. Surgical duodenal bypass did not prevent future duodenal obstructions.
CONCLUSION: Patients with unresectable periampullary malignancies can safely be managed with endoscopic drainage on demand and with lower morbidity and shorter hospital stay than with surgical prophylactic bypass.

Ryska M, Hrabal P
Malignant tumours of the duodenum.
Rozhl Chir. 2015; 94(12):497-503 [PubMed] Related Publications
No comprehensive knowledge of duodenal tumours exists in the current literature; individual types of malignant tumours may be described within malignancies of the small bowel, sets of case reports, or individual cases. Ampullary carcinomas are the exception and they are detailed in the current WHO histological classification of tumours of digestive system. Neither national nor international literature sources provide a comprehensive review of their therapy. The situation is similar when searching for surgical procedures. Resection procedures on the duodenum should thus be performed in specialized centres with sufficient experience with hepato-pancreato-biliary surgery.

Schønnemann KR, Mortensen MB, Krogh M, et al.
Trends in upper gastro-intestinal cancer among the elderly in Denmark, 1980-2012.
Acta Oncol. 2016; 55 Suppl 1:23-8 [PubMed] Related Publications
BACKGROUND: Upper gastro-intestinal cancer (UGIC) includes malignancies in esophagus, stomach and small intestine, and represents some of the most frequent malignancies worldwide. The aim of the present analysis was to describe incidence, mortality and survival in UGIC patients in Denmark from 1980 to 2012 according to differences in age and time periods.
MATERIAL AND METHODS: UGIC was defined as ICD-10 codes C15-C17. Data derived from the NORDCAN database with comparable data on cancer incidence mortality, prevalence and relative survival in the Nordic countries, where the Danish data were delivered from the Danish Cancer Registry and the Danish Cause of Death Registry with follow-up for death or emigration until the end of 2013.
RESULTS: The proportion of male patients over the age of 70 years diagnosed with esophageal cancer was constant over time (around 42%) but increased in females to 49% in 2012. Incidence rates increased with time and continued to rise in all ages. Mortality rates were clearly separated by age groups with increasing mortality rates by increasing age group for both sexes. Relative survival increased slowly over time in all age groups. The proportion of older male and female patients with stomach cancer increased to 50% and 54%, respectively, in 2012. Incidence rates decreased steadily with time, especially from 1980 to 1990 but continued to decrease in all age groups. Mortality rates decreased considerably from 1980 to 90 and have been almost constant during the last decade for both women and men. Relative survival increased modest over time in both genders and all age groups. In 2012, only 1471 persons were alive after a diagnosis of stomach cancer.
CONCLUSION: There is a need for clinical trials focusing on patients over the age of 70 years with co-existing comorbidity.

Williamson JM, Stevens M, Mahon D
Metachronous small bowel metastasis from a mixed Müllerian mesodermal tumour.
Ann R Coll Surg Engl. 2016; 98(2):e26-8 [PubMed] Free Access to Full Article Related Publications
A mixed Müllerian mesodermal tumour (MMMT) is a rare aggressive carcinosarcoma. Metastatic progression is uncommon, and occurs via haematological, lymphatic and intraperitoneal spread. Although the latter is seen most frequently, the small intestine seems to be relatively preserved from disease progression with only one reported case of synchronous involvement. We report a case of metachronous MMMT involvement of the small bowel presenting with subacute obstruction that was successfully resected at operation.

Aronson M, Gallinger S, Cohen Z, et al.
Gastrointestinal Findings in the Largest Series of Patients With Hereditary Biallelic Mismatch Repair Deficiency Syndrome: Report from the International Consortium.
Am J Gastroenterol. 2016; 111(2):275-84 [PubMed] Related Publications
OBJECTIVES: Hereditary biallelic mismatch repair deficiency (BMMRD) is caused by biallelic mutations in the mismatch repair (MMR) genes and manifests features of neurofibromatosis type 1, gastrointestinal (GI) polyposis, and GI, brain, and hematological cancers. This is the first study to characterize the GI phenotype in BMMRD using both retrospective and prospective surveillance data.
METHODS: The International BMMRD Consortium was created to collect information on BMMRD families referred from around the world. All patients had germline biallelic MMR mutations or lack of MMR protein staining in normal and tumor tissue. GI screening data were obtained through medical records with annual updates.
RESULTS: Thirty-five individuals from seven countries were identified with BMMRD. GI data were available on 24 of 33 individuals (73%) of screening age, totaling 53 person-years. The youngest age of colonic adenomas was 7, and small bowel adenoma was 11. Eight patients had 19 colorectal adenocarcinomas (CRC; median age 16.7 years, range 8-25), and 11 of 18 (61%) CRC were distal to the splenic flexure. Eleven patients had 15 colorectal surgeries (median 14 years, range 9-25). Four patients had five small bowel adenocarcinomas (SBC; median 18 years, range 11-33). Two CRC and two SBC were detected during surveillance within 6-11 months and 9-16 months, respectively, of last consecutive endoscopy. No patient undergoing surveillance died of a GI malignancy. Familial clustering of GI cancer was observed.
CONCLUSIONS: The prevalence and penetrance of GI neoplasia in children with BMMRD is high, with rapid development of carcinoma. Colorectal and small bowel surveillance should commence at ages 3-5 and 8 years, respectively.

Liu Y, Ishibashi H, Takeshita K, et al.
Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy for Peritoneal Dissemination from Small Bowel Malignancy: Results from a Single Specialized Center.
Ann Surg Oncol. 2016; 23(5):1625-31 [PubMed] Related Publications
BACKGROUND: Peritoneal dissemination is a frequent pattern of recurrence and metastasis of small bowel malignancy (SBM). However, the survival of patients with peritoneal dissemination from SBM is not clear, and there is no consensus on the treatment for it.
PATIENTS AND METHODS: A total of 31 selected patients with peritoneal dissemination from SBM were treated by cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) between January 2006 and January 2014. The major focus of this study was overall survival, as well as morbidity and mortality.
RESULTS: Twenty-five patients had small bowel adenocarcinoma (SBA), and six patients had non-adenocarcinoma SBM. HIPEC was performed on 21 patients, and 21 patients received complete cytoreduction. There was no treatment-related mortality, and eight (25.8 %) patients had grade 3-4 complications. Until the latest follow-up, the median survival of 31 patients after CRS and HIPEC was 36 months (range 5-95 months), and the median survival after diagnosis was 51 months (range 18-101 months). For 25 patients with peritoneal carcinomatosis from SBA, the median survival after CRS and HIPEC was 36 months (range 6-95 months), and the median survival after diagnosis was 50 months (range 18-101 months). Multivariate analysis revealed that peritoneal cancer index <15 (p = 0.009) and HIPEC (p < 0.001) were independent predictors of better survival in patients with peritoneal dissemination from SBM treated by CRS and HIPEC.
CONCLUSIONS: Until more data become available, a reasonable strategy for the treatment of SBM is CRS and HIPEC. It can be applied with acceptable safety in selected patients with peritoneal dissemination from SBM.

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