Small bowel tumors(SBTs)have been increasingly diagnosed in recent decades.The pathogenesis of this increment is largely unknown,but advances in radiological and endoscopic methods facilitate the improvement of the di...Small bowel tumors(SBTs)have been increasingly diagnosed in recent decades.The pathogenesis of this increment is largely unknown,but advances in radiological and endoscopic methods facilitate the improvement of the diagnosis.Capsule endoscopy(CE)and device-assisted enteroscopy(DAE)allow the clinician to assess the entire small bowel in the search for suspicious lesions,or a cause of symptoms.In this review,we discuss the role of enteroscopy,techniques and strategies in the diagnosis and management of SBTs,and a brief description of the most common tumors.展开更多
Objective: Small intestinal tumors (SBTs) were difficult to be discovered before surgery. The emergence of dou- ble-balloon enteroscopy (DBE) and capsule endoscopy (CE) have greatly improved the evaluation of S...Objective: Small intestinal tumors (SBTs) were difficult to be discovered before surgery. The emergence of dou- ble-balloon enteroscopy (DBE) and capsule endoscopy (CE) have greatly improved the evaluation of SBTs and facilitated a more precise preoperative diagnosis. Yet little data exist in China regarding clinical efficacy of DBE and clinical characteristics of SBTs. The aim of our study was to clarify the clinical characteristics of SBTs and analyze the efficacy of DBE examination in the detection and diagnosis of SBTs. Methods: We retrospectively reviewed patients who underwent DBE in our hospital between June 2011 and July 2014. Patients who were diagnosed with SBTs by DBE were included in the study. Results: A total of 316 patients underwent 321 DBE procedures, 80 (25.3%) of whom were suspected to have an SBT. And 59 of the 80 subjects were diagnosed with SBT finally. The majorities (59.3%, 35/59) of the patients diagnosed with SBT were males, whose average age was 61.8 years (SD _+11.0 years). DBE were frequently performed in patients with obscure gastrointes- tinal bleeding (66.1%), abdominal pain (16.9%). The common SBTs identified were: gastrointestinal stromal tumors (GISTs) (24/59, 40.7%), adenocarcinomas (13/59, 22.0%), lymphomas (6/59, 10.2%) and benign polyps (6/59, 10.2%). Jejunum had the highest detection rate (47.5%, 29/61). During the follow-up period (3 months to 3 years), 14 patients were submitted to chemotherapy and two patients died of tumor. Conclusion: The most high frequency type and location of SBTs detected in our hospital were different from western countries. DBE is a valuable and safe examination for SBIs.展开更多
AIM: To analyze the clinical characteristics of small bowel tumors detected by double-balloon enteroscopy (DBE) and to evaluate the diagnostic value of DBE in tumors. METHODS: Four hundred and forty consecutive DBE ex...AIM: To analyze the clinical characteristics of small bowel tumors detected by double-balloon enteroscopy (DBE) and to evaluate the diagnostic value of DBE in tumors. METHODS: Four hundred and forty consecutive DBE examinations were performed in 400 patients (250 males and 150 females, mean age 46.9 ± 16.3 years, range 14-86 years) between January 2007 and April 2012. Of these, 252 patients underwent the antegrade approach, and 188 patients underwent the retrograde approach. All the patients enrolled in our study were suspected of having small bowel diseases with a negative etiological diagnosis following other routine examinations, such as upper and lower gastrointestinal endoscopy and radiography tests. Data on tumors, such as clinical information, endoscopic findings and opera-tion results, were retrospectively collected. RESULTS: Small bowel tumors were diagnosed in 78 patients, of whom 67 were diagnosed using DBE, resulting in a diagnostic yield of 16.8% (67/400); the other 11 patients had negative DBE findings and were diagnosed through surgery or capsule endoscopy. Adenocarcinoma (29.5%, 23/78), gastrointestinal stromal tumor (24.4%, 19/78) and lymphoma (15.4%, 12/78) were the most common tumors. Among the 78 tumors, 60.3% (47/78) were located in the jejunum, and the overall number of malignant tumors was 74.4% (58/78). DBE examinations were frequently performed in patients with obscure gastrointestinal bleeding (47.4%) and abdominal pain (24.4%). The positive detection rate for DBE in the 78 patients with small bowel tumors was 85.9% (67/78), which was higher than that of a computed tomography scan (72.9%, 51/70). Based on the operation results, the accuracy rates of DBE for locating small bowel neoplasms, such as adenocarcinoma, gastrointestinal stromal tumor and lymphoma, were 94.4%, 100% and 100%, respectively. The positive biopsy rates for adenocarcinoma and lymphoma were 71.4% and 60%, respectively. CONCLUSION: DBE is a useful diagnostic tool with high clinical practice value and should be considered the gold standard for the investigation of small bowel tumors.展开更多
AIM:To characterize small bowel(SB)tumors detected by capsule endoscopy(CE),and identify missed tumors.METHODS:The study included 145 consecutive patients in whom 150 CEs were performed.Following CE,the medical record...AIM:To characterize small bowel(SB)tumors detected by capsule endoscopy(CE),and identify missed tumors.METHODS:The study included 145 consecutive patients in whom 150 CEs were performed.Following CE,the medical records of the study population were reviewed.Results of double-or single-balloon enteroscopy performed after CE and the results of surgery in all patients operated on were retrieved.The patients were contacted through telephone interviews or postal mail.In addition,the national cancer registry and the polish clinical gastrointestinal stromal tumor(GIST)Registry were searched to identify missed neoplasms.RESULTS:Indications for CE included overt and occult obscure gastrointestinal bleeding(n=81,53.7%),anemia(n=19,12.7%),malabsorption(n=18,12%),abnormal CB follow through(n=9,6%),abdominal pain(n=7,5%),celiac disease(n=5,3%),neuroendocrine tumor(n=3,2%),Crohn’s disease(n=2,<2%),Peutz-Jeghers syndrome(n=2,<2%),other polyposes(n=2,<2%),and diarrhea(n=2,<2%).The capsule reached the colon in 115(76.6%)examinations.In 150 investigations,CE identified 15SB tumors(10%),14 of which were operated on or treated endoscopically.Malignancies included metastatic melanoma(n=1),adenocarcinoma(n=2),and GIST(n=3).Benign neoplasms included dysplastic Peutz-Jeghers polyps(n=4).Non-neoplastic masses included venous malformation(n=1),inflammatory tumors(n=2),and a mass of unknown histology(n=1).During the follow-up period,three additional SB tumors were found(2 GISTs and one mesenteric tumor of undefined nature).The National Cancer Registry and Polish Clinical GIST Registry revealed no additional SB neoplasms in the post-examination period(follow-up:range 4.2-102.5 mo,median 39 mo).The sensitivity of CE for tumor detection was 83.3%,and the negative predictive value was 97.6%.The specificity and positive predictive value were both 100%.CONCLUSION:Neoplasms may be missed by CE,especially in the proximal SB.In overt obscure gastrointestinal bleeding,complementary endoscopic and/or radiologic diagnostic tests are indicated.展开更多
AIM: To assess the double-balloon enteroscopy(DBE) role in malignant small bowel tumors(MSBT).METHODS: This is a retrospective descriptive study performed in a single center. All consecutive patients who underwent a D...AIM: To assess the double-balloon enteroscopy(DBE) role in malignant small bowel tumors(MSBT).METHODS: This is a retrospective descriptive study performed in a single center. All consecutive patients who underwent a DBE with final diagnosis of a malignant neoplasm from 2004 to 2014 in our referral center were included. Patient demographic and clinical pathological characteristics were recorded and reviewed. MSBT diagnosis was achieved either by DBE directed biopsy with multiple tissue sampling, endoscopic findings or histological analysis of surgical specimen. We have analyzed double-balloon enteroscopy impact in outcome and clinical course of these patients. RESULTS: Of 627 patients, 28(4.5%)(mean age = 60 ± 17.3 years) underwent 30 procedures(25 anterograde, 5 retrograde) and were diagnosed of a malignant tumor. Patients presented with obscure gastrointestinal bleeding(n = 19, 67.9%), occlusion syndrome(n = 7, 25%) and diarrhea(n = 1, 3.6%). They were diagnosed by DBE biopsy(n = 18, 64.3%), histological analysis of surgical specimen(n = 7, 25%) and unequivocal endoscopic findings(n = 2, 7.1%). Gastrointestinal stromal tumor(n = 8, 28.6%), adenocarcinoma(n = 7, 25%), lymphoma(n = 4, 14.3%), neuroendocrine tumor(n = 4, 14.3%), metastatic(n = 3, 10.7%) and Kaposi sarcoma(n = 1, 3.6%) were identified. DBE modified outcome in 7 cases(25%), delaying or avoiding emergency surgery(n = 3), modifying surgery approach(n = 2) and indicating emergency SB partial resection instead of elective approach(n = 2).CONCLUSION: DBE may be critical in the management of MSBT providing additional information that may be decisive in the clinical course of these patients.展开更多
AIM:To assess the value of double-balloon enteroscopy(DBE) for the diagnosis of gastrointestinal mesenchymal tumors(GIMTs) in the small bowel and clarify their clinical and endoscopic characteristics.METHODS:A retrosp...AIM:To assess the value of double-balloon enteroscopy(DBE) for the diagnosis of gastrointestinal mesenchymal tumors(GIMTs) in the small bowel and clarify their clinical and endoscopic characteristics.METHODS:A retrospective review in a total of 783 patients who underwent a DBE procedure from January 2003 to December 2011 was conducted.Data from patients with pathologically confirmed GIMTs were analyzed at a single tertiary center with nine years' experience.The primary outcomes assessed included characteristics of patients with GIMTs,indications for DBE,overall diagnostic yield of GIMTs,endoscopic morphology,positive biopsy,comparison of diagnosis with capsule endoscopy,and subsequent interventional management.RESULTS:GIMTs were identified and analyzed in 77 patients.The mean age was 47.74 ± 14.14 years(range:20-77 years),with 63.6% being males.The majority of individuals presented with gastrointestinal bleeding,accounting for 81.8%,followed by abdominal pain,accounting for 10.4%.Small bowel pathologies were found in 71 patients,the detection rate was 92.2%.The diagnostic yield of DBE for GIMTs was 88.3%.DBE was superior to capsule endoscopy in the diagnosis of GIMTs(P = 0.006;McNemar's χ2 test).Gastrointestinal stromal tumor was the most frequent and leiomyoma was the second frequent GIMT.Single and focal lesions were typical of GIMTs,and masses with smooth or unsmooth surface were the most common in the small bowel.GIMTs were removed from all the patients surgically except one patient treated with endoscopic resection.CONCLUSION:DBE is a safe and valuable procedure for patients with suspected GIMTs,and it provides an accurate position for subsequent surgical intervention.展开更多
AIM:To evaluate the clinical presentation,treatment and survival of patients with primary malignant tumor of small bowel(PMTSB).METHODS:Clinicopathologic data about 141 surgically treated PMTSB patients(91 males and 5...AIM:To evaluate the clinical presentation,treatment and survival of patients with primary malignant tumor of small bowel(PMTSB).METHODS:Clinicopathologic data about 141 surgically treated PMTSB patients(91 males and 50 females) at the median age of 53.5 years(range 23-79 years) were retrospectively analyzed.RESULTS:The most common initial clinical features of the patients were intermittent abdominal discomfort or vague abdominal pain(67.4%),abdominal mass(31.2%),bowel obstruction(24.1%),hemotochezia(21.3%),jaundice(16.3%),fever(14.2%),coexistence of bowel perforation and peritonitis(5.7%),coexistence of gastrointestinal bleeding and shock(5.0%),and intraabdominal bleeding(1.4%).Ileum was the most common site of tumor(44.7%),followed by jejunum(30.5%)and duodenum(24.8%).PMTSB had a nonspecific clinical presentation.Segmental bowel resection(n=81)was the most common surgical procedure,followed by right hemicolectomy(n=15),pancreaticoduodenectomy(n=10),and others(n=19).Twenty-seven adenocarcinoma patients and 13 malignant lymphoma patients received adjuvant chemotherapy with 5-fluorouracil and cyclophosphamide,adriamycin,vincristine and prednisone,respectively.Information about 120 patients was obtained during the follow-up.The median survival time of PMTSB patients was 20.3 mo.The 1-,3-and 5-year survival rate was 75.0%(90/120),40.0%(48/120)and 20.8%(25/120),respectively.Adenocarcinoma was found in 73.7%(42/57),21.1%(12/57)and 15.8%(9/57)of the patients,respec-tively.Gastrointestinal stromal tumor was observed in 80.0%(20/25),72.0%(18/25)and 36.0%(9/25)of the patients,respectively.Carcinoid was detected in 100.0%(15/15),80.0%(12/15)and 46.7%(7/15)of the patients,respectively.Malignant lymphoma was demonstrated in 69.2%(9/13),30.8%(4/13)and 0%(0/13)of the patients,respectively.CONCLUSION:En bloc resection is the principal therapy for most PMTSB and chemotherapy is the important treatment modality for malignant lymphoma and other malignant tumors of small bowel which cannot be radically removed.展开更多
AIM: To retrospectively evaluate the diagnostic efficacy of interventional digital subtraction angiography (DSA) for bleeding small bowel gastrointestinal stromal tumors (GISTs).
Neuroendocrine tumors(NETs), defined as epithelial tumors with predominant neuroendocrine differentiation, are among the most frequent types of small bowel neoplasm. They represent a rare, slow-growing neoplasm with s...Neuroendocrine tumors(NETs), defined as epithelial tumors with predominant neuroendocrine differentiation, are among the most frequent types of small bowel neoplasm. They represent a rare, slow-growing neoplasm with some characteristics common to all forms and others attributable to the organ of origin. The diagnosis of this subgroup of neoplasia is not usually straight-forward for several reasons. Being a rare form of neoplasm they are frequently not readily considered in the differential diagnosis. Also, clinical manifestations are nonspecific lending the clinician no clue that points directly to this entity. However, the annual incidence of NETs has risen in the last years to 40 to 50 cases per million probably not due to a real increase in incidence but rather due to better diagnostic tools that have become progressively available. Being a rare malignancy, investigation regarding its pathophysiology and efforts toward better understanding and classification of these tumors has been limited until recently. Clinical societies dedicated to this matter are emerging(NANETS, ENETS and UKINETS) and several guidelines were published in an effort to standardize the nomenclature, grading and staging systems as well as diagnosis and management of NETs. Also, some investigation on the genetic behavior of small bowel NETs has been recently released, shedding some light on the pathophysiology of these tumors, and pointing some new directions on the possible treating options. In this review we focus on the current status of the overall knowledge about small bowel NETs, focusing on recent breakthroughs and its potential application on clinical practice.展开更多
Extraskeletal Ewing's sarcoma/peripheral primitive neuroectodermal tumor(E-EWS/pP NET) is a rare aggressive malignant small round cell tumor. In this report, we present the case of a 15-year-old boy who suffered f...Extraskeletal Ewing's sarcoma/peripheral primitive neuroectodermal tumor(E-EWS/pP NET) is a rare aggressive malignant small round cell tumor. In this report, we present the case of a 15-year-old boy who suffered from acute abdominal pain accompanied by hematemesis and melena, and was eventually diagnosed with E-EWS/p PNET. To date, there have been only five reported cases of E-EWS/pP NET of the small bowel including the patient in this report. To the best of our knowledge, this is the first documentation of a pP NET of the small bowel mesentery at nonage. All these have made this report rare and significant.展开更多
BACKGROUND Plexiform fibromyxoma is a rare, special type of mesenchymal tumor. The most common presenting symptoms are anemia, hematemesis, and hematochezia, without sex or age predilection. The reported cases have ma...BACKGROUND Plexiform fibromyxoma is a rare, special type of mesenchymal tumor. The most common presenting symptoms are anemia, hematemesis, and hematochezia, without sex or age predilection. The reported cases have mainly occurred in the gastric antrum and pylorus region, with some cases in the duodenum. CASE SUMMARY We report here a case of plexiform fibromyxoma in the upper segment of the jejunum, which was continuously followed up for 3 years after surgical removal. Plexiform fibromyxoma showed multinodular or plexiform growth. The cells in the tumor node were spindle-shaped but few in number and mitotic figures. Small blood vessels and mucous matrix were found among the tumor cells. Immunohistochemistry revealed that the plexiform fibromyxoma cells were positive for smooth muscle actin, focally positive for CD10, and negative for cytokeratin, CD117, DOG-1(discovered on GIST-1) desmin, S-100, epithelial membrane antigen, and CD34. Ki-67 labeling index was < 5%. Plexiform fibromyxoma showed benign biological behavior. After 3 years of consecutive postoperative follow-up, no obvious signs of metastasis or recurrence were found by imaging examination. CONCLUSION Plexiform fibromyxoma is a rare type of mesenchymal tumor. The diagnosis mainly depends on pathological examination, and it should be distinguished from other gastrointestinal mesenchymal tumors.展开更多
AIM: To investigate the prognostic significance of the primary site of disease for small bowel carcinoid(SBC) using a population-based analysis.METHODS: The Surveillance,Epidemiology and End Results(SEER) database was...AIM: To investigate the prognostic significance of the primary site of disease for small bowel carcinoid(SBC) using a population-based analysis.METHODS: The Surveillance,Epidemiology and End Results(SEER) database was queried for histologically confirmed SBC between the years 1988 and 2009.Overall survival(OS) and disease-specific survival(DSS) were analyzed using the Kaplan-Meier method and compared using Log rank testing.Log rank and multivariate Cox regression analyses were used to identify predictors of survival using age,year of diagnosis,race,gender,tumor histology/size/location,tumor-node-metastasis stage,number of lymph nodes(LNs) examined and percent of LNs with metastases.RESULTS: Of the 3763 patients,51.2% were male with a mean age of 62.13 years.Median follow-up was 50 mo.The 10-year OS and DSS for duodenal primaries were significantly better when compared to jejunal and ileal primaries(P = 0.02 and 【 0.0001,respectively).On multivariate Cox regression analysis,after adjusting for multiple factors,primary site location was not a significant predictor of survival(P = 0.752 for OS and P = 0.966 DSS) while age,number of primaries,number of LNs examined,T-stage and M-stage were independent predictors of survival.CONCLUSION: This 21-year,population-based study of SBC challenges the concept that location of the primary lesion alone is a significant predictor of survival.展开更多
BACKGROUND This is a unique case of a patient who was found to have two extremely rare primary malignancies synchronously,i.e.,an ampullary adenocarcinoma arising from a high-grade dysplastic tubulovillous adenoma of ...BACKGROUND This is a unique case of a patient who was found to have two extremely rare primary malignancies synchronously,i.e.,an ampullary adenocarcinoma arising from a high-grade dysplastic tubulovillous adenoma of the ampulla of Vater(TVAoA)with a high-grade ileal gastrointestinal stromal tumor(GIST).Based on a literature review and to the best of our knowledge,this is the first report of this synchronicity.Primary ampullary tumors are extremely rare,with an incidence of four cases per million population,which is approximately 0.0004%.Distal duodenal polyps are uncommon and have a preponderance of occurring around the ampulla of Vater.An adenoma of the ampulla(AoA)may occur sporadically or with a familial inheritance pattern,as in hereditary genetic polyposis syndrome such as familial adenomatous polyposis syndrome(FAPS).We report a case of a 77-year-old male who was admitted for painless obstructive jaundice with a 40-pound weight loss over a two-month period and who was subsequently diagnosed with two extremely rare primary malignancies,i.e.,an adenocarcinoma of the ampulla arising from a high-grade TVAoA and a high-grade ileal GIST found synch-ronously.CASE SUMMARY A 77-year-old male was admitted for generalized weakness with an associated weight loss of 40 pounds in the previous two months and was noted to have painless obstructive jaundice.The physical examination was benign except for bilateral scleral and palmar icterus.Lab results were significant for an obstructive pattern on liver enzymes.Serum lipase and carbohydrate antigen-19-9 levels were elevated.Computed tomography(CT)of the abdomen and pelvis and magnetic resonance cholangiopancreatography were consistent with a polypoid mass at the level of the common bile duct(CBD)and the ampulla of Vater with CBD dilatation.The same lesions were visualized with endoscopic retrograde cholangiopancreatography.Histopathology of endoscopic forceps biopsy showed TVAoA.Histopathology of the surgical specimen of the resected ampulla showed an adenocarcinoma arising from the TVAoA.Abdominal and pelvic CT also showed a coexisting heterogeneously enhancing,lobulated mass in the posterior pelvis originating from the ileum.The patient underwent ampullectomy and resection of the mass and ileo-ileal side-to-side anastomosis followed by chemoradiation.Histopathology of the resected mass confirmed it as a high-grade,spindle cell GIST.The patient is currently on imatinib,and a recent follow-up positron emission tomography(PET)scan showed a complete metabolic response.CONCLUSION This case is distinctive because the patient was diagnosed with two synchronous and extremely rare high-grade primary malignancies,i.e.,an ampullary adenocarcinoma arising from a highgrade dysplastic TVAoA with a high-grade ileal GIST.An AoA can occur sporadically and in a familial inheritance pattern in the setting of FAPS.We emphasize screening and surveillance colonoscopy when one encounters an AoA in upper endoscopy to check for FAPS.An AoA is a premalignant lesion,particularly in the setting of FAPS that carries a high risk of metamorphism to an ampullary adenocarcinoma.Final diagnosis should be based on a histopathologic study of the surgically resected ampullary specimen and not on endoscopic forceps biopsy.The diagnosis of AoA is usually incidental on upper endoscopy.However,patients can present with constitutional symptoms such as significant weight loss and obstructive symptoms such as painless jaundice,both of which occurred in our patient.Patient underwent ampullectomy with clear margins and ileal GIST resection.Patient is currently on imatinib adjuvant therapy and showed complete metabolic response on follow up PET scan.展开更多
文摘Small bowel tumors(SBTs)have been increasingly diagnosed in recent decades.The pathogenesis of this increment is largely unknown,but advances in radiological and endoscopic methods facilitate the improvement of the diagnosis.Capsule endoscopy(CE)and device-assisted enteroscopy(DAE)allow the clinician to assess the entire small bowel in the search for suspicious lesions,or a cause of symptoms.In this review,we discuss the role of enteroscopy,techniques and strategies in the diagnosis and management of SBTs,and a brief description of the most common tumors.
文摘Objective: Small intestinal tumors (SBTs) were difficult to be discovered before surgery. The emergence of dou- ble-balloon enteroscopy (DBE) and capsule endoscopy (CE) have greatly improved the evaluation of SBTs and facilitated a more precise preoperative diagnosis. Yet little data exist in China regarding clinical efficacy of DBE and clinical characteristics of SBTs. The aim of our study was to clarify the clinical characteristics of SBTs and analyze the efficacy of DBE examination in the detection and diagnosis of SBTs. Methods: We retrospectively reviewed patients who underwent DBE in our hospital between June 2011 and July 2014. Patients who were diagnosed with SBTs by DBE were included in the study. Results: A total of 316 patients underwent 321 DBE procedures, 80 (25.3%) of whom were suspected to have an SBT. And 59 of the 80 subjects were diagnosed with SBT finally. The majorities (59.3%, 35/59) of the patients diagnosed with SBT were males, whose average age was 61.8 years (SD _+11.0 years). DBE were frequently performed in patients with obscure gastrointes- tinal bleeding (66.1%), abdominal pain (16.9%). The common SBTs identified were: gastrointestinal stromal tumors (GISTs) (24/59, 40.7%), adenocarcinomas (13/59, 22.0%), lymphomas (6/59, 10.2%) and benign polyps (6/59, 10.2%). Jejunum had the highest detection rate (47.5%, 29/61). During the follow-up period (3 months to 3 years), 14 patients were submitted to chemotherapy and two patients died of tumor. Conclusion: The most high frequency type and location of SBTs detected in our hospital were different from western countries. DBE is a valuable and safe examination for SBIs.
文摘AIM: To analyze the clinical characteristics of small bowel tumors detected by double-balloon enteroscopy (DBE) and to evaluate the diagnostic value of DBE in tumors. METHODS: Four hundred and forty consecutive DBE examinations were performed in 400 patients (250 males and 150 females, mean age 46.9 ± 16.3 years, range 14-86 years) between January 2007 and April 2012. Of these, 252 patients underwent the antegrade approach, and 188 patients underwent the retrograde approach. All the patients enrolled in our study were suspected of having small bowel diseases with a negative etiological diagnosis following other routine examinations, such as upper and lower gastrointestinal endoscopy and radiography tests. Data on tumors, such as clinical information, endoscopic findings and opera-tion results, were retrospectively collected. RESULTS: Small bowel tumors were diagnosed in 78 patients, of whom 67 were diagnosed using DBE, resulting in a diagnostic yield of 16.8% (67/400); the other 11 patients had negative DBE findings and were diagnosed through surgery or capsule endoscopy. Adenocarcinoma (29.5%, 23/78), gastrointestinal stromal tumor (24.4%, 19/78) and lymphoma (15.4%, 12/78) were the most common tumors. Among the 78 tumors, 60.3% (47/78) were located in the jejunum, and the overall number of malignant tumors was 74.4% (58/78). DBE examinations were frequently performed in patients with obscure gastrointestinal bleeding (47.4%) and abdominal pain (24.4%). The positive detection rate for DBE in the 78 patients with small bowel tumors was 85.9% (67/78), which was higher than that of a computed tomography scan (72.9%, 51/70). Based on the operation results, the accuracy rates of DBE for locating small bowel neoplasms, such as adenocarcinoma, gastrointestinal stromal tumor and lymphoma, were 94.4%, 100% and 100%, respectively. The positive biopsy rates for adenocarcinoma and lymphoma were 71.4% and 60%, respectively. CONCLUSION: DBE is a useful diagnostic tool with high clinical practice value and should be considered the gold standard for the investigation of small bowel tumors.
基金Supported by The Polish Foundation for Gastroenterology
文摘AIM:To characterize small bowel(SB)tumors detected by capsule endoscopy(CE),and identify missed tumors.METHODS:The study included 145 consecutive patients in whom 150 CEs were performed.Following CE,the medical records of the study population were reviewed.Results of double-or single-balloon enteroscopy performed after CE and the results of surgery in all patients operated on were retrieved.The patients were contacted through telephone interviews or postal mail.In addition,the national cancer registry and the polish clinical gastrointestinal stromal tumor(GIST)Registry were searched to identify missed neoplasms.RESULTS:Indications for CE included overt and occult obscure gastrointestinal bleeding(n=81,53.7%),anemia(n=19,12.7%),malabsorption(n=18,12%),abnormal CB follow through(n=9,6%),abdominal pain(n=7,5%),celiac disease(n=5,3%),neuroendocrine tumor(n=3,2%),Crohn’s disease(n=2,<2%),Peutz-Jeghers syndrome(n=2,<2%),other polyposes(n=2,<2%),and diarrhea(n=2,<2%).The capsule reached the colon in 115(76.6%)examinations.In 150 investigations,CE identified 15SB tumors(10%),14 of which were operated on or treated endoscopically.Malignancies included metastatic melanoma(n=1),adenocarcinoma(n=2),and GIST(n=3).Benign neoplasms included dysplastic Peutz-Jeghers polyps(n=4).Non-neoplastic masses included venous malformation(n=1),inflammatory tumors(n=2),and a mass of unknown histology(n=1).During the follow-up period,three additional SB tumors were found(2 GISTs and one mesenteric tumor of undefined nature).The National Cancer Registry and Polish Clinical GIST Registry revealed no additional SB neoplasms in the post-examination period(follow-up:range 4.2-102.5 mo,median 39 mo).The sensitivity of CE for tumor detection was 83.3%,and the negative predictive value was 97.6%.The specificity and positive predictive value were both 100%.CONCLUSION:Neoplasms may be missed by CE,especially in the proximal SB.In overt obscure gastrointestinal bleeding,complementary endoscopic and/or radiologic diagnostic tests are indicated.
文摘AIM: To assess the double-balloon enteroscopy(DBE) role in malignant small bowel tumors(MSBT).METHODS: This is a retrospective descriptive study performed in a single center. All consecutive patients who underwent a DBE with final diagnosis of a malignant neoplasm from 2004 to 2014 in our referral center were included. Patient demographic and clinical pathological characteristics were recorded and reviewed. MSBT diagnosis was achieved either by DBE directed biopsy with multiple tissue sampling, endoscopic findings or histological analysis of surgical specimen. We have analyzed double-balloon enteroscopy impact in outcome and clinical course of these patients. RESULTS: Of 627 patients, 28(4.5%)(mean age = 60 ± 17.3 years) underwent 30 procedures(25 anterograde, 5 retrograde) and were diagnosed of a malignant tumor. Patients presented with obscure gastrointestinal bleeding(n = 19, 67.9%), occlusion syndrome(n = 7, 25%) and diarrhea(n = 1, 3.6%). They were diagnosed by DBE biopsy(n = 18, 64.3%), histological analysis of surgical specimen(n = 7, 25%) and unequivocal endoscopic findings(n = 2, 7.1%). Gastrointestinal stromal tumor(n = 8, 28.6%), adenocarcinoma(n = 7, 25%), lymphoma(n = 4, 14.3%), neuroendocrine tumor(n = 4, 14.3%), metastatic(n = 3, 10.7%) and Kaposi sarcoma(n = 1, 3.6%) were identified. DBE modified outcome in 7 cases(25%), delaying or avoiding emergency surgery(n = 3), modifying surgery approach(n = 2) and indicating emergency SB partial resection instead of elective approach(n = 2).CONCLUSION: DBE may be critical in the management of MSBT providing additional information that may be decisive in the clinical course of these patients.
文摘AIM:To assess the value of double-balloon enteroscopy(DBE) for the diagnosis of gastrointestinal mesenchymal tumors(GIMTs) in the small bowel and clarify their clinical and endoscopic characteristics.METHODS:A retrospective review in a total of 783 patients who underwent a DBE procedure from January 2003 to December 2011 was conducted.Data from patients with pathologically confirmed GIMTs were analyzed at a single tertiary center with nine years' experience.The primary outcomes assessed included characteristics of patients with GIMTs,indications for DBE,overall diagnostic yield of GIMTs,endoscopic morphology,positive biopsy,comparison of diagnosis with capsule endoscopy,and subsequent interventional management.RESULTS:GIMTs were identified and analyzed in 77 patients.The mean age was 47.74 ± 14.14 years(range:20-77 years),with 63.6% being males.The majority of individuals presented with gastrointestinal bleeding,accounting for 81.8%,followed by abdominal pain,accounting for 10.4%.Small bowel pathologies were found in 71 patients,the detection rate was 92.2%.The diagnostic yield of DBE for GIMTs was 88.3%.DBE was superior to capsule endoscopy in the diagnosis of GIMTs(P = 0.006;McNemar's χ2 test).Gastrointestinal stromal tumor was the most frequent and leiomyoma was the second frequent GIMT.Single and focal lesions were typical of GIMTs,and masses with smooth or unsmooth surface were the most common in the small bowel.GIMTs were removed from all the patients surgically except one patient treated with endoscopic resection.CONCLUSION:DBE is a safe and valuable procedure for patients with suspected GIMTs,and it provides an accurate position for subsequent surgical intervention.
文摘AIM:To evaluate the clinical presentation,treatment and survival of patients with primary malignant tumor of small bowel(PMTSB).METHODS:Clinicopathologic data about 141 surgically treated PMTSB patients(91 males and 50 females) at the median age of 53.5 years(range 23-79 years) were retrospectively analyzed.RESULTS:The most common initial clinical features of the patients were intermittent abdominal discomfort or vague abdominal pain(67.4%),abdominal mass(31.2%),bowel obstruction(24.1%),hemotochezia(21.3%),jaundice(16.3%),fever(14.2%),coexistence of bowel perforation and peritonitis(5.7%),coexistence of gastrointestinal bleeding and shock(5.0%),and intraabdominal bleeding(1.4%).Ileum was the most common site of tumor(44.7%),followed by jejunum(30.5%)and duodenum(24.8%).PMTSB had a nonspecific clinical presentation.Segmental bowel resection(n=81)was the most common surgical procedure,followed by right hemicolectomy(n=15),pancreaticoduodenectomy(n=10),and others(n=19).Twenty-seven adenocarcinoma patients and 13 malignant lymphoma patients received adjuvant chemotherapy with 5-fluorouracil and cyclophosphamide,adriamycin,vincristine and prednisone,respectively.Information about 120 patients was obtained during the follow-up.The median survival time of PMTSB patients was 20.3 mo.The 1-,3-and 5-year survival rate was 75.0%(90/120),40.0%(48/120)and 20.8%(25/120),respectively.Adenocarcinoma was found in 73.7%(42/57),21.1%(12/57)and 15.8%(9/57)of the patients,respec-tively.Gastrointestinal stromal tumor was observed in 80.0%(20/25),72.0%(18/25)and 36.0%(9/25)of the patients,respectively.Carcinoid was detected in 100.0%(15/15),80.0%(12/15)and 46.7%(7/15)of the patients,respectively.Malignant lymphoma was demonstrated in 69.2%(9/13),30.8%(4/13)and 0%(0/13)of the patients,respectively.CONCLUSION:En bloc resection is the principal therapy for most PMTSB and chemotherapy is the important treatment modality for malignant lymphoma and other malignant tumors of small bowel which cannot be radically removed.
文摘AIM: To retrospectively evaluate the diagnostic efficacy of interventional digital subtraction angiography (DSA) for bleeding small bowel gastrointestinal stromal tumors (GISTs).
文摘Neuroendocrine tumors(NETs), defined as epithelial tumors with predominant neuroendocrine differentiation, are among the most frequent types of small bowel neoplasm. They represent a rare, slow-growing neoplasm with some characteristics common to all forms and others attributable to the organ of origin. The diagnosis of this subgroup of neoplasia is not usually straight-forward for several reasons. Being a rare form of neoplasm they are frequently not readily considered in the differential diagnosis. Also, clinical manifestations are nonspecific lending the clinician no clue that points directly to this entity. However, the annual incidence of NETs has risen in the last years to 40 to 50 cases per million probably not due to a real increase in incidence but rather due to better diagnostic tools that have become progressively available. Being a rare malignancy, investigation regarding its pathophysiology and efforts toward better understanding and classification of these tumors has been limited until recently. Clinical societies dedicated to this matter are emerging(NANETS, ENETS and UKINETS) and several guidelines were published in an effort to standardize the nomenclature, grading and staging systems as well as diagnosis and management of NETs. Also, some investigation on the genetic behavior of small bowel NETs has been recently released, shedding some light on the pathophysiology of these tumors, and pointing some new directions on the possible treating options. In this review we focus on the current status of the overall knowledge about small bowel NETs, focusing on recent breakthroughs and its potential application on clinical practice.
文摘Extraskeletal Ewing's sarcoma/peripheral primitive neuroectodermal tumor(E-EWS/pP NET) is a rare aggressive malignant small round cell tumor. In this report, we present the case of a 15-year-old boy who suffered from acute abdominal pain accompanied by hematemesis and melena, and was eventually diagnosed with E-EWS/p PNET. To date, there have been only five reported cases of E-EWS/pP NET of the small bowel including the patient in this report. To the best of our knowledge, this is the first documentation of a pP NET of the small bowel mesentery at nonage. All these have made this report rare and significant.
文摘BACKGROUND Plexiform fibromyxoma is a rare, special type of mesenchymal tumor. The most common presenting symptoms are anemia, hematemesis, and hematochezia, without sex or age predilection. The reported cases have mainly occurred in the gastric antrum and pylorus region, with some cases in the duodenum. CASE SUMMARY We report here a case of plexiform fibromyxoma in the upper segment of the jejunum, which was continuously followed up for 3 years after surgical removal. Plexiform fibromyxoma showed multinodular or plexiform growth. The cells in the tumor node were spindle-shaped but few in number and mitotic figures. Small blood vessels and mucous matrix were found among the tumor cells. Immunohistochemistry revealed that the plexiform fibromyxoma cells were positive for smooth muscle actin, focally positive for CD10, and negative for cytokeratin, CD117, DOG-1(discovered on GIST-1) desmin, S-100, epithelial membrane antigen, and CD34. Ki-67 labeling index was < 5%. Plexiform fibromyxoma showed benign biological behavior. After 3 years of consecutive postoperative follow-up, no obvious signs of metastasis or recurrence were found by imaging examination. CONCLUSION Plexiform fibromyxoma is a rare type of mesenchymal tumor. The diagnosis mainly depends on pathological examination, and it should be distinguished from other gastrointestinal mesenchymal tumors.
基金Supported by Cedars-Sinai Medical Center Department of Surgical Oncologyby Fellowship Funding from the WilliamRandolph Hearst Foundation(San Francisco,CA)(Dr.Hari DM)the Harold McAlister Charitable Foundation(Los Angeles,CA)(Dr.Leung AM)
文摘AIM: To investigate the prognostic significance of the primary site of disease for small bowel carcinoid(SBC) using a population-based analysis.METHODS: The Surveillance,Epidemiology and End Results(SEER) database was queried for histologically confirmed SBC between the years 1988 and 2009.Overall survival(OS) and disease-specific survival(DSS) were analyzed using the Kaplan-Meier method and compared using Log rank testing.Log rank and multivariate Cox regression analyses were used to identify predictors of survival using age,year of diagnosis,race,gender,tumor histology/size/location,tumor-node-metastasis stage,number of lymph nodes(LNs) examined and percent of LNs with metastases.RESULTS: Of the 3763 patients,51.2% were male with a mean age of 62.13 years.Median follow-up was 50 mo.The 10-year OS and DSS for duodenal primaries were significantly better when compared to jejunal and ileal primaries(P = 0.02 and 【 0.0001,respectively).On multivariate Cox regression analysis,after adjusting for multiple factors,primary site location was not a significant predictor of survival(P = 0.752 for OS and P = 0.966 DSS) while age,number of primaries,number of LNs examined,T-stage and M-stage were independent predictors of survival.CONCLUSION: This 21-year,population-based study of SBC challenges the concept that location of the primary lesion alone is a significant predictor of survival.
文摘BACKGROUND This is a unique case of a patient who was found to have two extremely rare primary malignancies synchronously,i.e.,an ampullary adenocarcinoma arising from a high-grade dysplastic tubulovillous adenoma of the ampulla of Vater(TVAoA)with a high-grade ileal gastrointestinal stromal tumor(GIST).Based on a literature review and to the best of our knowledge,this is the first report of this synchronicity.Primary ampullary tumors are extremely rare,with an incidence of four cases per million population,which is approximately 0.0004%.Distal duodenal polyps are uncommon and have a preponderance of occurring around the ampulla of Vater.An adenoma of the ampulla(AoA)may occur sporadically or with a familial inheritance pattern,as in hereditary genetic polyposis syndrome such as familial adenomatous polyposis syndrome(FAPS).We report a case of a 77-year-old male who was admitted for painless obstructive jaundice with a 40-pound weight loss over a two-month period and who was subsequently diagnosed with two extremely rare primary malignancies,i.e.,an adenocarcinoma of the ampulla arising from a high-grade TVAoA and a high-grade ileal GIST found synch-ronously.CASE SUMMARY A 77-year-old male was admitted for generalized weakness with an associated weight loss of 40 pounds in the previous two months and was noted to have painless obstructive jaundice.The physical examination was benign except for bilateral scleral and palmar icterus.Lab results were significant for an obstructive pattern on liver enzymes.Serum lipase and carbohydrate antigen-19-9 levels were elevated.Computed tomography(CT)of the abdomen and pelvis and magnetic resonance cholangiopancreatography were consistent with a polypoid mass at the level of the common bile duct(CBD)and the ampulla of Vater with CBD dilatation.The same lesions were visualized with endoscopic retrograde cholangiopancreatography.Histopathology of endoscopic forceps biopsy showed TVAoA.Histopathology of the surgical specimen of the resected ampulla showed an adenocarcinoma arising from the TVAoA.Abdominal and pelvic CT also showed a coexisting heterogeneously enhancing,lobulated mass in the posterior pelvis originating from the ileum.The patient underwent ampullectomy and resection of the mass and ileo-ileal side-to-side anastomosis followed by chemoradiation.Histopathology of the resected mass confirmed it as a high-grade,spindle cell GIST.The patient is currently on imatinib,and a recent follow-up positron emission tomography(PET)scan showed a complete metabolic response.CONCLUSION This case is distinctive because the patient was diagnosed with two synchronous and extremely rare high-grade primary malignancies,i.e.,an ampullary adenocarcinoma arising from a highgrade dysplastic TVAoA with a high-grade ileal GIST.An AoA can occur sporadically and in a familial inheritance pattern in the setting of FAPS.We emphasize screening and surveillance colonoscopy when one encounters an AoA in upper endoscopy to check for FAPS.An AoA is a premalignant lesion,particularly in the setting of FAPS that carries a high risk of metamorphism to an ampullary adenocarcinoma.Final diagnosis should be based on a histopathologic study of the surgically resected ampullary specimen and not on endoscopic forceps biopsy.The diagnosis of AoA is usually incidental on upper endoscopy.However,patients can present with constitutional symptoms such as significant weight loss and obstructive symptoms such as painless jaundice,both of which occurred in our patient.Patient underwent ampullectomy with clear margins and ileal GIST resection.Patient is currently on imatinib adjuvant therapy and showed complete metabolic response on follow up PET scan.