Association between intestinal neoplasms and celiac disease:A review

Celiac disease(CD)is a chronic immune-mediated intestinal disease with genetic susceptibility.It is characterized by inflammatory damage to the small intestine after ingestion of cereals and products containing gluten protein.In recent years,the global prevalence rate of CD has been approximately 1%,and is gradually increasing.CD patients adhere to a gluten-free diet(GFD)throughout their entire life.However,it is difficult to adhere strictly to a GFD.Untreated CD may be accompanied by gastrointestinal symptoms,such as diarrhea,abdominal pain,and extraintestinal symptoms caused by secondary malnutrition.Many studies have suggested that CD is associated with intestinal tumors such as enteropathyassociated T-cell lymphoma(EATL),small bowel cancer(SBC),and colorectal cancer.In this study,we reviewed related studies published in the literature to provide a reference for the prevention and treatment of intestinal tumors in patients with CD.Compared with the general population,CD patients had a high total risk of SBC and EATL,but not colorectal cancer.The protective effect of GFD on CD-related malignancies is controversial.Further studies are needed to confirm whether GFD treatment can reduce the risk of intestinal neoplasms in CD.

Surgical and Radiological Assessment of Small Intestinal Neoplasms

Purpose: To highlight the role of multi-detector computed tomography in evaluation of small intestinal neoplastic lesions. Patient and Methods: Thirty patients suspected to have small bowel neaoplasms were examined with 64 MDCT and underwent surgical exploration. Result: Contrast enhanced MDCT enterography easily diagnosed twenty patients with small intestinal neoplasms which were confirmed with surgical exploration and histopathological results. Conclusions: Our study has been proved that MDCT can be used as a front-line imaging modality for detection of small bowel neoplasms, regarding its ability to show intraluminal, mural and extraintestinal lesions with their characteristic density and features to successfully differentiate between the different neoplasms and sometimes stage them. However, operative exploration with curative resection or biopsy is still the confirmatory diagnostic method.

Characteristics and prognosis of small bowel tumors:A retrospective study

BACKGROUND Small bowel tumors(SBTs)are a heterogeneous group of difficult-to-diagnose tumors that account for 2%-5%of all gastrointestinal tumors.Single-balloon enteroscopy greatly enhances the diagnosis and treatment of SBTs.However,few epidemiological studies have been conducted in Taiwan to determine the clinical profile of SBTs.AIM To investigate the clinical characteristics,managements and prognosis of SBTs in a medical center in Taiwan.METHODS The study enrolled 51 patients aged 58.9±8.8 years(range,22-93)diagnosed with SBTs from November 2009 to July 2021.We retrospectively recorded clinical characteristics,indications,endoscopic findings,pathological results,management,and outcomes for further analysis.RESULTS A male preponderance was observed(56.8%).The most common indications were suspected small intestinal tumors(52.9%)and obscure gastrointestinal bleeding(39.2%).The most common tumor location was the ileum(41.2%).The performance of imaging studies(P=0.004)and the types of findings(P=0.005)differed significantly between malignant and benign SBTs.The most frequent imaging finding was a small intestinal mass(43.1%).The top three malignant tumor types were gastrointestinal stromal tumors(GISTs),adenocarcinomas,and lymphomas.Moreover,the proportions of benign and malignant tumors were 27.5%and 72.5%,respectively.The survival rates of patients with malignant tumors in the GIST and non-GIST groups differed significantly(P=0.015).Kaplan–Meier survival analysis showed a significant difference in survival between patients in the malignant and benign groups(P=0.04).All patients with lymphoma underwent chemotherapy(n=7/8,87.5%),whereas most patients with GISTs underwent surgery(n=13/14,92.8%).CONCLUSION Patients with GISTs have a significantly higher survival rate than those with other malignant SBTs.Therefore,a large-scale nationwide study is warranted to evaluate the population-based epidemiology of SBTs.

Primary intestinal malignant fibrous histiocytoma:two case reports

Malignant fibrous histiocytoma (MFH) occurs most commonly in the extremities and trunk, but rarely in the intestine. Here we report two cases of primary intestinal MFH. The first case was a 70-year old man admitted for recurrent right lower quadrant abdominal pain. At laparotomy, a tumor was found originating from the cecum, with a suspicious metastatic nodule on the surface of the right lobe of the liver. A right hemicolectomy was performed followed by an ileotransverse end-to-end anastomotic reconstruction. The second case was a 43-year old man with intussusceptions of the small intestine. An emergent laparotomy revealed 4 pedunculated masses in the small bowel and a partial resection of the small intestine was performed. Though the symptoms were not typical, based on histological and immunohistochemical studies, the patients were diagnosed as MFH of the intestine. They were not treated with chemotherapy or radiotherapy and both died within 3 mo. MFH of the intestine is an extremely rare neoplasm with an aggressive biological behavior. The pathogenesis of this disease has not been clarified to date. Complete surgical excision is preferred, adjuvant chemotherapy or radiotherapy may be advisable.

Uptake and outcomes of small intestinal and urinary tract cancer surveillance in Lynch syndrome

BACKGROUND Lynch syndrome(LS)is a hereditary cancer predisposition syndrome associated with increased risk of multiple cancers.While colorectal cancer surveillance decreases mortality in LS and is recommended by guidelines,there is lack of evidence for the efficacy of surveillance for extra-colonic cancers associated with LS,including small intestinal cancer(SIC)and urinary tract cancer(UTC).Given the limited evidence,guidelines do not consistently recommend surveillance for SIC and UTC,and it remains unclear how often individuals will choose to undergo and follow through with extra-colonic surveillance recommendations.AIM To study factors associated with SIC and UTC surveillance uptake and outcomes in LS.METHODS This is an IRB-approved retrospective analysis of individuals with LS seen at a tertiary care referral center.Included individuals had a pathogenic or likely pathogenic variant in MLH1,MSH2,MSH6,PMS2,or EPCAM,or were a confirmed obligate carrier,and had at least one documented visit to our center.Information regarding SIC and UTC surveillance was captured for each individual,and detailed personal and family history was obtained for individuals who had an initial LS management visit in our center’s dedicated high-risk LS clinic between January 1,2017 and October 29,2020.During these initial management visits,all patients had in-depth discussions of SIC and UTC surveillance with 1 of 3 providers experienced in LS management to promote informed decision-making about whether to pursue SIC and/or UTC surveillance.Statistical analysis using Pearson’s chi-squared test and Wilcoxon rank-sum test was completed to understand the factors associated with pursuit and completion of SIC and UTC surveillance,and a P value below 0.05 was deemed statistically significant.RESULTS Of 317 individuals with LS,86(27%)underwent a total of 105 SIC surveillance examinations,with 5 leading to additional work-up and no SICs diagnosed.Additionally,99(31%)patients underwent a total of 303 UTC surveillance examinations,with 19 requiring further evaluation and 1 UTC identified.Of 155 individuals who had an initial LS management visit between January 1,2017 and October 29,2020,63(41%)chose to undergo SIC surveillance and 58(37%)chose to undergo UTC surveillance.However,only 26(41%)and 32(55%)of those who initially chose to undergo SIC or UTC surveillance,respectively,successfully completed their surveillance examinations.Individuals with a pathogenic variant in MSH2 or EPCAM were more likely to initially choose to undergo SIC surveillance(P=0.034),and older individuals were more likely to complete SIC surveillance(P=0.007).Choosing to pursue UTC surveillance was more frequent among older individuals(P=0.018),and females more frequently completed UTC surveillance(P=0.002).Personal history of cancer and family history of SIC or UTC were not significantly associated with electing nor completing surveillance.Lastly,the provider discussing SIC/UTC surveillance was significantly associated with subsequent surveillance choices.CONCLUSION Pursuing and completing SIC/UTC surveillance in LS is influenced by several factors,however broad incorporation in LS management is likely unhelpful due to low yield and frequent false positive results.

Primary early-stage intestinal and colonic non-Hodgkin's lymphoma: Clinical features, management, and outcome of 37 patients

AIM： To analyze the clinical features, management, and outcome of treatment of patients with primary intestinal and colonic non-Hodgkin＇s lymphoma （PICL）. METHODS： A retrospective study was performed in 37 patients with early-stage PICL who were treated in our hospital from 1958 to 1998. Their clinical features, management, and outcome were assessed. Prognostic factors for survival were analyzed by univariate analysis using the Kaplan-Meier product-limit method and log-rank test. RESULTS： Twenty-five patients presented with Ann Arbor stage Ⅰ PICL and 12 with Ann Arbor stage Ⅱ PICL. Thirty-five patients underwent surgery （including 31 with complete resection）, 22 received postoperative chemotherapy or radiotherapy or both. Two patients with rectal tumors underwent biopsy and chemotherapy with or without radiotherapy. The 5- and 10-year overall survival （OS） rates were 51.9% and 44.5%. The corresponding diseasefree survival （DIS） rates were 42.4% and 37.7%. In univariate analysis, multiple-modality treatment was associated with a better DFS rate compared to single treatment （P= 0.001）. While age, tumor size, tumor site, stage, histology, or extent of surgery were not associated with OS and DFS, use of adjuvant chemotherapy significantly improved DFS （P = 0.031） for the 31 patients who underwent complete resection. Additional radiotherapy combined with chemotherapy led to a longer survival than chemotherapy alone in six patients with gross residual disease after surgery or biopsy.CONCLUSION： Combined surgery and chemotherapy is recommended for treatment of patients with PICL, Additional radiotherapy is needed to improve the outcome of patients who have gross residual disease after surgery.

A ruptured large extraluminal ileal gastrointestinal stromal tumor causing hemoperitoneum

We describe an 87-year-old woman with a large ileal gastrointestinal stromal tumor (GIST) causing hemoperitoneum. A CT scan demonstrated a large heterogeneous mass measuring about 13 cm × 11 cm in the pelvis and hemoperitoneum, with a non-uniform enhancement pattern. The mass was diagnosed as a GIST originating from the gastrointestinal tract. She underwent an urgent laparotomy and an ileal GIST with a rupture was found 130 cm from the anal to the Treitz’s ligament. Hemoperitoneum caused by ileal GIST rupture is a rare condition. Bleeding in the large tumor leading to rupture of the capsule might cause hemoperitoneum in the present case.

Free perforation of the small intestine in collagenous sprue

A 67-year-old man with celiac disease developed recurrent diarrhea,profound weakness and weight loss, with evidence of marked protein depletion.His clinical course was refractory to a strict gluten-free diet and steroid therapy.Postmortem studies led to definition of unrecognized collagenous sprue that caused ulceration and small intestinal perforation.Although PCR showed identical monoclonal T-cell populations in antemortem duodenal biopsies and postmortem jejunum,careful pathological evaluation demonstrated no frank lymphoma.Rarely,overt or even cryptic T-cell lymphoma may complicate collagenous sprue,however, small intestinal ulcers and perforation may also develop independently.The dramatic findings here may reflect an underlying or early molecular event in the eventual clinical appearance of overt T-cell lymphoma.

Ewing sarcoma of the ileum with wide multiorgan metastases:A case report and review of literature

BACKGROUND Ewing sarcoma(ES)is an aggressive small round cell tumor that usually occurs in younger children and young adults but rarely in older patients.Its occurrence in elderly individuals is rare.ES of the ileum with wide multiorgan metastases is rarely reported and difficult to distinguish radiologically from other gastrointestinal tract tumors.CASE SUMMARY A 53-year-old man presented with right lower quadrant pain for 2 wk.Computed tomography results showed a heterogeneous mass within the ileum and widespread multiorgan metastases.This mass was biopsied,and pathological examination of the resected specimen revealed features consistent with an extraskeletal ES.CONCLUSION This case emphasizes the importance of recognizing this rare presentation in the small intestine to broaden the differential diagnosis of adult intraabdominal tumors.

Small intestine bleeding due to multifocal angiosarcoma

We report a case of an 84-year-old male patient with primary small intestinal angiosarcoma.The patient initially presented with anemia and melena.Consecutive endoscopy revealed no signs of upper or lower active gastrointestinal bleeding.The patient had been diagnosed 3 years previously with an aortic dilation,which was treated with a stent.Computed tomography suggested an aorto-intestinal fistula as the cause of the intestinal bleeding,leading to operative stent explantation and aortic replacement.However,an aorto-intestinal fistula was not found,and the intestinal bleeding did not arrest postoperatively.The constant need for blood transfusions made an exploratory laparotomy imperative,which showed multiple bleeding sites,predominately in the jejunal wall.A distal loop jejunostomy was conducted to contain the small intestinal bleeding and a segmental resection for histological evaluation was performed.The histological analysis revealed a lessdifferentiated tumor with characteristic CD31,cytokeratin,and vimentin expression,which led to the diagnosis of small intestinal angiosarcoma.Consequently,the infiltrated part of the jejunum was successfully resected in a subsequent operation,and adjuvant chemotherapy with paclitaxel was planned.Angiosarcoma of the small intestine is an extremely rare malignant neoplasm that presents with bleeding and high mortality.Early diagnosis and treatment are essential to improve outcome.A small intestinal angiosarcoma is a challenging diagnosis to make because of its rarity,nonspecific symptoms of altered intestinal function,nonspecific abdominal pain,severe melena,and acute abdominal signs.Therefore,a quick clinical and histological diagnosis and decisive measures including surgery and adjuvant chemotherapy should be the aim.

Primary mucinous cystadenoma of the ileum

Mucinous cystadenoma is a rare benign tumor. Because it has no specific clinical manifestations or imaging features, preoperative clinical and radiologic diagnoses are difficult to make.l-3 We report here a case of primary mucinous cystadenoma of the terminal ileum wall. In this report, the characteristics of the disease will be discussed and the previous reports in literature on mucinous cystadenoma in other organs will be reviewed.