Small Bowel Lymphoma Research Articles

Synchronous small bowel neuroendocrine tumour and lymphoma in a centenarian.

Synchronous small bowel neuroendocrine tumour and lymphoma in a centenarian.

2634 Small Bowel Lymphoma Presenting With Perforation: A Case Report

INTRODUCTION: Lymphoma account for only 1 to 4 % of neoplasms in the gastrointestinal tract (GIT), with the stomach being the most common location (68 to 75%) and the small bowel as the second most common (30 %). Among the possible presentations of GIT lymphomas, small bowel perforation is one of the most lethal and rare. Here we present a case of a patient that had a perforated jejunum secondary to lymphoma. CASE DESCRIPTION/METHODS: 57 year old female with history of diabetes, presented with 2 days of severe abdominal pain and vomiting. Physical exam revealed peritoneal signs and absent bowel sounds. CT showed multifocal small bowel thickening, pneumoperitoneum and extraluminal contrast indicating perforation. The patient was rushed to have explorative laparotomy which revealed multiple masses along the small bowel and had resection of a perforated jejunum. Pathology revealed lymphoma. Immunohistochemical staining was positive for CD20 and CD5, and was negative for Bcl-6 and CD10. FISH analysis was negative for translocation t(11,14), t(14,18), and MYC. The patient’s wounds healed and she was eventually treated with cyclophosphamide, doxorubicin, vincristine, prednisone (CHOP) along with rituximab for 6 cycles and Intrathecal (IT) chemotherapy. DISCUSSION: The WHO classify small bowel lymphomas (SBL) into B-cell subtypes (90 % of cases), T-Cell subtypes and Hodgkins Lymphoma.The most common symptoms are abdominal pain (70% of cases), ileus, and weight loss. Perforation was only found in approximately 16% of cases. CT may show polypoid lesions, multiple masses, and small bowel thickening. Usually, SBL is surgically resected for both diagnosis and treatment. Historically, endoscopy has been difficult because of the location. However, with the advent of capsule endoscopy and double balloon techniques, endoscopy can potentially avoid invasive surgeries. Our patient had an acute abdomen and therefore underwent surgical evaluation. Presence of CD20 and CD5, indicated a CD5 positive B-Cell lymphoma. FISH did not show any genetic rearrangements for Cyclin D- t(11,14), a marker for Mantle cell lymphoma, nor Myc and Bcl, markers for Burkitt’s and Follicular cell lymphomas, leading to the diagnosis of DLBCL. Localized tumors undergo surgical resection followed by radiation. TNM stage IIIE or higher is treated with combination chemotherapy, CHOP and rituximab, as was our patient. SBL is a rare entity; this case provides some insight and we hope such knowledge can only improve the management of patients to come.

2519 A Rare Case of Mucosa-Associated Lymphoid Tissue (MALT) Lymphoma Involving Duodenum

INTRODUCTION: Primary small bowel lymphomas account for only 8-9% primary gastrointestinal malignancies and mostly represent extra nodal B- or T-cell origin. Immunoproliferative small bowel disease (IPSID) is a common small bowel extra nodal primary lymphoma. Associated Lymphoid Tissue B cell lymphoma (MALT lymphoma) is a common primary gastric lymphoma. However, it is very rare to find MALT lymphoma in small bowel especially in duodenum. We report a unique case of MALT lymphoma involving duodenal bulb. CASE DESCRIPTION/METHODS: A 58-Yr-Old African American female, with history of type II diabetes, Hypertension, Osteoarthritis, and chronic GERD with symptom control on once a day low dose omeprazole was referred for evaluation of epigastric discomfort, bloating, and nausea for 2 months. Abdominal examination normal. Labs showed normal CBC with differential, and CMP. An upper endoscopy showed mild patchy erythema in stomach and a localized, raised, and nodular area about 4-5 cm in the duodenal bulb. Biopsies from stomach showed Helicobacter pylori chronic active gastritis. Biopsies from duodenum showed homogenous population of lymphoid cells with moderate amounts of clear cytoplasm expanding the lamina propria. Molecular studies showed positive B-cell Gene Rearrangement with positive Framework 1, 2, and 3. Immunohistochemical staining was positive for CD20, bcl2, and kappa; and negative for CD3, CD5, CD10, bcl6, CD43, Cyclin D1 and lamda. The pathology findings confirmed Margina zone lymphoma of Mucosa Associated Lymphoid Tissue (MALT lymphoma). Colonoscopy was normal except few diverticula. A CT scan of abdomen, pelvis, and chest did not show any other masses except prominent lymph nodes at the gastrohepatic ligament and duodenum. She is treated with antibiotic therapy for H pylori gastritis, and referred to oncology for further management. DISCUSSION: MALT lymphomas of stomach usually occur in females and can be associated with Helicobacter pylori infection. They are usually low grade lymphomas and localized to gastrointestinal epithelium. Clinical features, imaging, or endoscopy findings are not specific. Immunohistochemical staining shows positive for B cell phenotype markers like CD20, Bcl-2, and negative for CD 23, CD 10, and CD 5. Usually, they present with non-specific symptoms like epigastric pain, nausea, vomiting, early satiety, though can present with obstruction when the tumor is too large. Treatment of associated H pylori infection may help though chemoradiotherapy may be needed rarely.

Small bowel obstruction in a virgin abdomen

IntroductionSurgical exploration is still considered mandatory in the setting of small bowel obstruction (SBO) and a virgin abdomen by some large centers. The aim of this study is to determine the etiology of SBO in patients without prior abdominal operation. MethodsRetrospective review of the patients treated for SBO and virgin abdomen at the Mayo Clinic between 2006 and 2016 was performed. Follow up data, operative and pathologic findings were examined to determine the etiology of SBO. ResultsSixty patients met inclusion criteria; abdominal exploration was performed in 50 patients (83%) and 10 patients (17%) were managed non-operatively. Exploration was therapeutic in 29 (58%), negative in 20 (40%) and non-therapeutic in one patient (2%). Overall, 8 patients (13%) were diagnosed with a malignancy: right-sided colon cancer (n = 3), small bowel (SB) neuroendocrine tumor (n = 2), SB lymphoma (n = 2) and carcinomatosis peritonei (n = 1); Upon retrospective review, both SB neuroendocrine tumors and one SB lymphoma were visible on the initial imaging. Leukocytosis (p = .03) and no recent weight loss (p = .04) were associated with negative exploration. ConclusionPatients with SBO and virgin abdomen frequently have a benign etiology. Careful imaging review directed at subtle signs of an underlying malignancy is warranted. If non-operative management is chosen, close follow up is essential and it should include a careful personal and family history as well as updated colonoscopy.

Free perforation of primary small bowel lymphoma in a patient with celiac sprue and dermatitis herpetiformis

Small bowel lymphomas are rare and constitute approximately 1% of the malignant gastrointestinal tumors. However, the risk of malignant disease in adult celiac disease is about 8-10%, and non-Hodgkin lymphoma is the most common. In the literature, cases with celiac disease and small bowel lymphoma have been reported, but the emphasis on emergency surgery is extremely rare. We herein present a case of primary small intestinal lymphoma diagnosed after surgery in a 55-year-old male patient who presented to our emergency department with findings of gastrointestinal perforation and had a history of celiac disease and dermatitis herpetiformis. The purpose of this report is to review this situation briefly and discuss it in the light of literature.

Colorectal cancer after negative colonoscopy in fecal immunochemical test-positive participants from a colorectal cancer screening program.

Background and study aims Colorectal cancer (CRC) risk after a positive fecal immunochemical test (FIT) and negative colonoscopy is unknown. We aimed to ascertain the cumulative incidence of post-colonoscopy colorectal cancer (PCCRC) and the manifestation of other lesions that could explain the test positivity in individuals with a negative colonoscopy in a population screening program. Patients and method Observational study in participants from the first round of a CRC screening program (2010 – 2012) with positive-FIT (≥ 20 μg/g of feces) and negative colonoscopy (without neoplasia). A 42- to 76-month follow-up was performed searching in the National Health Service database and by a brief structured telephonic interview. Results Of 2659 FIT-positive individuals who underwent colonoscopy, 811 (30.5 %) had a negative colonoscopy. Three PCCRC (0.4 %) were detected within 11 – 28 months and accelerated carcinogenesis was ruled out. Among those with normal colonoscopy, 32 (5 %) relevant lesions were detected at follow-up. One-third of them (11/32) were significant neoplasias: a gastric cancer, a small-bowel lymphoma, six advanced colorectal adenomas, and the three PCCRC. The 21 remaining lesions were inflammatory, vascular disorders, or non-advanced colorectal adenomas. Conclusions The vast majority (95 %) of individuals did not present any subsequent lesion that could explain the FIT positivity. The very low incidence (0.4 %) and characteristics of PCCRC observed in our cohort reinforce the concept that, although a positive FIT preselects high risk individuals, a high quality colonoscopy is the paramount factor in preventing PCCRC. Improving quality standards of colonoscopy are required to strengthen the current CRC screening strategies.

Small bowel lymphoma presenting as inguinal hernia: case report and literature review

BackgroundInguinal hernia is one of the most common benign pathologies that primarily affects men. Primary gastrointestinal non-Hodgkin’s lymphoma (PGI NHL) is the most common type of extranodal lymphoma. This study reports a rare case in which these two conditions co-exist.Case presentationAn 85-year-old male complained of bowel movement pattern change, abdominal distension and loss of weight, without vomiting but with nausea. A computed tomographic scan of the abdomen showed a small bowel obstruction caused by a migration of a small bowel loop in the right inguinal canal, with a clinically non-reducible inguinal hernia. The patient underwent surgery. The histopathological report showed small bowel large B cell non-Hodgkin’s lymphoma.ConclusionWhen the diagnosis of the contents of an inguinal hernia is not well-established, surgery should be performed as soon as possible to ensure the cure of the disease and the correct diagnosis of the contents.

Primary small bowel lymphoma presenting as invagination.

One of the causes of invagination in adults is primary small bowel lymphoma. Primary small bowel lymphomas are rare, present themselves with complications due to diagnostic difficulties, and are diagnosed only after surgical intervention. In case of invagination, one of the complications of these tumors, namely primary small bowel lymphoma, should also be considered as a cause in the diagnosis. In this paper, the diagnosis and therapy of a rare case of primary polypoid-type small intestinal lymphoma demonstrating findings of obstruction due to invagination have been presented and discussed in the light of the literature.

Small Bowel Lymphoma Coupled with Incidental Appendectomy in a Developing Community

Small Bowel Lymphoma Coupled with Incidental Appendectomy in a Developing Community

Small Intestine: Neuroendocrine and Miscellaneous Tumors

Carcinoid tumors are the most common nonduodenal small bowel tumors. Although the diagnosis of any small bowel tumor is challenging, serum tumor markers and specialized imaging can aid in the diagnosis of carcinoid. Localized carcinoids are treated with surgical resection, whereas metastatic tumors are treated with somatostatin analogues, although liver-directed therapies can improve disease-related symptoms. In contrast, small bowel lymphomas are primarily treated with chemotherapy and sometimes radiation, although surgical intervention may be necessary for diagnosis and resection for palliation of symptoms. Furthermore, there are many benign etiologies of small bowel tumors, including adenoma, leiomyoma, and lipoma. The small bowel can also be a site of distant metastases for which surgical management is reserved for the treatment of complications such as bleeding, obstruction, perforation, or pain. This review contains 3 figures, 3 tables and 17 references. Key words: carcinoid tumor, desmoid tumor, locoregional therapy, mesentery, neuroendocrine tumor, small bowel, small bowel lymphoma, somatostatin

Small Intestine: Neuroendocrine and Miscellaneous Tumors

Carcinoid tumors are the most common nonduodenal small bowel tumors. Although the diagnosis of any small bowel tumor is challenging, serum tumor markers and specialized imaging can aid in the diagnosis of carcinoid. Localized carcinoids are treated with surgical resection, whereas metastatic tumors are treated with somatostatin analogues, although liver-directed therapies can improve disease-related symptoms. In contrast, small bowel lymphomas are primarily treated with chemotherapy and sometimes radiation, although surgical intervention may be necessary for diagnosis and resection for palliation of symptoms. Furthermore, there are many benign etiologies of small bowel tumors, including adenoma, leiomyoma, and lipoma. The small bowel can also be a site of distant metastases for which surgical management is reserved for the treatment of complications such as bleeding, obstruction, perforation, or pain. This review contains 3 figures, 3 tables and 17 references. Key words: carcinoid tumor, desmoid tumor, locoregional therapy, mesentery, neuroendocrine tumor, small bowel, small bowel lymphoma, somatostatin

The HIV manifestations within the gastrointestinal tract: A pictorial review.

The aim of the pictorial review are to review the HIV manifestations within the gastrointestinal tract. We have detailed five conditions, with reference to the patients’ CD4 count – gastrointestinal tuberculosis, Kaposi’s sarcoma, small bowel lymphoma, cytomegalovirus colitis and anal carcinoma.

The role of surgical management in primary small bowel lymphoma: A single-center experience

IntroductionInformation on primary small intestinal lymphoma is more limited than for gastric lymphoma because most of the previous studies did not focus on the former. Few prognostic indicators in primary intestinal lymphoma have been reliably established because of limited patient numbers and variations in criteria for patient selection. In this study, we retrospectively reviewed the clinical and pathological characteristics of small intestinal lymphoma cases from our hospital, to determine prognostic factors and to clarify the effect of surgical resection on prognosis. MethodsEighty-two patients were enrolled in this retrospective study between January 1997 and December 2012. Patients were divided into two groups based on whether or not they underwent surgical management. Gross resection was defined as complete removal of the primary lesion(s), as confirmed by the naked eye. Combined therapy refers to concurrent surgery and chemotherapy. The clinicopathological characteristics and long-term outcomes of patients were analyzed and compared between the two groups. ResultsMost of the patients had abdominal pain (75.6%), and some had loss of body weight (29.3%) and bowel perforation (22.0%). Sixty-two patients (75.6%) underwent surgical management. Patients in the surgery group presented with fewer B symptoms (fever, night sweats, and weight loss; P = 0.035) but more bulky disease (P = 0.009). The ileocecal region was the most common site of solitary involvement (34.1%). The most common reason for surgery was for tumor-related complications (61.3%). Seven patients (11.3%) developed major complications of surgery, but these were not related to the indication, timing, or type of surgery. Only major surgical complications were statistically significant in relation to early mortality (P = 0.004). The estimated 5-year progression-free survival (PFS) was 35.1% and 5-year overall survival (OS) was 43.2%. Univariate analysis revealed that patients in the surgery group had improved 5-year PFS (P = 0.028). T-cell lymphoma, involvement of multiple gastrointestinal regions and extranodal involvement, higher scores for International Prognostic Index (IPI), more advanced Ann Arbor stage, lactate dehydrogenase (LDH) levels above 215 U/L, and management without combined therapy were prognostic for shorter PFS and OS in univariate analyses. Individuals who received R0 resection or gross resection had improved 5-year PFS and OS. Cox regression analysis demonstrated that primary T-cell lymphoma was an independent negative prognostic factor for both OS and PFS. ConclusionCombined therapy is an independent prognostic factor for long-term survival in small intestinal lymphoma. Gross resection is recommended in patients with small intestinal lymphoma and leads to improved PFS without significantly increasing the risk of complications. Emergency surgery does not lead to poor prognosis. However, caution is warranted in the management of all patients, because of the high risk of post-operative complications and potential for early mortality.

A Case of a Rapidly Progressive Small Bowel Lymphoma with High Ki-67 Expression

A Case of a Rapidly Progressive Small Bowel Lymphoma with High Ki-67 Expression

PTU-011 Small Bowel Malignancy in Patients Undergoing Capsule Endoscopy in A Tertiary Care Academic Institution

Introduction Cancer of the small-bowel (SB) is rare accounting for Methods Retrospective study; the records of all patients who underwent SBCE at our centre from Mar 2005 – Oct 2015 were reviewed; we retrieved those whose CEs were reported as suggestive of neoplasia. Further data was gathered on preceding and subsequent investigations, management and outcome of these patients. Results From a total of 1949 CE studies (1082 PillCamTM/867 MiroCamTM), SB neoplasia was diagnosed in 7 patients (0.36%; 2 F/5 M; median age 50, range 34–67). Two had lymphoma, 2 gastrointestinal stromal tumours (GIST), 2 duodenal adenocarcinomas, 1 jejunal metastasis from a sarcomatoid lung tumour. In these patients, CE was performed for: iron-deficiency anaemia (IDA) (n = 5), diarrhoea (n = 1) & suspicion of SB lymphoma (n = 1). 6/7 patients had prior negative bidirectional GI endoscopies; 1 had a normal gastroscopy. Prior to CE, two patients had abdominal USS, 4 had CT scan, 2 had SB follow-through and 1 had a bone marrow aspirate. Two patients had capsule retention; one was removed with a gastroscope, the other with push enteroscopy. All 7 patients had further investigations after CE. Six had a chest, abdomen & pelvis CT scan for staging. Two patients had push enteroscopy, both of whom were diagnosed with duodenal adenocarcinoma. One had double balloon enteroscopy (DBE), two had colonoscopy, two had UGIE; there was one abdominal USS and one bone marrow aspirate. Four pts underwent SB resection. Following resection, 1 patient with GIST had imatinib chemotherapy. Of the two individuals with duodenal adenocarcinoma, one underwent gastroenterostomy and the other had an elective Whipple procedure. Four patients passed away. Two remain under follow up with oncology and one with the GI team. Conclusion SB cancers are rare and our experience is in agreement with other studies. The median age of 50 indicates that SB malignancy is more common in relatively younger patients. Unexplained iron deficiency anaemia was the main presenting complaint in our patients which triggered further investigation despite negative bidirectional endoscopies. CE is effective in picking up SB neoplasia where other imaging modalities have failed. References 1 Schottenfeld D, Beebe-Dimmer JL, Vigneau FD. The epidemiology and pathogenesis of neoplasia in the small intestine. Ann Epidemiol 2009;19:58–69. 2 Pennazio M, Rondonotti E, de Franchis R. Capsule endoscopy in neoplastic diseases. World J Gastroenterol 2008;14:5245–53. 3 Pennazio M, Spada C, Eliakim R, et al. Small-bowel capsule endoscopy and device-assisted enteroscopy for diagnosis and treatment of small-bowel disorders: European Society of Gastrointestinal Endoscopy (ESGE) Clinical Guideline. Endoscopy 2015;47:352–86. 4 Rondonotti E, Pennazio M, Toth E, et al. Small-bowel neoplasms in patients undergoing video capsule endoscopy: a multicenter European study. Endoscopy 2008;40:488–95. Disclosure of Interest C. Johnston: None Declared, D. Yung: None Declared, A. Koulaouzidis Grant/research support from: ESGE- Given®Imaging Research grant 2011, J. Plevris: None Declared

Ileo-ileal Intussusception and Bowel Obstruction Caused by Plasmablastic Lymphoma of Small Bowel- A Rare Entity in Rare Location

Intussusception of small bowel is considered a rare cause of bowel obstruction in adults accounting for only about 1% of bowel obstruction in adults. Intussusception in adults is uncommon with 95% cases of intussusceptions occurring in children. Adult intussusception from small intestinal lymphoma is also rare with only 36 cases reported in the literature between 2000 and 2011. Plasmablastic lymphoma (PBL) is an aggressive lymphoid neoplasm usually seen in the oral cavity in the clinical setting of human immunodeficiency virus (HIV) infection. Plasmablastic lymphoma of the small intestine is extremely rare. Here, we report a case of plasmablastic lymphoma of small bowel with ileoileal intussusception in an HIV-negative immunocompetent male patient.

Radiological Features of Gastrointestinal Lymphoma.

Gastrointestinal lymphomas represent 5–20% of extranodal lymphomas and mainly occur in the stomach and small intestine. Clinical findings are not specific, thus often determining a delay in the diagnosis. Imaging features at conventional and cross-sectional imaging must be known by the radiologist since he/she plays a pivotal role in the diagnosis and disease assessment, thus assisting in the choice of the optimal treatment to patients. This review focuses on the wide variety of imaging presentation of esophageal, gastric, and small and large bowel lymphoma presenting their main imaging appearances at conventional and cross-sectional imaging, mainly focusing on computed tomography and magnetic resonance, helping in the choice of the best imaging technique for the disease characterization and assessment and the recognition of potential complications.

Metastatic Cardiac Liposarcoma of the Small Intestine Causing Eneteroenteric Intussusception: A Case Report and a Review of the Literature

Introduction: Primary cardiac liposarcoma is exceedingly rare and its metastatic potential varies based on tumor subclass. Intestinal intussusception is an uncommon cause of bowel obstruction in adults. We report a case of a primary cardiac liposarcoma in a pregnant woman with small bowel seeding leading to bowel intussusception. Case report: 29-year-old female with history of cardiac liposarcoma presents to the hospital at 27 weeks of gestational age with abdominal pain associated with intractable nausea and vomiting. Three months earlier she was admitted with shortness of breath and dyspnea on exertion. A left intra-atrial mass was found on Cardiac MRI. The patient underwent resection of the mass and mitral valve replacement. Pathology specimen was consistent with dedifferentiated liposarcoma. She was discharged on postoperative day 14. An MRI of the abdomen and pelvis obtained on latest admission revealed an entero-enteric intestinal intussusception with signs of small bowel obstruction.Due to acute cardiopulmonary decline and fetal distress, emergency C-section and exploratory laparotomy were performed. A healthy female infant was delivered. A 10 cm long intussuscepted proximal small bowel segment was identified. Partial small bowel resection with end-to-end anastomosis was performed. Gross pathology showed two polypoid lesions of 5 and 1cm in size. Pathology revealed spindle cells, macronucleoli and lipoid cytoplasmic vacuoles. Immunohistochemistry was positive for MDM2 and CDK4 stains. These findings were identical to the original neoplasm resected from the heart 3 months earlier. The patient succumbed to respiratory failure 8 days postoperatively. Discussion. Primary cardiac neoplasms are very rare. Liposarcoma accounts for about 13% of primary malignant tumors of the heart. Dedifferentiated liposarcoma commonly rises from the retroperitoneum and extremities and carries a lower incidence of distal seeding compared to the pleomorphic variant. Small bowel metastasis are rare. Surgical management is the preferred approach for cardiac liposarcoma. Intestinal intussusception is a rare cause of intestinal obstruction in adults. Enteroenteric intussusceptions due to malignancy accounts for up to 30% of all cases. Metastatic disease, adenocarcinoma and small bowel lymphomas are some of the leading causes.CT Scan of the abdomen and pelvis is the preferred imaging modality for diagnosis.Abdominal Ultrasound and abdominal MRI are two alternatives that can be used in pregnancy to minimize radiation exposure to the fetus. Surgical resection of the intussuscepted area is the treatment of choice in adults. To the best of our knowledge, this is the first reported case of a primary cardiac dedifferentiated liposarcoma causing metastasis to the small bowel with associated intussuception.Figure 1Figure 2Figure 3

Tax-deductible provisions for gluten-free diet in Canada compared with systems for gluten-free diet coverage available in various countries.

Celiac disease affects 1% of the North American population, with an estimated 350,000 Canadians diagnosed with this condition. The disease is triggered by the ingestion of gluten, and a lifelong, strict gluten-free diet (GFD) is the only currently available treatment. Compliance with a strict GFD is essential not only for intestinal mucosal recovery and alleviation of symptoms, but also for the prevention of complications such as anemia, osteoporotic fractures and small bowel lymphoma. However, a GFD is difficult to follow, socially inconvenient and expensive. Different approaches, such as tax reduction, cash transfer, food provision, prescription and subsidy, have been used to reduce the additional costs of the GFD to patients with celiac disease. The current review showed that the systems in place exhibit particular advantages and disadvantages in relation to promoting uptake and compliance with GFD. The tax offset system used in Canada for GFD coverage takes the form of a reimbursement of a cost previously incurred. Hence, the program does not help celiac patients meet the incremental cost of the GFD - it simply provides some future refund of that cost. An ideal balanced approach would involve subsidizing gluten-free products through controlled vouchers or direct food provision to those who most need it, independently of 'ability or willingness to pay'. Moreover, if the cost of such a program is inhibitive, the value of the benefits could be made taxable to ensure that any patient contribution, in terms of additional taxation, is directly related to ability to pay. The limited coverage of GFD in Canada is concerning. There is an unmet need for GFD among celiac patients in Canada. More efforts are required by the Canadian medical community and the Canadian Celiac Association to act as agents in identifying ways of improving resource allocation in celiac disease.

Primary small-bowel malignancy: update in tumor biology, markers, and management strategies.

Primary small-bowel malignancies (SBM) are rare tumors but their incidence is rising. An estimated 9160 new cases and 1210 deaths due to SBM may occur in the USA in 2014. We review advances made in tumor biology, immunohistochemistry, and discuss treatment strategies for these malignancies. Relevant articles from PubMed/Medline and Embase searches were collected using the phrases "small-bowel adenocarcinoma, gastrointestinal carcinoids, gastrointestinal stromal tumors, small-bowel leiomyosarcoma, and small-bowel lymphoma". Advances in imaging techniques such as wireless capsule endoscopy, CT and MRI enterography, and endoscopy (balloon enteroscopy) along with discovery of molecular markers such as c-kit and PDGFRA for GIST tumors have improved our ability to diagnose, localize, and treat these patients. Early detection and surgical resection offers the best chance for long-term survival in all tumors except bowel lymphoma where chemotherapy plays the main role. Adjuvant therapy with imatinib has improved overall survival for GIST tumors, somatostatin analogs have improved symptoms and also inhibited tumor growth and stabilized metastatic disease in carcinoid disease, but chemotherapy has not improved survival for adenocarcinoma. Recent advances in molecular characterization holds promise in novel targeted therapies. Currently ongoing trials are exploring efficacy of targeted therapies and role of adjuvant therapy for adenocarcinoma and results are awaited. Early detection and aggressive surgical therapy for all localized tumors and lymph node sampling particularly for adenocarcinoma remains the main treatment modality.