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Dias E, Medas R, Marques M, Andrade P, Cardoso H, Macedo G. Clinicopathological characteristics and prognostic factors of small bowel lymphomas: a retrospective single-center study. Porto Biomed J 2023; 8:e217. [PMID: 37362020 PMCID: PMC10289779 DOI: 10.1097/j.pbj.0000000000000217] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/27/2023] [Accepted: 05/21/2023] [Indexed: 06/28/2023] Open
Abstract
Background There is little information on diagnosis and management of small bowel lymphomas, and optimal management strategies are still undefined. This study aims to describe their main clinical and pathological characteristics and identify poor prognostic factors. Methods A retrospective observational study of all patients with histological diagnosis of small bowel lymphoma between January 2010 and December 2020 was performed. Results We included 40 patients, with male predominance (60%) and mean age of 60.7 years. The ileum was the most common location, and the most common histological subtypes were follicular lymphoma and diffuse large B-cell lymphoma. Clinical presentation was variable from asymptomatic patients (30%) to acute surgical complications (35%) including perforation, intestinal obstruction, ileal intussusception, or severe bleeding. Diagnosis was established by endoscopy in 22 patients (55%), and the most common findings included polyps, single mass, diffuse infiltration, or ulceration, whereas 18 (45%) required surgery because of acute presentations or tumor resection, and lymphoma was diagnosed postoperatively. Surgery was curative in one-third of those patients. Median survival was 52 months. Acute presentation (P = 0.001), symptomatic disease (P = 0.003), advanced stage (P = 0.008), diffuse large B-cell lymphoma (P = 0.007), anemia (P = 0.006), hypoalbuminemia (P < 0.001), elevated lactate dehydrogenase (P = 0.02), elevated C-reactive protein (P < 0.001), and absence of treatment response (P < 0.001) were significant predictors of mortality. Conclusion Small bowel lymphoma is a rare malignancy with diverse clinical and endoscopic presentations that require a high index of suspicion. Primary factors associated with worse outcome included acute presentation, advanced stage, histological subtype, biochemical abnormalities, and absence of treatment response.
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Affiliation(s)
- Emanuel Dias
- Corresponding author. Address: Gastrenterology Department, Centro Hospitalar Universitário de São João, Alameda Professor Hernâni Monteiro, 4200-319, Porto, Portugal. E-mail address: (Emanuel Dias)
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Characteristic endoscopic findings of gastrointestinal malignant lymphomas other than mucosa-associated lymphoid tissue lymphoma. Acta Gastroenterol Belg 2022; 85:477-483. [DOI: 10.51821/85.3.9712] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/12/2022]
Abstract
Background and study aims: The gastrointestinal (GI) tract is the most common site of extra-nodal involvement for non-Hodgkin’s lymphoma (NHL). The features of GI NHLs remain unclear. The aim of this study was to clarify endoscopic characteristics of GI NHLs.
Patients and methods: We retrospectively analyzed the morphological characteristics of 63 GI malignant lymphomas other than mucosa-associated lymphoid tissue lymphoma. Lesions were diagnosed between 2005 and 2020. Macroscopic findings were classified into five subtypes: superficial (S); protruding without ulcer (P); protruding with ulcer (PU); fungating (F); and multiple nodules (MN).
Results: Thirty-one lesions in the stomach were classified as S type in 3 cases (9.6%), P type in 6 (19%), PU type in 13 (42%), and F type in 9 (29%). In the stomach, the ulcerated phenotype was more frequent for diffuse large B-cell lymphoma (DLBCL) (89.5%) than for other histological types (41.7%; P = 0.01). In the intestine, 23 tumors were classified as S type in 4 cases (17%), P type in 1 (4%), PU type in 6 (26%), F type in 1 (4%), and MN in 11 (48%). Eleven of the 14 cases (78.6%) of intestinal follicular lymphoma lesions showed MN type. In the colon, eight tumors were classified as S type in 2 cases (25%), P type in 2 (25%), PU type in 1 (13%), and F type in 3 (38%).
Conclusion: We have clarified the endoscopic features of GI NHL using macroscopic classifications. The ulcerated phenotype was the most frequent endoscopic finding for DLBCL.
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Horie T, Hosoe N, Takabayashi K, Hayashi Y, Kamiya KJLL, Miyanaga R, Mizuno S, Fukuhara K, Fukuhara S, Naganuma M, Shimoda M, Ogata H, Kanai T. Endoscopic characteristics of small intestinal malignant tumors observed by balloon-assisted enteroscopy. World J Gastrointest Endosc 2019; 11:373-382. [PMID: 31205598 PMCID: PMC6556486 DOI: 10.4253/wjge.v11.i5.373] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/23/2019] [Revised: 05/11/2019] [Accepted: 05/13/2019] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Capsule endoscopy and balloon-assisted enteroscopy (BAE) enable visualization of rare small bowel conditions such as small intestinal malignant tumors. However, details of the endoscopic characteristics of small intestinal malignant tumors are still unknown. AIM To elucidate the endoscopic characteristics of small intestinal malignant tumors. METHODS From March 2005 to February 2017, 1328 BAE procedures were performed at Keio University Hospital. Of these procedures, malignant tumors were classified into three groups, Group 1: epithelial tumors including primary small intestinal cancer, metastatic small intestinal cancer, and direct small intestinal invasion by an adjacent organ cancer; Group 2: small intestinal malignant lymphoma; and Group 3, small intestinal gastrointestinal stromal tumors. We systematically collected clinical and endoscopic data from patients' medical records to determine the endoscopic characteristics for each group. RESULTS The number of patients in each group was 16 (Group 1), 22 (Group 2), and 6 (Group 3), and the percentage of solitary tumors was 100%, 45.5%, and 100%, respectively (P < 0.001). Patients' clinical background parameters including age, symptoms, and laboratory data were not significantly different between the groups. Seventy-five percent of epithelial tumors (Group 1) were located in the upper small intestine (duodenum and ileum), and approximately 70% of gastrointestinal stromal tumors (Group 3) were located in the jejunum. Solitary protruding or mass-type tumors were not seen in malignant lymphoma (Group 2) (P < 0.001). Stenosis was seen more often in Group 1, (68.8%, 27.3%, and 0%; Group 1, 2, and 3, respectively; P = 0.004). Enlarged white villi inside and/or surrounding the tumor were seen in 12.5%, 54.5%, and 0% in Group 1, 2, and 3, respectively (P = 0.001). CONCLUSION The differential diagnosis of small intestinal malignant tumors could be tentatively made based on BAE findings.
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Affiliation(s)
- Tomofumi Horie
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, School of Medicine, Keio University, Tokyo 160-8582, Japan
| | - Naoki Hosoe
- Center for Diagnostic and Therapeutic Endoscopy, School of Medicine, Keio University, Tokyo 160-8582, Japan
| | - Kaoru Takabayashi
- Center for Diagnostic and Therapeutic Endoscopy, School of Medicine, Keio University, Tokyo 160-8582, Japan
| | - Yukie Hayashi
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, School of Medicine, Keio University, Tokyo 160-8582, Japan
| | - Kenji JL Limpias Kamiya
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, School of Medicine, Keio University, Tokyo 160-8582, Japan
| | - Ryoichi Miyanaga
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, School of Medicine, Keio University, Tokyo 160-8582, Japan
| | - Shinta Mizuno
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, School of Medicine, Keio University, Tokyo 160-8582, Japan
| | - Kayoko Fukuhara
- Center for Diagnostic and Therapeutic Endoscopy, School of Medicine, Keio University, Tokyo 160-8582, Japan
| | - Seiichiro Fukuhara
- Center for Diagnostic and Therapeutic Endoscopy, School of Medicine, Keio University, Tokyo 160-8582, Japan
| | - Makoto Naganuma
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, School of Medicine, Keio University, Tokyo 160-8582, Japan
| | - Masayuki Shimoda
- Department of Pathology, School of Medicine, Keio University, Tokyo 160-8582, Japan
| | - Haruhiko Ogata
- Center for Diagnostic and Therapeutic Endoscopy, School of Medicine, Keio University, Tokyo 160-8582, Japan
| | - Takanori Kanai
- Division of Gastroenterology and Hepatology, Department of Internal Medicine, School of Medicine, Keio University, Tokyo 160-8582, Japan
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Aoki T, Yamada A, Takahashi M, Niikura R, Toyama K, Ushiku T, Kurokawa M, Momose T, Fukayama M, Koike K. Development and internal validation of a risk scoring system for gastrointestinal events requiring surgery in gastrointestinal lymphoma patients. J Gastroenterol Hepatol 2019; 34:693-699. [PMID: 30151937 DOI: 10.1111/jgh.14452] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/04/2018] [Revised: 07/18/2018] [Accepted: 08/19/2018] [Indexed: 12/16/2022]
Abstract
BACKGROUND AND AIM The predictors of severe gastrointestinal (GI) events in GI lymphoma patients are unclear. We aimed to develop a risk scoring system for GI events requiring surgery. METHODS In this retrospective study of 192 patients with GI lymphoma, the state of lymphoma, macroscopic findings, examination results, and International Prognostic Index were assessed. We developed a risk score for GI events that required surgery and assessed its accuracy by calculating the area under the receiver operating characteristic curve (AUC). Internal validation was performed using bootstrap resampling. RESULTS Severe GI events occurred in 21 (11%) patients. We developed a 4-point scoring system (the FLASH score) comprising the following three independent predictors (weighted by regression coefficients): (i) focal appearance and large size (≥ 40 mm), 1 point; (ii) aggressive lymphoma of the small bowel, 2 points; and (iii) high (18)F-fluorodeoxyglucose positron emission tomography uptake, 1 point. The score predicted severe GI events with an AUC value of 0.91 (internal validation; AUC, 0.86). Risk was classified into three categories: the GI event rate was 0% in the low-risk group (0 points), 9% in the intermediate-risk group (1-2 points), and 61% in the high-risk group (3-4 points) (AUC, 0.89). CONCLUSIONS We developed and internally validated a risk scoring system (the FLASH score) that included macroscopic findings to predict severe GI events in GI lymphoma patients. Patients with high scores are candidates for elective surgery to prevent GI events.
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Affiliation(s)
- Tomonori Aoki
- Department of Gastroenterology Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | - Atsuo Yamada
- Department of Gastroenterology Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | - Miwako Takahashi
- Division of Nuclear Medicine, Department of Radiology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | - Ryota Niikura
- Department of Gastroenterology Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | - Kazuhiro Toyama
- Department of Hematology and Oncology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | - Tetsuo Ushiku
- Department of Pathology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | - Mineo Kurokawa
- Department of Hematology and Oncology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | - Toshimitsu Momose
- Division of Nuclear Medicine, Department of Radiology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | - Masashi Fukayama
- Department of Pathology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
| | - Kazuhiko Koike
- Department of Gastroenterology Graduate School of Medicine, The University of Tokyo, Tokyo, Japan
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Gastrointestinal mantle cell lymphoma with isolated mass and multiple lymphomatous polyposis: report of two cases. Clin J Gastroenterol 2017; 10:327-330. [PMID: 28451951 DOI: 10.1007/s12328-017-0740-5] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/05/2017] [Accepted: 04/15/2017] [Indexed: 10/19/2022]
Abstract
We herein report two patients with mantle cell lymphoma (MCL), who had isolated mass and multiple lymphomatous polyposis (MLP) in the gastrointestinal tract. In case 1, esophagogastroduodenoscopy revealed a protruding mass in the duodenum and double-balloon endoscopy disclosed numerous polypoid lesions in the ileum. Case 2 had polyposis in the duodenum and a large mass-forming lesion in the ascending colon. Based on the histologic and immunohistochemical findings of the biopsy specimens, the diagnosis of MCL was made in both patients. A combination of isolated mass and MLP is considered as characteristic endoscopic findings of intestinal lesions of MCL.
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Celiac Disease and Double-Balloon Enteroscopy: What Can We Achieve?: The Experience of 2 European Tertiary Referral Centers. J Clin Gastroenterol 2016; 50:313-7. [PMID: 26524152 DOI: 10.1097/mcg.0000000000000424] [Citation(s) in RCA: 19] [Impact Index Per Article: 2.1] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/13/2022]
Abstract
BACKGROUND Indications to double-balloon enteroscopy (DBE) are not standardized in celiac disease (CD). GOALS To evaluate the clinical usefulness of DBE in complicated CD. STUDY DBE findings in celiac patients with suspected small bowel (SB) complications were retrospectively evaluated in 2 tertiary referral centers (Milan and Sheffield). Demographic data of the studied cohort were compared with a database of 1000 noncomplicated CD patients. RESULTS Twenty-four CD cases (12 males, P=0.01 vs. controls) were reviewed. Mean age at CD diagnosis (y±SD) was 37±20 versus 27±18 and at SB evaluation 47±15 versus 38±13 (P<0.01 compared with controls). Indications for DBE were refractory CD (#9), gastrointestinal symptoms (#6), severe iron-deficiency anemia (#6), and long standing poor dietary adherence (#3). Two jejunal adenocarcinomas and an ileal neuroendocrine tumor were detected in presence of iron-deficiency anemia. Three type I and 3 type II refractory CD patients showed jejunal ulcerations; 2 of type II presented small white raised patches. Patchy atrophy was observed in nonadherent patients and in 2 on a gluten-free diet for a short time. Therapy was planned in 33% of patients after DBE. No adverse events were detected at follow-up [21 mo (range, 0 to 60 mo)]. CONCLUSIONS This is the largest international study on the outcomes of DBE in CD demonstrating its usefulness to exclude/confirm malignant or premalignant conditions, associated with even minor lesions. Studies are needed to understand the clinical relevance of the SB endoscopic features and to optimize DBE indications.
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Nakamura S, Matsumoto T. Treatment Strategy for Gastric Mucosa-Associated Lymphoid Tissue Lymphoma. Gastroenterol Clin North Am 2015; 44:649-60. [PMID: 26314674 DOI: 10.1016/j.gtc.2015.05.012] [Citation(s) in RCA: 14] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
Recent trends and current knowledge on the diagnosis and treatment strategy for gastric mucosa-associated lymphoid tissue (MALT) lymphoma are reviewed. Helicobacter pylori infection plays the causative role in the pathogenesis, and H pylori eradication is the first-line treatment of this disease, which leads to complete remission in 60% to 90% of cases. A Japanese multicenter study confirmed that the long-term outcome of gastric MALT lymphoma after H pylori eradication is excellent. Treatment strategies for patients not responding to H pylori eradication including "watch and wait" strategy, radiotherapy, chemotherapy, rituximab immunotherapy, and combination of these should be tailored in consideration of the disease extent in each patient.
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Affiliation(s)
- Shotaro Nakamura
- Division of Gastroenterology, Department of Internal Medicine, School of Medicine, Iwate Medical University, Uchimaru 19-1, Morioka 020-8505, Japan.
| | - Takayuki Matsumoto
- Division of Gastroenterology, Department of Internal Medicine, School of Medicine, Iwate Medical University, Uchimaru 19-1, Morioka 020-8505, Japan
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Vetro C, Bonanno G, Giulietti G, Romano A, Conticello C, Chiarenza A, Spina P, Coppolino F, Cunsolo R, Raimondo FD. Rare gastrointestinal lymphomas: The endoscopic investigation. World J Gastrointest Endosc 2015; 7:928-949. [PMID: 26265987 PMCID: PMC4530327 DOI: 10.4253/wjge.v7.i10.928] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/12/2015] [Revised: 06/01/2015] [Accepted: 07/09/2015] [Indexed: 02/05/2023] Open
Abstract
Gastrointestinal lymphomas represent up to 10% of gastrointestinal malignancies and about one third of non-Hodgkin lymphomas. The most prominent histologies are mucosa-associated lymphoid tissue lymphoma and diffuse large B-cell lymphoma. However, the gastrointestinal tract can be the site of rarer lymphoma subtypes as a primary or secondary localization. Due to their rarity and the multifaceted histology, an endoscopic classification has not been validated yet. This review aims to analyze the endoscopic presentation of rare gastrointestinal lymphomas from disease diagnosis to follow-up, according to the involved site and lymphoma subtype. Existing, new and emerging endoscopic technologies have been examined. In particular, we investigated the diagnostic, prognostic and follow-up endoscopic features of T-cell and natural killer lymphomas, lymphomatous polyposis and mantle cell lymphoma, follicular lymphoma, plasma cell related disease, gastrointestinal lymphomas in immunodeficiency and Hodgkin’s lymphoma of the gastrointestinal tract. Contrarily to more frequent gastrointestinal lymphomas, data about rare lymphomas are mostly extracted from case series and case reports. Due to the data paucity, a synergism between gastroenterologists and hematologists is required in order to better manage the disease. Indeed, clinical and prognostic features are different from nodal and extranodal or the bone marrow (in case of plasma cell disease) counterpart. Therefore, the approach should be based on the knowledge of the peculiar behavior and natural history of disease.
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Fujiya M, Kashima S, Ikuta K, Dokoshi T, Sakatani A, Tanaka K, Ando K, Ueno N, Tominaga M, Inaba Y, Ito T, Moriichi K, Tanabe H, Saitoh Y, Kohgo Y. Decreased numbers of vascular networks and irregular vessels on narrow-band imaging are useful findings for distinguishing intestinal lymphoma from lymphoid hyperplasia. Gastrointest Endosc 2014; 80:1064-71. [PMID: 24830575 DOI: 10.1016/j.gie.2014.03.030] [Citation(s) in RCA: 7] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/20/2013] [Accepted: 03/18/2014] [Indexed: 02/08/2023]
Abstract
BACKGROUND No method for sufficiently making the differential diagnosis of intestinal lymphoma resembling lymphoid hyperplasia (LH) on endoscopy has yet been established. OBJECTIVE The aim of this study was to evaluate the usefulness of narrow-band imaging (NBI) in diagnosing intestinal lymphoma. DESIGN Prospective study. SETTING Single-center study. PATIENTS Sixty-one patients with primary or systemic lymphoma were enrolled in this study. INTERVENTIONS The terminal ileum and entire colon were observed by using conventional endoscopy. NBI was subsequently performed when small polypoid lesions were detected. A decrease in the number of vascular networks (DVNs) and the presence of irregular vessels on the surface of the epithelia were defined as characteristic findings of intestinal lymphoma. The diagnostic accuracy of these 2 findings in distinguishing intestinal lymphoma from LH was examined. MAIN OUTCOME MEASUREMENTS The ability to use NBI to distinguish intestinal lymphoma from LH. RESULTS Two hundred ninety-four small polypoid lesions, including 59 lymphomas and 235 LH lesions, were detected. The rates of detecting DVNs and the presence of irregular vessels were significantly higher in the lymphoma samples (81.4% and 62.7%) than in the LH samples (25.5% and 4.7%). Based on these findings, the diagnostic accuracy, sensitivity, specificity, and positive and negative predictive values for differentiating intestinal lymphoma from LH were 88.8%, 62.7%, 95.3%, 77.1%, and 91.1%, respectively, which are significantly higher than those of conventional endoscopy. LIMITATIONS Single-center study. CONCLUSION DVNs and the presence of irregular vessels on NBI are thus considered to be useful findings for differentiating intestinal lymphoma from benign LH.
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Affiliation(s)
- Mikihiro Fujiya
- Division of Gastroenterology and Hematology/Oncology, Department of Medicine, Asahikawa Medical University, Asahikawa, Japan
| | - Shin Kashima
- Division of Gastroenterology and Hematology/Oncology, Department of Medicine, Asahikawa Medical University, Asahikawa, Japan
| | - Katsuya Ikuta
- Division of Gastroenterology and Hematology/Oncology, Department of Medicine, Asahikawa Medical University, Asahikawa, Japan
| | - Tatsuya Dokoshi
- Division of Gastroenterology and Hematology/Oncology, Department of Medicine, Asahikawa Medical University, Asahikawa, Japan
| | - Aki Sakatani
- Division of Gastroenterology and Hematology/Oncology, Department of Medicine, Asahikawa Medical University, Asahikawa, Japan
| | - Kazuyuki Tanaka
- Division of Gastroenterology and Hematology/Oncology, Department of Medicine, Asahikawa Medical University, Asahikawa, Japan
| | - Katsuyoshi Ando
- Division of Gastroenterology and Hematology/Oncology, Department of Medicine, Asahikawa Medical University, Asahikawa, Japan
| | - Nobuhiro Ueno
- Division of Gastroenterology and Hematology/Oncology, Department of Medicine, Asahikawa Medical University, Asahikawa, Japan
| | - Motoya Tominaga
- Division of Gastroenterology and Hematology/Oncology, Department of Medicine, Asahikawa Medical University, Asahikawa, Japan
| | - Yuhei Inaba
- Division of Gastroenterology and Hematology/Oncology, Department of Medicine, Asahikawa Medical University, Asahikawa, Japan
| | - Takahiro Ito
- Division of Gastroenterology and Hematology/Oncology, Department of Medicine, Asahikawa Medical University, Asahikawa, Japan
| | - Kentaro Moriichi
- Division of Gastroenterology and Hematology/Oncology, Department of Medicine, Asahikawa Medical University, Asahikawa, Japan
| | - Hiroki Tanabe
- Division of Gastroenterology and Hematology/Oncology, Department of Medicine, Asahikawa Medical University, Asahikawa, Japan
| | - Yusuke Saitoh
- Digestive Disease Center, Asahikawa City Hospital, Asahikawa, Japan
| | - Yutaka Kohgo
- Division of Gastroenterology and Hematology/Oncology, Department of Medicine, Asahikawa Medical University, Asahikawa, Japan
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Nakamura S, Matsumoto T. Helicobacter pylori and gastric mucosa-associated lymphoid tissue lymphoma: Recent progress in pathogenesis and management. World J Gastroenterol 2013; 19:8181-8187. [PMID: 24363507 PMCID: PMC3857439 DOI: 10.3748/wjg.v19.i45.8181] [Citation(s) in RCA: 45] [Impact Index Per Article: 3.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/25/2013] [Revised: 10/22/2013] [Accepted: 11/05/2013] [Indexed: 02/06/2023] Open
Abstract
Recent progress in the research regarding the molecular pathogenesis and management of gastric mucosa-associated lymphoid tissue (MALT) lymphoma is reviewed. In approximately 90% of cases, Helicobacter pylori (H. pylori) infection plays the causative role in the pathogenesis, and H. pylori eradication is nowadays the first-line treatment for this disease, which leads to complete disease remission in 50%-90% of cases. In H. pylori-dependent cases, microbe-generated immune responses, including interaction between B and T cells involving CD40 and CD40L co-stimulatory molecules, are considered to induce the development of MALT lymphoma. In H. pylori-independent cases, activation of the nuclear factor-κB pathway by oncogenic products of specific chromosomal translocations such as t(11;18)/API2-MALT1, or inactivation of tumor necrosis factor alpha-induced protein 3 (A20) are considered to contribute to the lymphomagenesis. Recently, a large-scale Japanese multicenter study confirmed that the long-term clinical outcome of gastric MALT lymphoma after H. pylori eradication is excellent. Treatment modalities for patients not responding to H. pylori eradication include a “watch and wait” strategy, radiotherapy, chemotherapy, rituximab immunotherapy, and a combination of these. Because of the indolent behavior of MALT lymphoma, second-line treatment should be tailored in consideration of the clinical stage and extent of the disease in each patient.
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Okada E, Araki A, Suzuki S, Watanabe H, Ikeda T, Watanabe T, Kurata M, Eishi Y, Watanabe M. Histological diagnosis of follicular lymphoma by biopsy of small intestinal normal mucosa. Dig Endosc 2013; 25:544-6. [PMID: 23369148 DOI: 10.1111/j.1443-1661.2012.01386.x] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/11/2012] [Accepted: 08/20/2012] [Indexed: 02/08/2023]
Abstract
Due to advances in double-balloon endoscopy, various types of malignant lymphoma of the small intestine have been observed at very early stages. We report here that after remission of diffuse large B-cell lymphoma, follicular lymphoma recurred in the mucosa of the small intestine. Furthermore, these lesions were diagnosed by biopsy from normal mucosa as diminutive erosions and reddish looking. If a small intestinal lesion is suspected of being a malignant lymphoma, a double-balloon endoscopy should be carried out, and a biopsy should be taken and inspected.
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Affiliation(s)
- Eriko Okada
- Department of Gastroenterology and Hepatology, Tokyo Medical and Dental University, Tokyo, Japan
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Abstract
Enteropathy-associated T-cell lymphoma (EATL) is a complication of celiac disease (CD). This tumor derives from the neoplastic transformation of aberrant intraepithelial T lymphocytes emerging in celiac patients unresponsive to a gluten-free diet. Poor adherence to a gluten-free diet, HLA-DQ2 homozygosity, and late diagnosis of CD are recognized as risk factors for malignant evolution of CD. Recurrence of diarrhea, unexplained weight loss, abdominal pain, fever, and night sweating should alert physicians to this complication. The suspicion of EATL should lead to an extensive diagnostic workup in which magnetic resonance enteroclysis, positron emission tomography scan, and histologic identification of lesions represent the best options. Treatment includes high-dose chemotherapy preceded by surgical resection and followed by autologous stem cell transplantation, although biologic therapies seem to be promising. Strict adherence to a gluten-free diet remains the only way to prevent EATL.
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Bennani-Baiti N, Daw HA, Cotta C, Martin P, Mitchell KW, Ambinder RF, Macklis R, Pollock R, Spiro T. Low-grade follicular lymphoma of the small intestine: a challenge for management. Semin Oncol 2011; 38:714-20. [PMID: 22082756 DOI: 10.1053/j.seminoncol.2011.08.002] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/16/2022]
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Yanai S, Nakamura S, Takeshita M, Fujita K, Hirahashi M, Kawasaki K, Kurahara K, Sakai Y, Matsumoto T. Translocation t(14;18)/IGH-BCL2 in gastrointestinal follicular lymphoma: correlation with clinicopathologic features in 48 patients. Cancer 2010; 117:2467-77. [PMID: 24048795 DOI: 10.1002/cncr.25811] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/07/2010] [Revised: 10/16/2010] [Accepted: 10/28/2010] [Indexed: 12/19/2022]
Abstract
BACKGROUND Chromosomal translocation t(14;18)(q32;q21) involving the immunoglobulin heavy chain gene (IGH) and the BCL2 gene (t[14;18][q32;q21]/IGH-BCL2) is present in 60% to 90% of nodal follicular lymphomas. To the authors' knowledge, the prevalence and clinical significance of this translocation have not been examined previously in gastrointestinal follicular lymphomas. METHODS Clinicopathologic and molecular features were investigated in 48 patients who had gastrointestinal follicular lymphoma. The site of involvement was the duodenum in 54% of patients, the jejunum in 52%, the ileum in 52%, the stomach in 29%, and the colorectum in 15%. The presence of the t(14;18)/IGH-BCL2 translocation was detected by interphase fluorescence in situ hybridization. RESULTS Treatment modalities included surgical resection (n = 16), rituximab plus chemotherapy (n = 13), rituximab alone (n = 6), antibiotics (n = 5), and watchful waiting (n = 8). Complete remission (CR) of lymphoma was achieved in 31 patients (65%). The overall survival and event-free survival rates after 5 years were 93% and 68%, respectively. The t(14;18)/IGH-BCL2 was detected in 39 patients (81%). The involvement of multiple sites (69% vs 0%), manifestation of the lymphomatous polyposis type (72% vs 22%), and histologic grade 1 or 2 tumors (92% vs 56%) were more frequent in the t(14;18)-positive group than in the negative group. In addition, the CR rate was lower in the t(14;18)-positive group than in the negative group (56% vs 100%; P = .0179), and a trend was observed toward poorer event-free survival in the positive group (P = .089). CONCLUSIONS The t(14;18)/IGH-BCL2 chromosomal translocation occurred frequently in gastrointestinal follicular lymphomas. The current results indicated that this translocation may be a predictor of an adverse clinical course.
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Affiliation(s)
- Shunichi Yanai
- Department of Medicine and Clinical Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
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van de Water JMW, Cillessen SAGM, Visser OJ, Verbeek WHM, Meijer CJLM, Mulder CJJ. Enteropathy associated T-cell lymphoma and its precursor lesions. Best Pract Res Clin Gastroenterol 2010; 24:43-56. [PMID: 20206108 DOI: 10.1016/j.bpg.2009.11.002] [Citation(s) in RCA: 78] [Impact Index Per Article: 5.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/18/2009] [Accepted: 11/16/2009] [Indexed: 01/31/2023]
Abstract
Enteropathy Associated T-cell Lymphoma (EATL) is an intestinal tumour of intra-epithelial lymphocytes. Based on morphology, immunohistochemistry and genetic profile EATL can be divided into two groups. EATL type I is a large cell lymphoma which is highly associated with Coeliac Disease (CD) and mostly presents with malabsorption, weight loss and CD-related symptoms. EATL type II consists of small to medium-sized cells and presents often with obstruction or perforation of the small bowel. This type of EATL has no known association with CD. When EATL has been diagnosed a thorough diagnostic work-up is needed. This work-up preferably includes video capsule enteroscopy (VCE), double-balloon enteroscopy (DBE), computed tomography (CT) combined with 18F-fluorodeoxyglucose positron emission tomography scan (18F-FDG-PET scan) if possible and magnetic resonance enteroclysis (MRE). Nowadays, most EATL patients are treated with chemotherapy mostly preceded by resection of the tumour and followed by stem cell transplantation. Despite these therapies outcome of EATL remains very poor with a 5-year survival of 8-20%. In order to improve survival prospective multicentre trials, studying new therapies are needed. The combination of chemotherapy, monoclonal antibodies and/or apoptosis inducing small molecules might be a potential treatment for EATL in the (nearby) future.
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Affiliation(s)
- Jolanda M W van de Water
- Department of Gastroenterology and Hepatology, VU University Medical Centre, Amsterdam, The Netherlands.
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Wakabayashi S, Arai A, Oshikawa G, Araki A, Watanabe M, Uchida N, Taniguchi S, Miura O. Extranodal NK/T cell lymphoma, nasal type, of the small intestine diagnosed by double-balloon endoscopy. Int J Hematol 2009; 90:605-610. [PMID: 19936878 DOI: 10.1007/s12185-009-0438-7] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/31/2009] [Revised: 09/30/2009] [Accepted: 10/13/2009] [Indexed: 12/15/2022]
Abstract
Extranodal NK/T-cell lymphoma (ENKL), nasal type, is rare and the small intestine is quite extraordinary as a primary lesion site. We report a 47-year-old man with ENKL of the small intestine. He was referred to our hospital because of bloody stool and the diagnosis was made by double-balloon endoscopy (DBE) of the small intestine without surgical procedure. His clinical stage was IVB and he was categorized in group 4 by prognostic index of ENKL. He went into complete remission (CR) after intensive chemotherapy (DeVIC) and subsequently underwent allogeneic bone marrow transplantation (BMT). Although he remained in CR for about 8 months after BMT, he died of disease recurrence 14 months after the diagnosis was made. ENKL of the small intestine follows a highly aggressive course. We describe the usefulness of DBE for diagnosis and management for ENKL of the small intestine. Additional cases, however, should be accumulated to establish optimal treatment strategy.
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Affiliation(s)
- Shihoko Wakabayashi
- Department of Hematology, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyo-ku, Tokyo, 113-8519, Japan
| | - Ayako Arai
- Department of Hematology, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyo-ku, Tokyo, 113-8519, Japan.
| | - Gaku Oshikawa
- Department of Hematology, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyo-ku, Tokyo, 113-8519, Japan
| | - Akihiro Araki
- Department of Gastroenterology and Hepatology, Graduate School of Medicine, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyo-ku, Tokyo, 113-8519, Japan
| | - Mamoru Watanabe
- Department of Gastroenterology and Hepatology, Graduate School of Medicine, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyo-ku, Tokyo, 113-8519, Japan
| | - Naoyuki Uchida
- Department of Hematology, Toranomon Hospital, 2-2-2 Toranomon, Minato-ku, Tokyo, 105-8470, Japan
| | - Shuichi Taniguchi
- Department of Hematology, Toranomon Hospital, 2-2-2 Toranomon, Minato-ku, Tokyo, 105-8470, Japan
| | - Osamu Miura
- Department of Hematology, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyo-ku, Tokyo, 113-8519, Japan
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