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1
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Bosch-Schips J, Parisi X, Climent F, Vega F. Bridging clinicopathologic features and genetics in follicular lymphoma: Towards enhanced diagnostic accuracy and subtype differentiation. Hum Pathol 2025; 156:105676. [PMID: 39490765 DOI: 10.1016/j.humpath.2024.105676] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/01/2024] [Accepted: 10/23/2024] [Indexed: 11/05/2024]
Abstract
Follicular lymphoma (FL) is a neoplasm that originates from germinal center B cells and typically forms at least a partial follicular pattern. Approximately 85% of FL cases harbor the t(14;18)(q32;q21)/IGH::BCL2 which leads to the overexpression of BCL2. These cases are referred to as classic FL in the current World Health Organization classification [1]. These neoplasms often exhibit hallmark epigenetic deregulation due to recurrent mutations in genes such as KMT2D, CREBBP, and EZH2, with KMT2D and CREBBP considered founding events in FL lymphomagenesis. In contrast, about 15% of FL cases are negative for the t(14;18), which could present diagnostic challenges. These cases may lack the typical genetic markers and require careful pathological and molecular analysis for accurate diagnosis. This review aims to provide an up-to-date pathology resource on FL, focusing on the pathological and molecular characteristics of these neoplasms. We will detail the diagnostic criteria for FL and emphasize the importance of genetic and mutational analyses in accurately characterizing and distinguishing FL subtypes. Furthermore, we will propose methodologies and best practices for the diagnostic work-up of FL to enhance diagnostic accuracy.
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Affiliation(s)
- Jan Bosch-Schips
- Department of Pathology, Hospital Universitari de Bellvitge, University of Barcelona, L'Hospitalet de Llobregat, Barcelona, Spain
| | - Xenia Parisi
- Hematopathology, Division of Pathology and Laboratory Medicine, The University of Texas MD Anderson Cancer Center, Houston, TX, 77030, USA
| | - Fina Climent
- Department of Pathology, Hospital Universitari de Bellvitge, University of Barcelona, L'Hospitalet de Llobregat, Barcelona, Spain.
| | - Francisco Vega
- Hematopathology, Division of Pathology and Laboratory Medicine, The University of Texas MD Anderson Cancer Center, Houston, TX, 77030, USA.
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2
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Delabie J, Sakhdari A. Indolent clonal lymphoid disorders. Hum Pathol 2025; 156:105715. [PMID: 39793932 DOI: 10.1016/j.humpath.2025.105715] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/25/2024] [Revised: 12/31/2024] [Accepted: 01/06/2025] [Indexed: 01/13/2025]
Abstract
Indolent clonal lymphoid disorders are not recognized as lymphomas as they generally need no systemic treatment, and depending on the lesion, need only limited clinical follow-up. These lesions are usually incidentally diagnosed during the work up for other disease. The recognition of indolent clonal lymphoid disorders is important to avoid misdiagnosis as lymphoma and unnecessary treatment. Notwithstanding, some indolent disorders, especially B-cell disorders, may give important morbidity that is not related to disease burden but related to auto-immune disease which may need treatment. Further, some of these lesions may, at various rates, ultimately progress to lymphoma. As such, the indolent clonal lymphoid disorders also give an insight into the earliest stages of clonal lymphoid disease that may increase our understanding of lymphoma, although much needs yet to be elucidated. In this article both B- and T-cell indolent clonal lymphoid disorders are reviewed. Not included in this review are lymphoid lesions that may be mistaken for lymphoma, but are not clonal, such as indolent T-lymphoblastic proliferation or marginal zone hyperplasia with immunoglobulin light chain restriction. Further, an emphasis has been given to clonal lymphoid lesions and therefore indolent plasma cell lesions have not been included. Also excluded is indolent lymphoma that may not need treatment but nonetheless requires more regular follow up. One may rightfully argue that there may be a gray zone between what constitutes an indolent clonal lymphoid disorder and an indolent lymphoma. This discussion is reflected in the different terminology used for some entities between editions of the WHO classification and between the Fifth Edition of the WHO Classification and the International Consensus Classification (ICC). The former has been used as a selection basis for this review, but cross-reference has been made to the ICC nomenclature when that differs as well as to the earlier Revised Fourth Edition of the WHO Classification (WHO-r4). For this reason, indolent T-cell lymphoma of the gastrointestinal tract (ICC: indolent clonal T-cell lymphoproliferative disorder of the gastrointestinal tract) is not included in this review.
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Affiliation(s)
- Jan Delabie
- University Health Network and University of Toronto, Canada.
| | - Ali Sakhdari
- University Health Network and University of Toronto, Canada
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3
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Kang D, Min GJ, Kim TY, Jeon YW, Cho Y, Park JM, O JH, Choi BO, Park G, Cho SG. The Role of Small Bowel Capsule Endoscopy in Determining the Treatment Strategy for Duodenal Follicular Lymphoma: A Single-Center Retrospective Study. Diagnostics (Basel) 2025; 15:193. [PMID: 39857077 PMCID: PMC11765495 DOI: 10.3390/diagnostics15020193] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/02/2024] [Revised: 01/04/2025] [Accepted: 01/13/2025] [Indexed: 01/27/2025] Open
Abstract
Objectives: In this single-center retrospective study, we aimed to verify the extent of duodenal follicular lymphoma (DFL) and investigate the role and clinical significance of video capsule endoscopy (VCE) in the treatment process. Methods: We analyzed the clinical and imaging data of 40 patients diagnosed with DFL. Results: Imaging workup and bone marrow biopsies revealed DFL only in the gastrointestinal tract (stage I) in 22 patients and in local lymph nodes (stage II1), distant lymph nodes (stage II2), pancreas (stage II2Epancreas), and extranodal regions (stage IV) in 1, 3, 1, and 13 patients, respectively. Fifteen of the 23 patients with localized (stages I and II1) DFL underwent VCE for comprehensive small bowel evaluation, which revealed lesion extension beyond the duodenum in 10 patients (66.7%). A watch-and-wait strategy was implemented for one patient and systemic chemotherapy was administered to the remaining nine. Of the eight patients without VCE, seven and one received radiotherapy and observation, respectively. Nine of the 23 patients (39.1%) received systemic treatment based on positive VCE results. Only one of the 17 patients with advanced-stage DFL (stages II2 and IV) accepted radiotherapy; 16 underwent systemic chemotherapy. During follow-up (median, 48.4 months), two relapse events occurred in the advanced stage, with no lymphoma-associated deaths. DFL tends to be indolent and has favorable outcomes. Conclusions: Proactive VCE for diagnosing DFL is recommended to determine small bowel involvement, which may influence subsequent treatment decisions.
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Affiliation(s)
- Donghoon Kang
- Department of Gastroenterology and Hepatology, Catholic University Lymphoma Group, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Banpo-daero 222, Seocho-Gu, Seoul 06591, Republic of Korea; (D.K.); (Y.C.); (J.M.P.)
| | - Gi-June Min
- Department of Hematology, Catholic University Lymphoma Group, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Banpo-daero 222, Seocho-Gu, Seoul 06591, Republic of Korea;
| | - Tong Yoon Kim
- Department of Hematology, Catholic University Lymphoma Group, Yeouido St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, 10 63-ro, Yeongdeungpo-gu, Seoul 07345, Republic of Korea; (T.Y.K.); (Y.-W.J.)
| | - Young-Woo Jeon
- Department of Hematology, Catholic University Lymphoma Group, Yeouido St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, 10 63-ro, Yeongdeungpo-gu, Seoul 07345, Republic of Korea; (T.Y.K.); (Y.-W.J.)
| | - Yukyung Cho
- Department of Gastroenterology and Hepatology, Catholic University Lymphoma Group, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Banpo-daero 222, Seocho-Gu, Seoul 06591, Republic of Korea; (D.K.); (Y.C.); (J.M.P.)
| | - Jae Myung Park
- Department of Gastroenterology and Hepatology, Catholic University Lymphoma Group, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Banpo-daero 222, Seocho-Gu, Seoul 06591, Republic of Korea; (D.K.); (Y.C.); (J.M.P.)
| | - Joo Hyun O
- Department of Nuclear Medicine, Catholic University Lymphoma Group, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Banpo-daero 222, Seocho-Gu, Seoul 06591, Republic of Korea;
| | - Byung-Ock Choi
- Department of Radiation Oncology, Catholic University Lymphoma Group, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Banpo-daero 222, Seocho-Gu, Seoul 06591, Republic of Korea;
| | - Gyeongsin Park
- Department of Hospital Pathology, Catholic University Lymphoma Group, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Banpo-daero 222, Seocho-Gu, Seoul 06591, Republic of Korea;
| | - Seok-Goo Cho
- Department of Hematology, Catholic University Lymphoma Group, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Banpo-daero 222, Seocho-Gu, Seoul 06591, Republic of Korea;
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4
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Duffles G, Braga A, Silveira T, Novis Y, Arrais C, Tucunduva L, Fonseca AR, Borducchi D, Neffa P, Blumm F, Gonçalves MDC, Moreira F, Nucci F, Rego E, Rocha V. Clinical characteristics and outcomes of Brazilian patients with duodenal-type follicular lymphoma: a multicenter retrospective study. Leuk Lymphoma 2025; 66:95-101. [PMID: 39257204 DOI: 10.1080/10428194.2024.2401098] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/11/2024] [Revised: 08/30/2024] [Accepted: 09/01/2024] [Indexed: 09/12/2024]
Abstract
Duodenal-type follicular lymphoma (DFL) is a rare subtype classified by the 5th edition of the WHO and international consensus classifications of lymphoid neoplasms, typically presenting as localized disease with favorable outcomes. This multicenter retrospective study examines 53 Brazilian DFL patients with a median age of 58.2 years (33-85), with males comprising 50% (n = 27). According to Lugano GI tract classification, 40 patients (75%) were stage I. Median follow-up was 2.9 years (range 0.1-11). Incidental diagnosis occurred in 28 patients (52.8%) during routine endoscopy; 24 patients (45%) presented mild gastrointestinal symptoms. Treatments included watchful waiting (32 patients, 60.4%), rituximab monotherapy (15 patients, 28.3%), radiotherapy (three patients, 5.7%), and chemoimmunotherapy (three patients, 5.7%). Three patients experienced disease progression; watchful waiting showed three spontaneous remissions. No deaths occurred during follow-up. This study, the first from Latin America, demonstrates a good prognosis across treatments, highlighting Watchful waiting's effectiveness.
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Affiliation(s)
- Guilherme Duffles
- Rede Dor Sao Luiz, Sao Paulo, Brazil
- University of Campinas, Hematology and Hemotherapy Centre, Sao Paulo, Brazil
| | - Arthur Braga
- Hematology, AC Camargo Cancer Center, Sao Paulo, Brazil
| | | | - Yana Novis
- Hematology, Hospital Sirio-Libanes, Sao Paulo, Brazil
| | | | | | | | - Davimar Borducchi
- Rede Dor Sao Luiz, Sao Paulo, Brazil
- Centro Universitário da Faculdade de medicina do ABC, Sao Paulo, Brazil
| | - Pedro Neffa
- Rede Dor Sao Luiz, Sao Paulo, Brazil
- Instituto do câncer do estado de São Paulo, ICESP, Sao Paulo, Brazil
| | | | | | - Frederico Moreira
- Department of Hematology, Hemotherapy & Cell Therapy, Faculty of Medicine, University of São Paulo (FMUSP), Sao Paulo, Brazil
| | - Fabio Nucci
- Rede Dor Sao Luiz, Sao Paulo, Brazil
- Hospital universitário Antônio Pedro, Universidade Federal Fluminense, UFF, Niterói, Rio de Janeiro, Brazil
| | - Eduardo Rego
- Department of Hematology, Hemotherapy & Cell Therapy, Faculty of Medicine, University of São Paulo (FMUSP), Sao Paulo, Brazil
- Instituto Dor de pesquisa e Ensino, IDOR, Sao Paulo, Brazil
| | - Vanderson Rocha
- Rede Dor Sao Luiz, Sao Paulo, Brazil
- Department of Hematology, Hemotherapy & Cell Therapy, Faculty of Medicine, University of São Paulo (FMUSP), Sao Paulo, Brazil
- Laboratory of Medical Investigation in Pathogenesis and Directed Therapy in Onco-Immuno-Hematology (LIM-31), University of São Paulo (USP), Sao Paulo, SP, Brazil
- Churchill Hospital, Oxford University, Oxford, United Kingdom
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5
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Ng LCK, Ong SY, Huang X, Ghosh A, Nagarajan C, Goh YT, Chen Y, Lim FLWI, Tan MSY, Lee XH, Tan JY, Grigoropoulos NF, Yang VS, Hoe JTM, Chiang J, Chang EWY, Poon EYL, Tan YH, Somasundaram N, Tao M, Lim ST, Chan JY. Outcome of extra-nodal follicular lymphoma affected by choice of induction chemoimmunotherapy and maintenance rituximab - real-world retrospective study. Leuk Lymphoma 2024:1-7. [PMID: 39731562 DOI: 10.1080/10428194.2024.2446615] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/29/2024] [Revised: 12/03/2024] [Accepted: 12/18/2024] [Indexed: 12/30/2024]
Abstract
The importance of extra-nodal disease has been well recognized in follicular lymphoma, and is incorporated into various prognostic tools. However, the optimal treatment strategy for this subgroup remains unclear. In this multicenter retrospective study, we analyzed 143 patients who received either R-CHOP or Bendamustine-Rituximab (BR), with a median follow-up of 69.7 months. Our findings indicate that extra-nodal disease confers poorer PFS (5-year PFS 58% vs 66%, p = 0.03) while high-risk relapse predict poorer OS (5-year OS 56% vs 94%, p < 0.001). Subgroup analysis on 98 patients with extra-nodal disease revealed that BR induction conferred superior PFS compared to R-CHOP (HR 0.49, p = 0.032) while maintenance rituximab also improved PFS (HR 0.44, p = 0.033). Patients who received R-CHOP without maintenance rituximab had significantly worse PFS (5-year PFS 41% vs 68%, p = 0.005) and OS (5-year OS 83% vs 100%, p = 0.007) compared to those with maintenance rituximab. Role of maintenance rituximab post BR remained unclear. In this retrospective study of follicular lymphoma patients with extra-nodal disease, BR induction yielded favorable PFS compared to R-CHOP and could be a preferred treatment approach. Maintenance rituximab after R-CHOP significantly improve PFS and OS and should be considered in all appropriate patients.
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Affiliation(s)
- Lawrence Cheng Kiat Ng
- Department of Hematology, Singapore General Hospital, Singapore, Singapore
- Department of Medical Oncology, National Cancer Centre Singapore, Singapore, Singapore
| | - Shin Yeu Ong
- Department of Hematology, Singapore General Hospital, Singapore, Singapore
- Department of Medical Oncology, National Cancer Centre Singapore, Singapore, Singapore
| | - Xinxin Huang
- Department of Hematology, Singapore General Hospital, Singapore, Singapore
| | - Aditi Ghosh
- Department of Hematology, Singapore General Hospital, Singapore, Singapore
- Department of Medical Oncology, National Cancer Centre Singapore, Singapore, Singapore
| | - Chandramouli Nagarajan
- Department of Hematology, Singapore General Hospital, Singapore, Singapore
- Department of Medical Oncology, National Cancer Centre Singapore, Singapore, Singapore
| | - Yeow Tee Goh
- Department of Hematology, Singapore General Hospital, Singapore, Singapore
- Department of Medical Oncology, National Cancer Centre Singapore, Singapore, Singapore
| | - Yunxin Chen
- Department of Hematology, Singapore General Hospital, Singapore, Singapore
- Department of Medical Oncology, National Cancer Centre Singapore, Singapore, Singapore
| | - Francesca Lorraine Wei Inng Lim
- Department of Hematology, Singapore General Hospital, Singapore, Singapore
- Department of Medical Oncology, National Cancer Centre Singapore, Singapore, Singapore
| | - Melinda Si Yun Tan
- Department of Hematology, Singapore General Hospital, Singapore, Singapore
- Department of Medical Oncology, National Cancer Centre Singapore, Singapore, Singapore
| | - Xiu Hue Lee
- Department of Hematology, Singapore General Hospital, Singapore, Singapore
- Department of Medical Oncology, National Cancer Centre Singapore, Singapore, Singapore
| | - Jing Yuan Tan
- Department of Hematology, Singapore General Hospital, Singapore, Singapore
- Department of Medical Oncology, National Cancer Centre Singapore, Singapore, Singapore
| | - Nicholas Francis Grigoropoulos
- Department of Hematology, Singapore General Hospital, Singapore, Singapore
- Department of Medical Oncology, National Cancer Centre Singapore, Singapore, Singapore
| | - Valerie Shiwen Yang
- Department of Medical Oncology, National Cancer Centre Singapore, Singapore, Singapore
| | - Joshua Tian Ming Hoe
- Department of Medical Oncology, National Cancer Centre Singapore, Singapore, Singapore
| | - Jianbang Chiang
- Department of Medical Oncology, National Cancer Centre Singapore, Singapore, Singapore
| | - Esther Wei Yin Chang
- Department of Medical Oncology, National Cancer Centre Singapore, Singapore, Singapore
| | - Eileen Yi Ling Poon
- Department of Medical Oncology, National Cancer Centre Singapore, Singapore, Singapore
| | - Ya Hwee Tan
- Department of Medical Oncology, National Cancer Centre Singapore, Singapore, Singapore
| | | | - Miriam Tao
- Department of Medical Oncology, National Cancer Centre Singapore, Singapore, Singapore
| | - Soon Thye Lim
- Department of Medical Oncology, National Cancer Centre Singapore, Singapore, Singapore
| | - Jason Yongsheng Chan
- Department of Medical Oncology, National Cancer Centre Singapore, Singapore, Singapore
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6
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Hagen R, Tidwell J, Weng E, Goldenberg SA, Shaikh A. Duodenal Polyposis: An Incidental Finding of Duodenal-Type Follicular Lymphoma. Cureus 2024; 16:e73237. [PMID: 39650864 PMCID: PMC11624914 DOI: 10.7759/cureus.73237] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 11/06/2024] [Indexed: 12/11/2024] Open
Abstract
Duodenal-type follicular lymphoma is a newly recognized and rare variant of follicular lymphoma with a good prognosis. Patients may present with non-specific gastrointestinal symptoms, but they are often asymptomatic. Diagnosis usually occurs incidentally during EGD when duodenal polyps are biopsied. We describe the unique case of incidentally found biopsy-proven duodenal-type B cell follicular lymphoma. Secondary involvement by systemic type B-cell lymphoma was excluded. The patient underwent localized radiotherapy, obtaining complete remission six months later, confirmed by pathology. Physicians should be aware of this rare pathology, considering the potential for extra-duodenal systemic B-cell lymphoma.
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Affiliation(s)
- Rachael Hagen
- Internal Medicine, University of Connecticut, Farmington, USA
| | - Jasmine Tidwell
- Internal Medicine, University of Connecticut Health, Farmington, USA
| | - Emily Weng
- Gastroenterology and Hepatology, University of Connecticut Health, Farmington, USA
| | - Steven A Goldenberg
- Gastroenterology and Hepatology, University of Connecticut Health, Farmington, USA
| | - Anjiya Shaikh
- Gastroenterology and Hepatology, Yale New Haven Hospital, New Haven, USA
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7
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Alnughmush A, Fakih RE, Alyamany R, Bakshi N, Alhayli S, Aljurf M. Duodenal-type follicular lymphoma: comprehensive insights into disease characteristics and established treatment strategies. Curr Opin Oncol 2024; 36:577-582. [PMID: 39246163 DOI: 10.1097/cco.0000000000001093] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 09/10/2024]
Abstract
PURPOSE OF REVIEW This review aims to detail the characteristics and outcomes of duodenal-type follicular lymphoma (DTFL), a rare lymphoma variant. It focuses on integrating recent reports in treatment modalities and highlights emerging insights into the unique biological features of the disease. RECENT FINDING Recent studies confirm the indolent nature of DTFL, with extended follow-up periods showing favorable outcomes under watchful waiting strategies and a notable proportion of patients experiencing spontaneous remission. Additionally, advancements in understanding the disease's biology revealed that the tumor microenvironment is marked by specific genomic expressions indicative of chronic inflammation. SUMMARY The observations of spontaneous resolution and the generally favorable progression of DTFL call for a conservative approach in initiating treatment. Clinical management should judiciously consider the disease's typically benign course against the potential risks of intervention, promoting customized treatment protocols tailored for cases with clinical necessity. Additionally, the discovery of an inflammatory tumor microenvironment and molecular evidence suggesting an antigen-driven process highlight critical areas for future research.
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Affiliation(s)
- Ahmed Alnughmush
- Department of Hematology, Stem Cell Transplant and Cellular Therapy, Oncology Centre
| | - Riad El Fakih
- Department of Hematology, Stem Cell Transplant and Cellular Therapy, Oncology Centre
| | - Ruah Alyamany
- Department of Hematology, Stem Cell Transplant and Cellular Therapy, Oncology Centre
| | - Nasir Bakshi
- Department of Pathology and Laboratory Medicine, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia
| | - Saud Alhayli
- Department of Hematology, Stem Cell Transplant and Cellular Therapy, Oncology Centre
| | - Mahmoud Aljurf
- Department of Hematology, Stem Cell Transplant and Cellular Therapy, Oncology Centre
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8
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Huang RW, Jia L, Sun YC, Liu YP. Duodenal-type follicular lymphoma: Report of four cases and review of the literature. WORLD CHINESE JOURNAL OF DIGESTOLOGY 2024; 32:538-544. [DOI: 10.11569/wcjd.v32.i7.538] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 12/29/2024]
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9
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Iwamuro M, Tanaka T, Ennishi D, Otsuka M. Recent updates on treatment options for primary follicular lymphoma of the gastrointestinal tract. Expert Rev Gastroenterol Hepatol 2024; 18:367-375. [PMID: 38997226 DOI: 10.1080/17474124.2024.2380337] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/05/2024] [Accepted: 07/11/2024] [Indexed: 07/14/2024]
Abstract
BACKGROUND Primary gastrointestinal follicular lymphoma is a subtype of follicular lymphoma that originates directly from the gastrointestinal tract. Pathologically, it exhibits substantial similarities with the secondary gastrointestinal involvement observed in nodal follicular lymphoma. However, primary gastrointestinal follicular lymphoma presents clinically distinct features, necessitating divergent considerations in treatment selection compared with nodal follicular lymphoma. AREAS COVERED This narrative review focused on recent articles (2018-2023) regarding the long-term prognosis and treatment options for gastrointestinal follicular lymphoma. In addition, a brief overview of gastrointestinal follicular lymphomas is provided. EXPERT OPINION Patients with primary gastrointestinal follicular lymphoma often present with a low tumor burden. Lymphoma lesions typically remain asymptomatic for several years or may undergo spontaneous regression without immediate treatment. Therefore, a 'watch and wait' approach is justified. Conversely, when large tumor masses are identified in the gastrointestinal tract, the potential for tumor bleeding or intestinal obstruction requires timely therapeutic interventions.
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Affiliation(s)
- Masaya Iwamuro
- Department of Gastroenterology and Hepatology, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama, Japan
| | - Takehiro Tanaka
- Department of Pathology, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama, Japan
| | - Daisuke Ennishi
- Department of Hematology and Oncology, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama, Japan
| | - Motoyuki Otsuka
- Department of Gastroenterology and Hepatology, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama, Japan
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10
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Li X, Gao F, Meng X, Zhang X, Sun C, Liu H, Yu J, Liu X, Han X, Li L, Qiu L, Qian Z, Zhou S, Gong W, Golchehre Z, Chavoshzadeh Z, Ren X, Wang X, Zhang H. Epidemiological features and prognosis for primary gastrointestinal follicular lymphoma. Br J Haematol 2024; 204:1771-1779. [PMID: 38447995 DOI: 10.1111/bjh.19393] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/02/2023] [Revised: 02/26/2024] [Accepted: 02/27/2024] [Indexed: 03/08/2024]
Abstract
Primary gastrointestinal follicular lymphoma (PGI-FL) is a rare extra-nodal lymphoma. Its epidemiology and prognosis remain unclear. We performed a retrospective analysis of eligible patients with 1648 PGI-FL and 34 892 nodal FL (N-FL) in the Surveillance, Epidemiology and End Results (SEER) database. The age-adjusted average annual incidence of PGI-FL was 0.111/100000. The median overall survival (OS) for PGI-FL and N-FL patients was 207 and 165 months respectively. The 5-year diffuse large B-cell lymphoma (DLBCL) transformation rates were 2.1% and 2.6% respectively. Age, sex, grade, Ann Arbor stage, primary site and radiation were independent prognostic factors (p < 0.05). Nomograms were constructed to predict 1-, 5- and 10-year OS and disease-specific survival (DSS). The receiver operating characteristic curves and calibration plots showed the established nomograms had robust and accurate performance. Patients were classified into three risk groups according to nomogram score. In conclusion, the incidence of PGI-FL has increased over the past 40 years, and PGI-FL has a better prognosis and a lower DLBCL transformation rate than N-FL. The nomograms were developed and validated as an individualized tool to predict survival. Patients were divided into three risk groups to assist clinicians in identifying high-risk patients and choosing the optimal individualized treatments.
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MESH Headings
- Humans
- Lymphoma, Follicular/mortality
- Lymphoma, Follicular/epidemiology
- Lymphoma, Follicular/therapy
- Lymphoma, Follicular/diagnosis
- Female
- Male
- Middle Aged
- Aged
- Gastrointestinal Neoplasms/epidemiology
- Gastrointestinal Neoplasms/mortality
- Gastrointestinal Neoplasms/diagnosis
- Gastrointestinal Neoplasms/therapy
- Adult
- Retrospective Studies
- Prognosis
- SEER Program
- Aged, 80 and over
- Nomograms
- Incidence
- Lymphoma, Large B-Cell, Diffuse/epidemiology
- Lymphoma, Large B-Cell, Diffuse/mortality
- Lymphoma, Large B-Cell, Diffuse/diagnosis
- Lymphoma, Large B-Cell, Diffuse/therapy
- Adolescent
- Young Adult
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Affiliation(s)
- Xuelei Li
- Department of Lymphoma and National Key Laboratory of Druggability Evaluation and Systematic Translational Medicine, Tianjin Medical University Cancer Institute and Hospital, National Clinical Research Center for Cancer, Tianjin's Clinical Research Center for Cancer, Key Laboratory of Cancer Prevention and Therapy, The Sino-US Center for Lymphoma and Leukemia Research, Tianjin, China
| | - Fenghua Gao
- Department of Lymphoma and National Key Laboratory of Druggability Evaluation and Systematic Translational Medicine, Tianjin Medical University Cancer Institute and Hospital, National Clinical Research Center for Cancer, Tianjin's Clinical Research Center for Cancer, Key Laboratory of Cancer Prevention and Therapy, The Sino-US Center for Lymphoma and Leukemia Research, Tianjin, China
| | - Xiangrui Meng
- Department of Lymphoma and National Key Laboratory of Druggability Evaluation and Systematic Translational Medicine, Tianjin Medical University Cancer Institute and Hospital, National Clinical Research Center for Cancer, Tianjin's Clinical Research Center for Cancer, Key Laboratory of Cancer Prevention and Therapy, The Sino-US Center for Lymphoma and Leukemia Research, Tianjin, China
| | - Xiaoyan Zhang
- State Key Laboratory of Experimental Hematology and Division of Pediatric Blood Diseases Center, Institute of Hematology and Blood Diseases Hospital, Peking Union Medical College, Chinese Academy of Medical Sciences, Tianjin, China
| | - Cong Sun
- Department of Lymphoma and National Key Laboratory of Druggability Evaluation and Systematic Translational Medicine, Tianjin Medical University Cancer Institute and Hospital, National Clinical Research Center for Cancer, Tianjin's Clinical Research Center for Cancer, Key Laboratory of Cancer Prevention and Therapy, The Sino-US Center for Lymphoma and Leukemia Research, Tianjin, China
| | - Hengqi Liu
- Department of Lymphoma and National Key Laboratory of Druggability Evaluation and Systematic Translational Medicine, Tianjin Medical University Cancer Institute and Hospital, National Clinical Research Center for Cancer, Tianjin's Clinical Research Center for Cancer, Key Laboratory of Cancer Prevention and Therapy, The Sino-US Center for Lymphoma and Leukemia Research, Tianjin, China
| | - Jingwei Yu
- Department of Lymphoma and National Key Laboratory of Druggability Evaluation and Systematic Translational Medicine, Tianjin Medical University Cancer Institute and Hospital, National Clinical Research Center for Cancer, Tianjin's Clinical Research Center for Cancer, Key Laboratory of Cancer Prevention and Therapy, The Sino-US Center for Lymphoma and Leukemia Research, Tianjin, China
| | - Xia Liu
- Department of Lymphoma and National Key Laboratory of Druggability Evaluation and Systematic Translational Medicine, Tianjin Medical University Cancer Institute and Hospital, National Clinical Research Center for Cancer, Tianjin's Clinical Research Center for Cancer, Key Laboratory of Cancer Prevention and Therapy, The Sino-US Center for Lymphoma and Leukemia Research, Tianjin, China
| | - Xue Han
- Department of Lymphoma and National Key Laboratory of Druggability Evaluation and Systematic Translational Medicine, Tianjin Medical University Cancer Institute and Hospital, National Clinical Research Center for Cancer, Tianjin's Clinical Research Center for Cancer, Key Laboratory of Cancer Prevention and Therapy, The Sino-US Center for Lymphoma and Leukemia Research, Tianjin, China
| | - Lanfang Li
- Department of Lymphoma and National Key Laboratory of Druggability Evaluation and Systematic Translational Medicine, Tianjin Medical University Cancer Institute and Hospital, National Clinical Research Center for Cancer, Tianjin's Clinical Research Center for Cancer, Key Laboratory of Cancer Prevention and Therapy, The Sino-US Center for Lymphoma and Leukemia Research, Tianjin, China
| | - Lihua Qiu
- Department of Lymphoma and National Key Laboratory of Druggability Evaluation and Systematic Translational Medicine, Tianjin Medical University Cancer Institute and Hospital, National Clinical Research Center for Cancer, Tianjin's Clinical Research Center for Cancer, Key Laboratory of Cancer Prevention and Therapy, The Sino-US Center for Lymphoma and Leukemia Research, Tianjin, China
| | - Zhengzi Qian
- Department of Lymphoma and National Key Laboratory of Druggability Evaluation and Systematic Translational Medicine, Tianjin Medical University Cancer Institute and Hospital, National Clinical Research Center for Cancer, Tianjin's Clinical Research Center for Cancer, Key Laboratory of Cancer Prevention and Therapy, The Sino-US Center for Lymphoma and Leukemia Research, Tianjin, China
| | - Shiyong Zhou
- Department of Lymphoma and National Key Laboratory of Druggability Evaluation and Systematic Translational Medicine, Tianjin Medical University Cancer Institute and Hospital, National Clinical Research Center for Cancer, Tianjin's Clinical Research Center for Cancer, Key Laboratory of Cancer Prevention and Therapy, The Sino-US Center for Lymphoma and Leukemia Research, Tianjin, China
| | - Wenchen Gong
- Department of Pathology, Tianjin Medical University Cancer Institute and Hospital, Tianjin, China
| | - Zahra Golchehre
- Department of Medical Genetics, Tehran University of Medical Sciences, Tehran, Iran
| | - Zahra Chavoshzadeh
- Department of Immunology/Allergy, Pediatric Infections Research Center, Mofid Children's Hospital, Shahid Beheshti University of Medical Sciences, Tehran, Iran
| | - Xiubao Ren
- Department of Immunology/Biotherapy, Tianjin Medical University Cancer Institute and Hospital, Tianjin, China
| | - Xianhuo Wang
- Department of Lymphoma and National Key Laboratory of Druggability Evaluation and Systematic Translational Medicine, Tianjin Medical University Cancer Institute and Hospital, National Clinical Research Center for Cancer, Tianjin's Clinical Research Center for Cancer, Key Laboratory of Cancer Prevention and Therapy, The Sino-US Center for Lymphoma and Leukemia Research, Tianjin, China
| | - Huilai Zhang
- Department of Lymphoma and National Key Laboratory of Druggability Evaluation and Systematic Translational Medicine, Tianjin Medical University Cancer Institute and Hospital, National Clinical Research Center for Cancer, Tianjin's Clinical Research Center for Cancer, Key Laboratory of Cancer Prevention and Therapy, The Sino-US Center for Lymphoma and Leukemia Research, Tianjin, China
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11
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Gordon MJ, Duan Z, Zhao H, Nastoupil L, Iyer S, Ferrajoli A, Danilov AV, Giordano SH. Comparison of Comorbidity Models Within a Population-Based Cohort of Older Adults With Non-Hodgkin Lymphoma. JCO Clin Cancer Inform 2024; 8:e2300223. [PMID: 38684043 PMCID: PMC11476108 DOI: 10.1200/cci.23.00223] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/25/2023] [Revised: 01/25/2024] [Accepted: 03/08/2024] [Indexed: 05/02/2024] Open
Abstract
PURPOSE Compare the association of individual comorbidities, comorbidity indices, and survival in older adults with non-Hodgkin lymphoma (NHL), including in specific NHL subtypes. METHODS Data source was SEER-Medicare, a population-based registry of adults age 65 years and older with cancer. We included all incident cases of NHL diagnosed during 2008-2017 who met study inclusion criteria. Comorbidities were classified using the three-factor risk estimate scale (TRES), Charlson comorbidity index (CCI), and National Cancer Institute (NCI) comorbidity index categories and weights. Overall survival (OS) and lymphoma-specific survival, with death from other causes treated as a competing risk, were estimated using the Kaplan-Meier method from time of diagnosis. Multivariable Cox models were constructed, and Harrel C-statistics were used to compare comorbidity models. A two-sided P value of <.05 was considered significant. RESULTS A total of 40,486 patients with newly diagnosed NHL were included. Patients with aggressive NHL had higher rates of baseline comorbidity. Despite differences in baseline comorbidity between NHL subtypes, cardiovascular, pulmonary, diabetes, and renal comorbidities were frequent and consistently associated with OS in most NHL subtypes. These categories were used to construct a candidate comorbidity score, the non-Hodgkin lymphoma 5 (NHL-5). Comparing three validated comorbidity scores, TRES, CCI, NCI, and the novel NHL-5 score, we found similar associations with OS and lymphoma-specific survival, which was confirmed in sensitivity analyses by NHL subtypes. CONCLUSION The optimal measure of comorbidity in NHL is unknown. Here, we demonstrate that the three-category TRES and five-category NHL-5 scores perform as well as the 14-16 category CCI and NCI scores in terms of association with OS and lymphoma-specific survival. These simple scores could be more easily used in clinical practice without prognostic loss.
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Affiliation(s)
- Max J. Gordon
- The University of Texas MD Anderson Cancer Center, Department of Cancer Medicine, Houston, TX, USA
- National Cancer Institute, Lymphoid Malignancy Branch, Bethesda, MD, USA
| | - Zhigang Duan
- The University of Texas MD Anderson Cancer Center, Department of Health Services Research, Houston, TX, USA
| | - Hui Zhao
- The University of Texas MD Anderson Cancer Center, Department of Health Services Research, Houston, TX, USA
| | - Loretta Nastoupil
- The University of Texas MD Anderson Cancer Center, Department of Lymphoma and Myeloma, Houston, TX, USA
| | - Swaminathan Iyer
- The University of Texas MD Anderson Cancer Center, Department of Lymphoma and Myeloma, Houston, TX, USA
| | - Alessandra Ferrajoli
- The University of Texas MD Anderson Cancer Center, Department of Leukemia, Houston, TX, USA
| | - Alexey V. Danilov
- City of Hope National Medical Center, Department of Hematology & Hematopoietic Cell Transplantation, Duarte, CA, USA
| | - Sharon H. Giordano
- The University of Texas MD Anderson Cancer Center, Department of Health Services Research, Houston, TX, USA
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12
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Bavi P, Mansoor A, Auer I, Hardin J. In Situ Follicular B-Cell Neoplasm Presenting as Miliary Facial Papules: A Unique Clinical Presentation and Spontaneous Regression. Am J Dermatopathol 2024; 46:167-169. [PMID: 37910140 DOI: 10.1097/dad.0000000000002579] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/03/2023]
Abstract
ABSTRACT In situ follicular B-cell neoplasm (ISFN) is a variant of follicular lymphoma, presenting as an incidental histologic finding in lymph node biopsy or excisional specimens. ISFN presents with a B-cell population that strongly expresses BCL2 and CD10 within the germinal centers of a lymph node or extranodal site. Genetic analysis shows t(14;18) translocation. Herein, we report a case of ISFN presenting as military and agminated facial papules in a young woman, which resolved spontaneously in the postpartum period. To our knowledge, this is the only report of a cutaneous site of involvement of this rare entity.
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Affiliation(s)
- Prashant Bavi
- Department of Pathology, Division of Hematopathology, University of Calgary; and
| | - Adnan Mansoor
- Department of Pathology, Division of Hematopathology, University of Calgary; and
| | - Iwona Auer
- Department of Pathology, Division of Hematopathology, University of Calgary; and
| | - Jori Hardin
- Department of Medicine, Division of Dermatology, University of Calgary
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13
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Ma HL. Clinicopathological characteristics of duodenal-type follicular lymphoma: A case report. Shijie Huaren Xiaohua Zazhi 2024; 32:93-96. [DOI: 10.11569/wcjd.v32.i1.93] [Citation(s) in RCA: 1] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/07/2023] [Revised: 12/04/2023] [Accepted: 01/11/2024] [Indexed: 01/26/2024] Open
Abstract
BACKGROUND Duodenal-type follicular lymphoma (D-FL) is very rare in clinical practice. Since its clinical manifestations are similar to those of intestinal inflammation or polyps, D-FL is prone to misdiagnosis or missed diagnosis.
CASE SUMMARY Inexperienced clinical physicians are prone to misdiagnosing D-FL as polyps or inflammation, and it should be distin-guished from intense follicular hyperplasia and lymphoma with follicular structure. Immunohistochemistry (IHC) and molecular techniques are required for its clear diagnosis.
CONCLUSION D-FL mainly occurs in the descending part of the duodenum, but it can also affect the distal small intestine. Endoscopically, the mucosa shows polyp-like proliferation. IHC staining shows that the tumor cells are positive for CD20, CD79α, CD10, Bcl-6, and Bcl-2, and CD21 labeled follcular dendritic cells show a characteristic "duodenal pattern". BCL2/t(14;18) fusion is often present. D-FL has a good prognosis, and its prognosis needs follow-up observation.
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Affiliation(s)
- Hua-Ling Ma
- Department of Pathology, Caidian District People's Hospital, Wuhan 430100, Hubei Province, China
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14
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Du YR, Li J, Li SX, Guan CY, Li HL, Gao ZF, Li X, Dong GH. [Studies on clinicopathological features of duodenal-type follicular lymphoma of 18 patients]. ZHONGHUA XUE YE XUE ZA ZHI = ZHONGHUA XUEYEXUE ZAZHI 2024; 45:94-97. [PMID: 38527846 PMCID: PMC10951120 DOI: 10.3760/cma.j.cn121090-20230915-00125] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Subscribe] [Scholar Register] [Received: 09/15/2023] [Indexed: 03/27/2024]
Abstract
To investigate the clinical and pathological characteristics of duodenal-type follicular lymphoma (D-FL), and to deepen the understanding of Duodenal-type follicular lymphoma. The clinical symptoms, endoscopic features, pathologic features, immunophenotype, molecular pathological features and treatment follow-up of 18 D-FL patients diagnosed in Department of Pathology, Beijing Tiantan Hospital affiliated to Capital Medical University between January 2020 and July 2023 were summarized. A total of 18 patients with D-FL were included, including 10 males and 8 females. The median age was 49 (32-69) years respectively. Most of the patients were found during gastroenteroscopy or presented with the common gastrointestinal symptoms of stomach pain, acid reflux, vomiting and diarrhea. Most endoscopic findings were multiple small gray and white polyposis. In the pathological morphology, the mucous layer and submucous layer showed lymphoid follicular structures with full and strained follicles. The immunophenotype showed that the tumor cells strongly expressed CD20 and BCL2 and had low proliferation activity. Immunoglobulin clonal analysis of 1 case showed IgK monoclonal rearrangement (1/1). FISH showed 1 case of BCL2 gene rearrangement (1/3). All patients did not receive targeted chemotherapy and adopted a wait-and-see strategy. Median follow-up was 12 (2-34) months. This study shows that D-FL is an indolent lymphoma, which tends to occur in the duodenum and has a good prognosis.
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Affiliation(s)
- Y R Du
- Department of Pathology, Beijing Tiantan Hospital, Capital Medical University, Beijing 100070, China
| | - J Li
- Department of Pathology, Beijing Tiantan Hospital, Capital Medical University, Beijing 100070, China
| | - S X Li
- Department of Pathology, Beijing Tiantan Hospital, Capital Medical University, Beijing 100070, China
| | - C Y Guan
- Department of Pathology, Beijing Tiantan Hospital, Capital Medical University, Beijing 100070, China
| | - H L Li
- Department of Pathology, Beijing Tiantan Hospital, Capital Medical University, Beijing 100070, China
| | - Z F Gao
- Department of Pathology, Beijing Tiantan Hospital, Capital Medical University, Beijing 100070, China
| | - X Li
- Department of Pathology, Beijing Tiantan Hospital, Capital Medical University, Beijing 100070, China
| | - G H Dong
- Department of Pathology, Beijing Tiantan Hospital, Capital Medical University, Beijing 100070, China
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15
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Watanabe T. Gene targeted and immune therapies for nodal and gastrointestinal follicular lymphomas. World J Gastroenterol 2023; 29:6179-6197. [PMID: 38186866 PMCID: PMC10768399 DOI: 10.3748/wjg.v29.i48.6179] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/24/2023] [Revised: 11/02/2023] [Accepted: 12/18/2023] [Indexed: 12/27/2023] Open
Abstract
Follicular lymphoma (FL) is the most common indolent B-cell lymphoma (BCL) globally. Recently, its incidence has increased in Europe, the United States, and Asia, with the number of gastrointestinal FL cases expected to increase. Genetic abnormalities related to t(14;18) translocation, BCL2 overexpression, NF-κB pathway-related factors, histone acetylases, and histone methyltransferases cause FL and enhance its proliferation. Meanwhile, microRNAs are commonly used in diagnosing FL and predicting patient prognosis. Many clinical trials on novel therapeutics targeting these genetic abnormalities and immunomodulatory mechanisms have been conducted, resulting in a marked improvement in therapeutic outcomes for FL. Although developing these innovative therapeutic agents targeting specific genetic mutations and immune pathways has provided hope for curative options, FL treatment has become more complex, requiring combinatorial therapeutic regimens. However, optimal treatment combinations have not yet been achieved, highlighting the importance of a complete under-standing regarding the pathogenesis of gastrointestinal FL. Accordingly, this article reviews key research on the molecular pathogenesis of nodal FL and novel therapies targeting the causative genetic mutations. Moreover, the results of clinical trials are summarized, with a particular focus on treating nodal and gastrointestinal FLs.
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Affiliation(s)
- Takuya Watanabe
- Department of Internal Medicine and Gastroenterology, Watanabe Internal Medicine Aoyama Clinic, Niigata 9502002, Japan
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16
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Sumioka A, Tsuboi A, Oka S, Kato Y, Matsubara Y, Hirata I, Takigawa H, Yuge R, Shimamoto F, Tada T, Tanaka S. Disease surveillance evaluation of primary small-bowel follicular lymphoma using capsule endoscopy images based on a deep convolutional neural network (with video). Gastrointest Endosc 2023; 98:968-976.e3. [PMID: 37482106 DOI: 10.1016/j.gie.2023.07.024] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/31/2023] [Revised: 07/01/2023] [Accepted: 07/09/2023] [Indexed: 07/25/2023]
Abstract
BACKGROUND AND AIMS Capsule endoscopy (CE) is useful in evaluating disease surveillance for primary small-bowel follicular lymphoma (FL), but some cases are difficult to evaluate objectively. This study evaluated the usefulness of a deep convolutional neural network (CNN) system using CE images for disease surveillance of primary small-bowel FL. METHODS We enrolled 26 consecutive patients with primary small-bowel FL diagnosed between January 2011 and January 2021 who underwent CE before and after a watch-and-wait strategy or chemotherapy. Disease surveillance by the CNN system was evaluated by the percentage of FL-detected images among all CE images of the small-bowel mucosa. RESULTS Eighteen cases (69%) were managed with a watch-and-wait approach, and 8 cases (31%) were treated with chemotherapy. Among the 18 cases managed with the watch-and-wait approach, the outcome of lesion evaluation by the CNN system was almost the same in 13 cases (72%), aggravation in 4 (22%), and improvement in 1 (6%). Among the 8 cases treated with chemotherapy, the outcome of lesion evaluation by the CNN system was improvement in 5 cases (63%), almost the same in 2 (25%), and aggravation in 1 (12%). The physician and CNN system reported similar results regarding disease surveillance evaluation in 23 of 26 cases (88%), whereas a discrepancy between the 2 was found in the remaining 3 cases (12%), attributed to poor small-bowel cleansing level. CONCLUSIONS Disease surveillance evaluation of primary small-bowel FL using CE images by the developed CNN system was useful under the condition of excellent small-bowel cleansing level.
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Affiliation(s)
- Akihiko Sumioka
- Department of Gastroenterology, Hiroshima University Hospital, Hiroshima, Japan
| | - Akiyoshi Tsuboi
- Department of Endoscopy, Hiroshima University Hospital, Hiroshima, Japan
| | - Shiro Oka
- Department of Gastroenterology, Hiroshima University Hospital, Hiroshima, Japan
| | | | - Yuka Matsubara
- Department of Gastroenterology, Hiroshima University Hospital, Hiroshima, Japan
| | - Issei Hirata
- Department of Gastroenterology, Hiroshima University Hospital, Hiroshima, Japan
| | - Hidehiko Takigawa
- Department of Endoscopy, Hiroshima University Hospital, Hiroshima, Japan
| | - Ryo Yuge
- Department of Endoscopy, Hiroshima University Hospital, Hiroshima, Japan
| | - Fumio Shimamoto
- Faculty of Health Sciences, Hiroshima Shudo University, Hiroshima, Japan
| | - Tomohiro Tada
- AI Medical Service Inc, Tokyo, Japan; Department of Surgical Oncology, Graduate School of Medicine, The University of Tokyo, Tokyo, Japan; Tada Tomohiro Institute of Gastroenterology and Proctology, Saitama, Japan
| | - Shinji Tanaka
- Department of Endoscopy, Hiroshima University Hospital, Hiroshima, Japan
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17
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Moreta Rodríguez M, Fernández Prada SJ, Maroto Martin C, Martínez Ortega A, Madrigal Rubiales B, de la Serna Higuera C. Biliary obstruction secondary to duodenal follicular lymphoma with papillary involvement. REVISTA ESPANOLA DE ENFERMEDADES DIGESTIVAS 2023; 115:663-665. [PMID: 37882167 DOI: 10.17235/reed.2023.9929/2023] [Citation(s) in RCA: 2] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 10/27/2023]
Abstract
Duodenal-type follicular lymphoma is a clinical variant of follicular lymphoma located in the intestine, presenting as multiple small polyps. The estimated frequency is 1/7000 people and extraduodenal involvement is rare. It predominantly affects middle-aged women, although some cases of young patients have been published in the literature. They are usually located in the second periampullary portion of the duodenum. It is diagnosed incidentally or after performing an imaging test due to non-specific digestive symptoms (abdominalgia, diarrhea or hyporexia). The prognosis is usually favorable without specific treatment5.
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18
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Takahashi Y, Taniguchi H, Haruhi F, Hattori D, Sasaki H, Makita S, Iwaki N, Fukuhara S, Munakata W, Saito Y, Izutsu K, Maeshima AM. Pathogenesis of Gastrointestinal Follicular Lymphomas: Consideration Based on Histopathology and Endoscopic Findings. Am J Surg Pathol 2023; 47:1134-1143. [PMID: 37493666 DOI: 10.1097/pas.0000000000002095] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 07/27/2023]
Abstract
Gastrointestinal (GI) follicular lymphoma (FL) is the most frequently diagnosed extranodal FL; however, its pathogenesis is debatable. We investigated the distribution, endoscopic, and histopathologic findings of 366 GI FL samples obtained from 298 patients. FLs were most frequently observed in the small intestine (71%), including the duodenum (52%), but were also commonly found in the stomach (15%) and colon (12%). The proportion of granular lesions in the duodenum, terminal ileum, colon, and stomach was 74%, 39%, 24%, and 0%, respectively. Submucosal or ulcerated tumors were frequently observed in the stomach (48%) and colon (52%). Most GI FL showed grade 1 to 2 histology (89%) as well as CD10 + (93%) and BCL2 + (98%) positivity. There were no significant differences in the endoscopic or histologic findings between primary and secondary GI FLs. As known, the mucosa of the small intestine is thin and villous, while the mucosa of the stomach and colon is thicker and has a smooth surface. Granular lesions corresponding to very small FL were detected in the former but rarely in the latter. Nine (7%) patients with primary GI FL developed histologic transformation to diffuse large B-cell lymphoma (n=8) or high-grade B-cell lymphoma (n=1) 10 months to 14 years after the diagnosis of FL. Two patients died of lymphoma. In conclusion, the incidence and endoscopic findings differed, but the histopathology was similar in FLs in each site. These differences might be attributed to variations in each GI site's mucosal structure and the neoplastic follicles' size. Due to its characteristic structure, very small classic FLs might be detectable mainly in the small intestine.
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Affiliation(s)
| | - Hirokazu Taniguchi
- Departments of Diagnostic Pathology
- Pathology and Clinical Laboratory, JR Tokyo General Hospital
| | | | | | | | | | | | | | | | - Yutaka Saito
- Endoscopy Division, National Cancer Center Hospital, Tokyo, Japan
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19
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Watanabe T. Recent advances in treatment of nodal and gastrointestinal follicular lymphoma. World J Gastroenterol 2023; 29:3574-3594. [PMID: 37398889 PMCID: PMC10311612 DOI: 10.3748/wjg.v29.i23.3574] [Citation(s) in RCA: 5] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/13/2023] [Revised: 05/14/2023] [Accepted: 05/22/2023] [Indexed: 06/16/2023] Open
Abstract
Follicular lymphoma (FL) is the most common low-grade lymphoma, and although nodal FL is highly responsive to treatment, the majority of patients relapse repeatedly, and the disease has been incurable with a poor prognosis. However, primary FL of the gastrointestinal tract has been increasingly detected in Japan, especially due to recent advances in small bowel endoscopy and increased opportunities for endoscopic examinations and endoscopic diagnosis. However, many cases are detected at an early stage, and the prognosis is good in many cases. In contrast, in Europe and the United States, gastrointestinal FL has long been considered to be present in 12%-24% of Stage-IV patients, and the number of advanced gastrointestinal cases is expected to increase. This editorial provides an overview of the recent therapeutic advances in nodal FL, including antibody-targeted therapy, bispecific antibody therapy, epigenetic modulation, and chimeric antigen receptor T-cell therapy, and reviews the latest therapeutic manuscripts published in the past year. Based on an understanding of the therapeutic advances in nodal FL, we also discuss future possibilities for gastroenterologists to treat gastrointestinal FL, especially in advanced cases.
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Affiliation(s)
- Takuya Watanabe
- Department of Internal Medicine and Gastroenterology, Watanabe Internal Medicine Aoyama Clinic, Niigata-city 9502002, Japan
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20
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Louissaint A. Navigating the Heterogeneity of Follicular Lymphoma and its Many Variants: An Updated Approach to Diagnosis and Classification. Surg Pathol Clin 2023; 16:233-247. [PMID: 37149358 DOI: 10.1016/j.path.2023.02.001] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 05/08/2023]
Abstract
Follicular lymphoma (FL) is a lymphoid neoplasm composed of follicle center (germinal center) B cells, with varying proportions of centrocytes and centroblasts, that usually has a predominantly follicular architectural pattern. Over the past decade, our understanding of FL has evolved significantly, with new recognition of several recently defined FL variants characterized by distinct clinical presentations, behaviors, genetic alterations, and biology. This manuscript aims to review the heterogeneity of FL and its variants, to provide an updated guide on their diagnosis and classification, and to describe how approaches to the histologic subclassification of classic FL have evolved in current classification schemes.
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Affiliation(s)
- Abner Louissaint
- Department of Pathology, Massachusetts General Hospital, 149 13th St, Charlestown, MA 02114, USA.
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21
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Iwamuro M, Tanaka T, Ennishi D, Matsueda K, Yoshioka M, Miyahara K, Sakaguchi C, Nishimura M, Nagahara T, Mannami T, Takenaka R, Oka S, Inoue M, Takimoto H, Inaba T, Kobayashi S, Toyokawa T, Tsugeno H, Suzuki S, Sawada S, Tanaka S, Tsuzuki T, Okada H. Long-term outcomes of patients with primary intestinal follicular lymphoma managed with watch-and-wait strategy. Sci Rep 2023; 13:5858. [PMID: 37041184 PMCID: PMC10090188 DOI: 10.1038/s41598-023-32736-9] [Citation(s) in RCA: 7] [Impact Index Per Article: 3.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/06/2022] [Accepted: 03/31/2023] [Indexed: 04/13/2023] Open
Abstract
Patients with primary intestinal follicular lymphoma are often followed-up without a specific treatment, and this approach is called the "watch-and-wait approach." However, the long-term outcomes of this patient group have not been sufficiently investigated. We enrolled patients with primary intestinal follicular lymphoma who were diagnosed before 2016 and managed with the watch-and-wait approach in 20 institutions. We retrospectively investigated the overall, disease-specific, and event-free survival rates as well as the rate of spontaneous regression. Among the 248 patients with follicular lymphoma with gastrointestinal involvement, 124 had localized disease (stage I or II1). We analyzed the data of 73 patients who were managed using the watch-and-wait approach. During the mean follow-up period of 8.3 years, the follicular lymphoma had spontaneously resolved in 16.4% of the patients. The 5-year and 10-year overall survival rates were 92.9% and 87.1%, respectively. With disease progression (n = 7), initiation of therapy (n = 7), and histologic transformation to aggressive lymphoma (n = 0) defined as events, the 5-year and 10-year event-free survival rates were 91.1% and 86.9%, respectively. No patient died of progressive lymphoma. Thus, both 5-year and 10-year disease-specific survival rates were 100%. In conclusion, an indolent long-term clinical course was confirmed in the patients with primary intestinal follicular lymphoma. The watch-and-wait strategy is a reasonable approach for the initial management of these patients.
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Affiliation(s)
- Masaya Iwamuro
- Department of Gastroenterology and Hepatology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, 2-5-1 Shikata-cho, Kita-ku, Okayama, Okayama, 700-8558, Japan.
| | - Takehiro Tanaka
- Department of Pathology, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, 2-5-1 Shikata-cho, Kita-ku, Okayama, Okayama, 700-8558, Japan
| | - Daisuke Ennishi
- Department of Hematology and Oncology, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, 2-5-1 Shikata-cho, Kita-ku, Okayama, Okayama, 700-8558, Japan
| | - Kazuhiro Matsueda
- Department of Gastroenterology and Hepatology, Kurashiki Central Hospital, 1-1-1 Miwa, Kurashiki, Okayama, 710-8602, Japan
| | - Masao Yoshioka
- Department of Internal Medicine, Okayama Saiseikai General Hospital, 2-25 Kokutai-cho, Kita-ku, Okayama, Okayama, 700-8511, Japan
| | - Koji Miyahara
- Department of Internal Medicine, Hiroshima City Hospital, 7-33 Motomachi, Naka-ku, Hiroshima, Hiroshima, 730-8518, Japan
| | - Chihiro Sakaguchi
- Department of Endoscopy, National Hospital Organization Shikoku Cancer Center, Kou 160, Minamiumemotomachi, Matsuyama, 791-0280, Japan
| | - Mamoru Nishimura
- Department of Internal Medicine, Okayama City Hospital, 3-20-1 Kitanagase Omote-cho, Kita-ku, Okayama, Okayama, 700‑8557, Japan
| | - Teruya Nagahara
- Department of Gastroenterology, Mitoyo General Hospital, 708 Himehama, Toyohama-cho, Kan'onji, Kagawa, 769-1695, Japan
| | - Tomohiko Mannami
- Department of Gastroenterology, National Hospital Organization Okayama Medical Center, 1711-1 Tamasu, Kita-ku, Okayama, Okayama, 701-1192, Japan
| | - Ryuta Takenaka
- Department of Internal Medicine, Tsuyama Chuo Hospital, 1756 Kawasaki, Tsuyama, Okayama, 708‑0841, Japan
| | - Shohei Oka
- Department of Gastroenterology, Nippon Kokan Fukuyama Hospital, 1844 Tsunoshita, Daimon-cho, Fukuyama, Hiroshima, 721-0927, Japan
| | - Masafumi Inoue
- Department of Gastroenterology, Japanese Red Cross Okayama Hospital, 2-1-1 Aoe, Kita-ku, Okayama, Okayama, 700-8607, Japan
| | - Hidetaka Takimoto
- Department of Internal Medicine, Kagawa Rosai Hospital, 3-3-1 Joto-cho, Marugame, Kagawa, 763‑8502, Japan
| | - Tomoki Inaba
- Department of Gastroenterology, Kagawa Prefectural Central Hospital, 1-2-1 Asahi-machi, Takamatsu, Kagawa, 760-8557, Japan
| | - Sayo Kobayashi
- Department of Internal Medicine, Fukuyama City Hospital, 5-23-1 Zao-cho, Fukuyama, Hiroshima, 721-8511, Japan
| | - Tatsuya Toyokawa
- Department of Gastroenterology, National Hospital Organization Fukuyama Medical Center, 4-14-17 Okinogami-cho, Fukuyama, Hiroshima, 720-8520, Japan
| | - Hirofumi Tsugeno
- Department of Gastroenterology, Okayama Rosai Hospital, 1-10 Chikkomidorimachi, Minami-ku, Okayama, Okayama, 702-8055, Japan
| | - Seiyuu Suzuki
- Department of Gastroenterology, Sumitomo Besshi Hospital, 3-1 Ojicho, Niihama, Ehime, 792‑8543, Japan
| | - Sachiko Sawada
- Department of Internal Medicine, St. Mary's Hospital, 650 Nibuno, Himeji, Hyogo, 670-0801, Japan
| | - Shouichi Tanaka
- Department of Gastroenterology, National Hospital Organization Iwakuni Clinical Center, 1-1-1 Atago-cho, Iwakuni, Yamaguchi, 740-8510, Japan
| | - Takao Tsuzuki
- Department of Internal Medicine, Japanese Red Cross Society Himeji Hospital, 1-12-1 Shimoteno, Himeji, Hyogo, 670-8540, Japan
| | - Hiroyuki Okada
- Department of Gastroenterology and Hepatology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, 2-5-1 Shikata-cho, Kita-ku, Okayama, Okayama, 700-8558, Japan
- Department of Internal Medicine, Japanese Red Cross Society Himeji Hospital, 1-12-1 Shimoteno, Himeji, Hyogo, 670-8540, Japan
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22
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Kim H, Shin D, Son SM, Cho J, Kim JE, Kim Y, Jeon TS, Ko YH. Clinicopathological and molecular genetic characteristics of primary gastric follicular lymphoma ☆,☆☆,☆☆☆. Hum Pathol 2023; 136:114-122. [PMID: 37054783 DOI: 10.1016/j.humpath.2023.04.007] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/01/2023] [Revised: 04/07/2023] [Accepted: 04/07/2023] [Indexed: 04/15/2023]
Abstract
Primary gastric follicular lymphomas (FLs) have been rarely reported and little is known about their characteristics. In the present study, we report five cases of primary gastric FL and describe their clinicopathological and molecular genetic features. A total of seven samples from five patients were investigated for clinicopathological characteristics and somatic mutations by the targeted sequencing of 50 lymphoma-related genes. Two cases were identified as slightly elevated submucosal tumors and three cases as polypoid tumors. Histologically, all cases were low-grade FLs. The immuno-profile was CD20+/CD10+/BCL2+ in four cases and CD20+/CD10+/BCL2- in one case. Immunostaining pattern for CD21 was similar to that of classic FL. BCL2 rearrangement was not identified in fluorescence in situ hybridization studies in any of the five cases. NGS analysis showed mutations in genes involved in epigenetic modifications (KMT2D, ARID1A, EP300, CREBBP), NK-kB pathway (CARD11), and JAK-STAT pathway as found in classic follicular lymphoma. All cases presented with clinical I without the involvement of regional or systemic lymph nodes. Four patients were well, whereas one patient who received endoscopic mucosal resection of tumor without additional chemotherapy or radiotherapy experienced three relapses. In conclusion, primary gastric FL is characterized by a low-grade neoplasm with infrequent BCL2 rearrangement. After resection of the lesion, additional treatment such as radiation therapy or chemotherapy is required as there is a possibility of recurrence.
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Affiliation(s)
- Hyunsung Kim
- Department of Pathology, Seoul Hospital, Hanyang University College of Medicine, Seoul 04763, South Korea
| | - Donghoon Shin
- Department of Pathology, Yangsan Hospital, Pusan National University College of Medicine, Yangsan 50612, South Korea
| | - Seng-Myoung Son
- Department of Pathology, Chungbuk National University College of Medicine, Cheongju 28644, South Korea
| | - Junhun Cho
- Department of Pathology, Samsung Medical Center, Sungkyunkwan University College of Medicine, Seoul 06351, South Korea
| | - Ji Eun Kim
- Department of Pathology, Seoul Metropolitan Government Seoul National University Boramae Medical Center, Seoul National University College of Medicine, Seoul 07061, South Korea
| | - Yeseul Kim
- Department of Pathology, Anam Hospital, Korea University College of Medicine, Seoul 02841, South Korea
| | - Tae-Sung Jeon
- Department of Pathology, Guro Hospital, Korea University College of Medicine, Seoul 08308, South Korea
| | - Young Hyeh Ko
- Department of Pathology, Guro Hospital, Korea University College of Medicine, Seoul 08308, South Korea; Department of Pathology, Cheju Halla General Hospital, Jeju 63127, South Korea.
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23
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Iwamuro M, Tanaka T, Okada H. Review of lymphoma in the duodenum: An update of diagnosis and management. World J Gastroenterol 2023; 29:1852-1862. [PMID: 37032723 PMCID: PMC10080700 DOI: 10.3748/wjg.v29.i12.1852] [Citation(s) in RCA: 9] [Impact Index Per Article: 4.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/05/2022] [Revised: 02/11/2023] [Accepted: 03/14/2023] [Indexed: 03/28/2023] Open
Abstract
The presentation, subtype, and macroscopic images of lymphoma vary depending on the site of the tumor within the gastrointestinal tract. We searched PubMed for publications between January 1, 2012 and October 10, 2022, and retrieved 130 articles relating to duodenal lymphoma. A further 22 articles were added based on the manual screening of relevant articles, yielding 152 articles for full-text review. The most predominant primary duodenal lymphoma was follicular lymphoma. In this review, we provide an update of the diagnosis and man-agement of representative lymphoma subtypes occurring in the duodenum: Follicular lymphoma, diffuse large B-cell lymphoma, extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue, mantle cell lymphoma, and T-cell lymphomas.
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MESH Headings
- Male
- Humans
- Adult
- Lymphoma, Follicular/diagnosis
- Lymphoma, Follicular/therapy
- Lymphoma, Follicular/pathology
- Lymphoma, B-Cell, Marginal Zone/diagnosis
- Lymphoma, B-Cell, Marginal Zone/therapy
- Lymphoma, B-Cell, Marginal Zone/pathology
- Lymphoma, Large B-Cell, Diffuse/diagnostic imaging
- Lymphoma, Large B-Cell, Diffuse/therapy
- Gastrointestinal Tract/pathology
- Duodenum/diagnostic imaging
- Duodenum/pathology
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Affiliation(s)
- Masaya Iwamuro
- Department of Gastroenterology and Hepatology, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama 700-8558, Japan
| | - Takehiro Tanaka
- Department of Pathology, Okayama University Hospital, Okayama 700-8558, Japan
| | - Hiroyuki Okada
- Department of Gastroenterology and Hepatology, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama 700-8558, Japan
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24
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Fischer L, Dreyling M. Follicular lymphoma: an update on biology and optimal therapy. Leuk Lymphoma 2023; 64:761-775. [PMID: 37061956 DOI: 10.1080/10428194.2023.2174804] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/11/2023]
Abstract
Follicular lymphoma (FL) is a mature B-cell neoplasm with a mostly indolent course. Genetic hallmark characteristics are Bcl-2 overexpression based on a t(14;18) translocation and additional secondary genetic and epigenetic aberrations. Standard treatment for early-stage patients has curative intent and usually consists of radiotherapy with or without rituximab. In the advanced stage, the main therapeutic focus is on prolonged remissions. Therefore, treatment in asymptomatic patients is usually deferred. Symptomatic patients are subject to immunochemotherapy induction followed by antibody maintenance. Importantly, about one in five patients subsequently experiences a more rapid clinical course, achieving only short remissions with multiple relapses (POD24). In those patients, there is still an urgent need for improved therapeutic options. Accordingly, a plethora of targeted and immunotherapeutic options, including immunomodulatory drugs, small molecule inhibitors, monoclonal antibodies as well as bispecific T-cell engager antibodies and CAR-T cell products have been recently evaluated in such relapsed high-risk patients.
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Affiliation(s)
- Luca Fischer
- Department of Medicine III, University Hospital, LMU Munich, Munich, Germany
| | - Martin Dreyling
- Department of Medicine III, University Hospital, LMU Munich, Munich, Germany
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25
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Follicular Lymphoma in the 5th Edition of the WHO-Classification of Haematolymphoid Neoplasms-Updated Classification and New Biological Data. Cancers (Basel) 2023; 15:cancers15030785. [PMID: 36765742 PMCID: PMC9913816 DOI: 10.3390/cancers15030785] [Citation(s) in RCA: 4] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/10/2023] [Revised: 01/23/2023] [Accepted: 01/24/2023] [Indexed: 01/31/2023] Open
Abstract
The conceptual description of Follicular lymphoma (FL) in the 5th edition of the World Health Organization (WHO) classification of haematolymphoid tumors (WHO-HAEM5) has undergone significant revision. The vast majority of FL (85%) with a follicular growth pattern are composed of centrocytes and centroblasts, harbor the t(14;18)(q32;q21) translocation and are now termed classic FL (cFL). They are set apart from three related subtypes, FL with predominantly follicular growth pattern, FL with unusual cytological features (uFL) and follicular large B-cell lymphoma (FLBCL). In contrast to the revised 4th edition of the WHO classification of haematolymphoid tumors (WHO-HAEM4R), grading of cFL is no longer mandatory. FL with a predominantly diffuse growth pattern had been previously recognized in WHO-HAEM4R. It frequently occurs as a large tumor in the inguinal region and is associated with CD23 expression. An absence of the IGH::BCL2 fusion and frequent STAT6 mutations along with 1p36 deletion or TNFRSF14 mutation is typical. The newly introduced subtype of uFL includes two subsets that significantly diverge from cFL: one with "blastoid" and one with "large centrocyte" variant cytological features. uFL more frequently displays variant immunophenotypic and genotypic features. FLBCL is largely identical to WHO-HAEM4R FL grade 3B and renaming was done for reasons of consistency throughout the classification. In-situ follicular B-cell neoplasm, pediatric-type FL, duodenal-type FL and primary cutaneous follicle center lymphoma are categorized as discrete entities. In addition, novel findings concerning underlying biological mechanisms in the pathogenesis of early and systemic follicular lymphoma will be presented.
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26
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Jacobsen E. Follicular lymphoma: 2023 update on diagnosis and management. Am J Hematol 2022; 97:1638-1651. [PMID: 36255040 DOI: 10.1002/ajh.26737] [Citation(s) in RCA: 25] [Impact Index Per Article: 8.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/13/2022] [Accepted: 09/15/2022] [Indexed: 01/31/2023]
Abstract
DISEASE OVERVIEW Follicular lymphoma (FL) is generally an indolent B cell lymphoproliferative disorder of transformed follicular center B cells. FL is characterized by diffuse lymphadenopathy, bone marrow involvement, and splenomegaly. Extranodal involvement is less common. Cytopenias are relatively common but constitutional symptoms of fever, night sweats, and weight loss are uncommon in the absence of transformation to diffuse large B cell lymphoma. DIAGNOSIS The diagnosis is based on histology from a biopsy of a lymph node or other affected tissue. Incisional biopsy is preferred over needle biopsies in order to give adequate tissue to assign grade and assess for transformation. Immunohistochemical staining is positive in virtually all cases for cell surface CD19, CD20, CD10, and monoclonal immunoglobulin, as well as cytoplasmic expression of bcl-2 protein. The overwhelming majority of cases have the characteristic t(14;18) translocation involving the IgH/bcl-2 genes. RISK STRATIFICATION The Follicular Lymphoma International Prognostic Index (FLIPI) uses five independent predictors of inferior survival: age >60 years, hemoglobin <12 g/dL, serum LDH > normal, Ann Arbor stage III/IV, number of involved nodal areas >4. The presence of 0-1, 2, and ≥3 adverse factors defines low, intermediate, and high-risk disease. There are other clinical prognostic models but the FLIPI remains the most common. Other factors such as time to relapse of less than 2 years from chemoimmunotherapy and specific gene mutations may also be useful for prognosis. Regardless of the prognostic model used, modern therapies have demonstrably improved prognosis. RISK-ADAPTED THERAPY Observation continues to be appropriate for asymptomatic patients with low bulk disease and no cytopenias. There is no overall survival (OS) advantage for early treatment with either chemotherapy or single-agent rituximab. For patients needing therapy, most patients are treated with chemoimmunotherapy, which has improved overall response rates (ORR), DOR, and OS. Randomized studies have shown additional benefits for maintenance of rituximab. Lenalidomide was non-inferior to chemoimmunotherapy in a randomized front-line study and, when combined with rituximab, was superior to rituximab alone in relapsed FL. Kinase inhibitors, stem cell transplantation (SCT), and chimeric antigen receptor T cells (CAR-T) are also considered for recurrent disease.
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Affiliation(s)
- Eric Jacobsen
- Department of Medical Oncology, Dana-Farber Cancer Institute, Boston, Massachusetts, USA
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27
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Sumioka A, Oka S, Hirata I, Iio S, Tsuboi A, Takigawa H, Yuge R, Urabe Y, Boda K, Kohno T, Okanobu H, Kitadai Y, Arihiro K, Tanaka S. Predictive factors for the progression of primary localized stage small-bowel follicular lymphoma. J Gastroenterol 2022; 57:667-675. [PMID: 35831477 DOI: 10.1007/s00535-022-01897-1] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/07/2022] [Accepted: 06/14/2022] [Indexed: 02/04/2023]
Abstract
BACKGROUND Primary small-bowel follicular lymphoma (FL) is mainly diagnosed as a duodenal lesion during esophagogastroduodenoscopy. Recently, with the widespread use of small-bowel endoscopy, FL in the jejunum and ileum has been detected. Most patients with small-bowel FL are diagnosed at the localized stage, and a watch-and-wait policy is used. However, the predictive factors for the progression of small-bowel FL have not been clarified. This study retrospectively examined the predictive factors for the progression of primary localized stage small-bowel FL based on clinicopathological and endoscopic findings. METHODS We enrolled 60 consecutive patients with primary small-bowel FL diagnosed at two tertiary hospitals between January 2005 and December 2020, with localized stage, low grade, and low tumor burden with the watch-and-wait policy. We examined the predictive factors for progression according to the clinicopathological and endoscopic findings. Endoscopic findings were focused on the color tone, circumferential location of follicular lesions (circumference ≥ 1/2 or < 1/2), fusion of follicular lesions (fusion [ +] or [ -]), and protruded lesions (≥ 6 mm or < 6 mm). RESULTS Progressive disease was observed in 12 (20%) patients (mean observation period, 76.4 ± 55.4 months). In the multivariate analysis, "circumference ≥ 1/2" and "fusion (+)" were significant predictive factors for progression. According to the Kaplan-Meier analysis, progression-free survival was significantly shorter in the "circumference ≥ 1/2" and/or "fusion (+)" group than in the "circumference < 1/2" and "fusion ( -)" group. CONCLUSIONS Endoscopic findings of "circumference ≥ 1/2" and "fusion (+)" were significant predictive factors for the progression of primary localized stage small-bowel FL.
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Affiliation(s)
- Akihiko Sumioka
- Department of Gastroenterology and Metabolism, Hiroshima University Hospital, 1-2-3 Kasumi, Minami-ku, Hiroshima, 734-8551, Japan
| | - Shiro Oka
- Department of Gastroenterology and Metabolism, Hiroshima University Hospital, 1-2-3 Kasumi, Minami-ku, Hiroshima, 734-8551, Japan.
| | - Issei Hirata
- Department of Gastroenterology and Metabolism, Hiroshima University Hospital, 1-2-3 Kasumi, Minami-ku, Hiroshima, 734-8551, Japan
| | - Sumio Iio
- Department of Gastroenterology and Metabolism, Hiroshima University Hospital, 1-2-3 Kasumi, Minami-ku, Hiroshima, 734-8551, Japan
| | - Akiyoshi Tsuboi
- Department of Endoscopy, Hiroshima University Hospital, Hiroshima, Japan
| | - Hidehiko Takigawa
- Department of Endoscopy, Hiroshima University Hospital, Hiroshima, Japan
| | - Ryo Yuge
- Department of Endoscopy, Hiroshima University Hospital, Hiroshima, Japan
| | - Yuji Urabe
- Department of Gastroenterology and Metabolism, Hiroshima University Hospital, 1-2-3 Kasumi, Minami-ku, Hiroshima, 734-8551, Japan
| | - Kazuki Boda
- Department of Gastroenterology, Hiroshima Red Cross Hospital and Atomic-Bomb Survivors Hospital, Hiroshima, Japan
| | - Tomohiko Kohno
- Department of Gastroenterology, Hiroshima Red Cross Hospital and Atomic-Bomb Survivors Hospital, Hiroshima, Japan
| | - Hideharu Okanobu
- Department of Gastroenterology, Hiroshima Red Cross Hospital and Atomic-Bomb Survivors Hospital, Hiroshima, Japan
| | - Yasuhiko Kitadai
- Department of Health and Science, Prefectural University of Hiroshima, Hiroshima, Japan
| | - Koji Arihiro
- Department of Anatomical Pathology, Hiroshima University Hospital, Hiroshima, Japan
| | - Shinji Tanaka
- Department of Endoscopy, Hiroshima University Hospital, Hiroshima, Japan
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28
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Saito M, Mori A, Tsukamoto S, Ishio T, Yokoyama E, Izumiyama K, Morioka M, Kondo T, Sugino H. Duodenal-type follicular lymphoma more than 10 years after treatment intervention: A retrospective single-center analysis. World J Gastrointest Oncol 2022; 14:1552-1561. [PMID: 36160741 PMCID: PMC9412938 DOI: 10.4251/wjgo.v14.i8.1552] [Citation(s) in RCA: 7] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/27/2021] [Revised: 01/29/2022] [Accepted: 07/26/2022] [Indexed: 02/05/2023] Open
Abstract
BACKGROUND Duodenal-type follicular lymphoma (D-FL) has been recognized as a rare entity that accounts for approximately 4% of primary gastrointestinal lymphomas. D-FL follows an indolent clinical course compared with common nodal FL and is generally considered to have a better prognosis. Therefore, the “watch and wait” approach is frequently adopted as the treatment method. Alternatively, there is an option to actively intervene in D-FL. However, the long-term outcomes of such cases are poorly understood.
AIM To clarify the clinical outcomes after long-term follow-up in cases of D-FL with treatment intervention.
METHODS We retrospectively analyzed patients who met the following criteria: the lesion was confirmed by endoscopy, the diagnosis of D-FL was confirmed histopathologically, and the patient was followed-up for more than 10 years after the intervention at our center.
RESULTS We identified 5 cases of D-FL. Two patients showed a small amount of bone marrow involvement (Stage IV). Rituximab was used as a treatment for remission in all 5 patients. It was also used in combination with chemotherapy in 2 Stage IV patients as well as for maintenance treatment. Radiation therapy was performed in 2 cases, which was followed by complete remission (CR). Eventually, all 5 patients achieved CR and survived for more than 10 years. However, 3 patients experienced recurrence. One patient achieved a second CR by retreatment, and in another case, the lesion showed spontaneous disappearance. The remaining patient had systemic widespread recurrence 13 years after the first CR. Biopsy results suggested that the FL lesions were transformed into diffuse large B-cell lymphoma. The patient died 4 years later despite receiving various chemotherapies.
CONCLUSION In this study, the treatment for patients of D-FL in Stage IV was successful. In the future, criteria for how to treat “advanced” D-FL should be established based on additional cases. This study of patients with D-FL indicates that whole-body follow-up examinations should continue for a long time due to a fatal recurrence 13 years after reaching CR.
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Affiliation(s)
- Makoto Saito
- Blood Disorders Center, Aiiku Hospital, Sapporo 064-0804, Japan
| | - Akio Mori
- Blood Disorders Center, Aiiku Hospital, Sapporo 064-0804, Japan
| | | | - Takashi Ishio
- Blood Disorders Center, Aiiku Hospital, Sapporo 064-0804, Japan
| | - Emi Yokoyama
- Blood Disorders Center, Aiiku Hospital, Sapporo 064-0804, Japan
| | - Koh Izumiyama
- Blood Disorders Center, Aiiku Hospital, Sapporo 064-0804, Japan
| | | | - Takeshi Kondo
- Blood Disorders Center, Aiiku Hospital, Sapporo 064-0804, Japan
| | - Hirokazu Sugino
- Department of Cancer Pathology, Hokkaido University, Faculty of Medicine, Sapporo 060-8638, Japan
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29
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Efficacy and Safety of Exploring Deeper Sections of the Infrapapillary Area of the Duodenum by Using Sedative Esophagogastroduodenoscopy. Gastroenterol Res Pract 2022; 2022:1381299. [PMID: 35935715 PMCID: PMC9348967 DOI: 10.1155/2022/1381299] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/16/2022] [Accepted: 07/04/2022] [Indexed: 11/17/2022] Open
Abstract
Background Using conventional esophagogastroduodenoscopy (EGD) to evaluate the infrapapillary area is not feasible. The use of sedative EGD may enable endoscopists to investigate the infrapapillary condition of the duodenum. In this study, we aimed to evaluate lesions in the infrapapillary regions by using sedative EGD. Methods In this retrospective observational study, we used the data of patients who underwent sedative EGD examinations at a tertiary hospital in southern Taiwan. The endoscopists evaluated the esophagus, stomach, and proximal duodenum and then attempted to explore the infrapapillary portion of the duodenum as deeply as possible. We assessed the success rate for the exploration of infrapapillary areas. Furthermore, we analyzed specific clinical findings of sedative EGD examination. Results In total, 2973 patients underwent sedative EGD between November 1, 2010, and December 31, 2011. For 2632 of these patients, it was their first sedative EGD examination. In 2511 patients (95.4%), the exploration of the infrapapillary areas was successful. In approximately 10% of the patients, specific findings were detected over the infrapapillary region, and 7 of these patients exhibited clinically significant findings (i.e., gallbladder cancer with metastasis, periampullary Vater adenoma, natural killer cell enteropathy, villous adenoma with moderate dysplasia, infrapapillary duodenal adenoma with dysplasia, duodenal perforation with tumor-like formation, and follicular lymphoma). No patient experienced minor or major adverse reactions during the sedative EGD procedure. Conclusions The current study provided evidence that sedative EGD examination enables a safe, comfortable, and effective endoscopic examination of deeper sections of the duodenum to evaluate the papillary and infrapapillary regions.
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30
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Minemura T, Kikuchi S, Mihara H, Kamihara Y, Wada A, Fuchino M, Nanjo S, Noguchi A, Minamisaka T, Murakami J, Yasuda I, Sato T. Protein-losing Enteropathy Complicated with Primary Intestinal Follicular Lymphoma. Intern Med 2022; 61:2051-2055. [PMID: 34840227 PMCID: PMC9334233 DOI: 10.2169/internalmedicine.8261-21] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/01/2022] Open
Abstract
Protein-losing enteropathy (PLE) is a rare syndrome characterized by hypoproteinemia due to gastrointestinal (GI) protein loss. Primary intestinal follicular lymphoma (PIFL), a specific variant of follicular lymphoma with essential only GI involvement, has not been reported as an etiology of PLE. We herein report a case of PLE complicated with PIFL that was successfully treated with rituximab, resulting in rapid improvement of PLE and a complete response of PIFL. Macroscopic findings of ulcerative lesions with diffuse involvement, which were precisely described by capsule and double-balloon enteroscopy at the diagnosis, also improved following the treatment. This case provides a clue suggesting factors that promote PLE in PIFL.
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Affiliation(s)
- Tomoki Minemura
- Department of Hematology, Toyama University Hospital, Japan
- Center for Medical Residency Training, Toyama University Hospital, Japan
| | - Shohei Kikuchi
- Department of Hematology, Toyama University Hospital, Japan
| | - Hiroshi Mihara
- Third Department of Internal Medicine, Toyama University Hospital, Japan
| | | | - Akinori Wada
- Department of Hematology, Toyama University Hospital, Japan
| | - Mayo Fuchino
- Third Department of Internal Medicine, Toyama University Hospital, Japan
| | - Sohachi Nanjo
- Third Department of Internal Medicine, Toyama University Hospital, Japan
| | - Akira Noguchi
- Department of Diagnostic Pathology, Toyama University Hospital, Japan
| | | | - Jun Murakami
- Division of Transfusion Medicine and Cell Therapy, Toyama University Hospital, Japan
| | - Ichiro Yasuda
- Third Department of Internal Medicine, Toyama University Hospital, Japan
| | - Tsutomu Sato
- Department of Hematology, Toyama University Hospital, Japan
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31
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Kamijo K, Shimomura Y, Yoshioka S, Yamashita D, Hara S, Ishikawa T. Clinical features and outcomes of duodenal-type follicular lymphoma: A single-center retrospective study. EJHAEM 2022; 3:379-384. [PMID: 35846028 PMCID: PMC9175859 DOI: 10.1002/jha2.384] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 11/25/2021] [Revised: 01/08/2022] [Accepted: 01/11/2022] [Indexed: 11/22/2022]
Abstract
Duodenal-type follicular lymphoma (FL) is a rare and newly recognized disease. Few data are available on the outcomes and treatment strategies for patients with duodenal-type FL. We aimed to investigate the clinical features and outcomes of duodenal-type FL. We defined duodenal-type FL as involvement of the duodenum, without nodal or extranodal lesions other than intestinal lesions, pathologically diagnosed as FL. We reviewed 26 patients with duodenal-type FL between January 2011 and December 2020 at Kobe City Hospital Organization, Kobe City Medical Center General Hospital. In particular, patients were selected for the watch and wait (WW) strategy and followed up with regular esophagogastroduodenoscopy about once a year at our institution. The patient characteristics were as follows: median age 63.5 years (range: 42-78), sex (male, 15; female, 11), stage (I, 26), and grade (I, 26). Regarding treatment strategies, 23 patients were selected for the WW strategy, and three patients received initial rituximab therapy. The median follow-up period was 65.5 months (range: 0.2-109). Five-year progression-free survival and 5-year overall survival rates were 86.3% and 100%, respectively. Among the 23 patients selected for the WW strategy, six had spontaneous complete regression, and 14 had stable disease, and three had progressive disease, including one with histologic transformation. The WW strategy for patients with duodenal-type FL could be an appropriate and safe treatment option. However, in several cases, disease progression was documented, and regular follow-up is important.
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Affiliation(s)
- Kimimori Kamijo
- Department of HematologyKobe City Hospital OrganizationKobe City Medical Center General HospitalChuo‐kuJapan
| | - Yoshimitsu Shimomura
- Department of HematologyKobe City Hospital OrganizationKobe City Medical Center General HospitalChuo‐kuJapan
- Department of Environmental Medicine and Population ScienceGraduate School of MedicineOsaka UniversitySuitaJapan
| | - Satoshi Yoshioka
- Department of HematologyKobe City Hospital OrganizationKobe City Medical Center General HospitalChuo‐kuJapan
| | - Daisuke Yamashita
- Department of PathologyKobe City Hospital OrganizationKobe City Medical Center General HospitalChuo‐kuJapan
| | - Shigeo Hara
- Department of PathologyKobe City Hospital OrganizationKobe City Medical Center General HospitalChuo‐kuJapan
| | - Takayuki Ishikawa
- Department of HematologyKobe City Hospital OrganizationKobe City Medical Center General HospitalChuo‐kuJapan
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32
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Varanese M, Lauro A, Lattina I, Tripodi D, Daralioti T, Khouzam S, Marino IR, Stigliano V, D'Andrea V, Frattaroli S, Sorrenti S. Duodenal Follicular Lymphoma: Track or Treat? Dig Dis Sci 2022; 67:1733-1738. [PMID: 35394594 DOI: 10.1007/s10620-022-07498-5] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Accepted: 03/04/2022] [Indexed: 12/09/2022]
Abstract
Duodenal follicular lymphoma (DFL) is a rare variety of non-Hodgkin's lymphoma of the gastrointestinal tract that usually carries a favorable course, recognized as a new entity in 2016. It is usually diagnosed at an early stage located predominantly in the second portion of the duodenum. We report the case of a 74-year-old male patient with epigastric pain in whom gastroscopy revealed white mucosal nodules that were pathologically diagnosed as grade 1-2 DFL. Staging investigations revealed secondary lesions in the spleen and at the base of the tongue together with latero-cervical adenopathy. The tumor was stage IV according to the Lugano staging system. We reviewed the recent (last five years) literature defining the importance of combination therapy in the advanced stage. The patient achieved complete remission of the disease through chemoimmunotherapy following the Rituximab-Bendamustine scheme.
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Affiliation(s)
- M Varanese
- Department of Surgical Sciences, Sapienza University, Rome, Italy
| | - A Lauro
- Department of Surgical Sciences, Sapienza University, Rome, Italy
| | - I Lattina
- Department of Surgical Sciences, Sapienza University, Rome, Italy.
| | - D Tripodi
- Department of Surgical Sciences, Sapienza University, Rome, Italy
| | - T Daralioti
- Gastroenterology & Digestive Endoscopy, IRCCS Regina Elena National Cancer Institute, Rome, Italy
| | - S Khouzam
- Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, PA, USA
| | - I R Marino
- Sidney Kimmel Medical College, Thomas Jefferson University, Philadelphia, PA, USA
| | - V Stigliano
- Gastroenterology & Digestive Endoscopy, IRCCS Regina Elena National Cancer Institute, Rome, Italy
| | - V D'Andrea
- Department of Surgical Sciences, Sapienza University, Rome, Italy
| | - S Frattaroli
- Department of Surgical Sciences, Sapienza University, Rome, Italy
| | - S Sorrenti
- Department of Surgical Sciences, Sapienza University, Rome, Italy
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33
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Ahn JY. Gastrointestinal Tract Lymphoma. THE KOREAN JOURNAL OF HELICOBACTER AND UPPER GASTROINTESTINAL RESEARCH 2022. [DOI: 10.7704/kjhugr.2021.0064] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/22/2022]
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34
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Matysiak-Budnik T, Jamet P, Chapelle N, Fabiani B, Coppo P, Ruskoné-Fourmestraux A. Primary Gastrointestinal Follicular Lymphomas: A Prospective Study of 31 Patients with Long-term Follow-up Registered in the French Gastrointestinal Lymphoma Study Group (GELD) of the French Federation of Digestive Oncology (FFCD). Gut Liver 2022; 16:207-215. [PMID: 35249892 PMCID: PMC8924797 DOI: 10.5009/gnl210300] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/01/2021] [Revised: 10/22/2021] [Accepted: 10/29/2021] [Indexed: 11/04/2022] Open
Abstract
Background/Aims Methods Results Conclusions
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Affiliation(s)
- Tamara Matysiak-Budnik
- IMAD, Department of Gastroenterology and Digestive Oncology, Hotel Dieu Hospital, Nantes, France
- UMR Inserm 1235, Nantes, France
| | - Philippe Jamet
- IMAD, Department of Gastroenterology and Digestive Oncology, Hotel Dieu Hospital, Nantes, France
| | - Nicolas Chapelle
- IMAD, Department of Gastroenterology and Digestive Oncology, Hotel Dieu Hospital, Nantes, France
- INSERM UMR 1064, University of Nantes, Nantes, France
| | - Bettina Fabiani
- Department of Pathology, Saint Antoine Hospital AP-HP, Paris, France
| | - Paul Coppo
- Department of Hematology, Saint Antoine Hospital AP-HP, Paris, France
| | - Agnès Ruskoné-Fourmestraux
- Gastroenterology, Saint Antoine Hospital AP-HP, Paris, France
- French Federation of Digestive Oncology, Dijon, France
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35
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Lim JH. Incidentally Found Mucosal Nodularity on the Second Portion of Duodenum. THE KOREAN JOURNAL OF HELICOBACTER AND UPPER GASTROINTESTINAL RESEARCH 2022. [DOI: 10.7704/kjhugr.2021.0048] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/22/2022]
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36
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Suzuki H, Hamada Y, Tanaka K, Horiki N, Nakagawa H. Primary Gastrointestinal Follicular Lymphoma Presenting With Bowel Stenosis. Cureus 2022; 14:e21278. [PMID: 35178328 PMCID: PMC8843071 DOI: 10.7759/cureus.21278] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 01/15/2022] [Indexed: 11/27/2022] Open
Abstract
Primary follicular lymphomas of the small bowel generally present with small whitish nodules and masses or polyp-like lesions; cases with other morphologies are extremely rare. We experienced a case of primary follicular lymphoma that presented with small bowel stenosis. The lesion needed to be differentiated from other causes, such as bowel tuberculosis, non-steroidal anti-inflammatory drug-related ulcers, Crohn’s disease, small bowel ischemia, trauma, and idiopathic bowel stenosis, but endoscopic biopsies did not result in a definite diagnosis. Therefore, the lesion was surgically resected and, consequently, a diagnosis of follicular lymphoma of the small bowel was finally made. We report the characteristics and macroscopic findings of follicular lymphoma of the small bowel along with a review of relevant literature.
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37
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Silva JF, Morais R, Fonseca E, Macedo G. A case of duodenal-type follicular lymphoma. REVISTA ESPANOLA DE ENFERMEDADES DIGESTIVAS 2021; 114:289-290. [PMID: 34470459 DOI: 10.17235/reed.2021.8233/2021] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 11/20/2022]
Abstract
An 83 years-old female with history of silicosis was referred for upper endoscopy for duodenal wall thickness detected in thoracic computer tomography (CT). Upper endoscopy revealed multiple small (1 to 5mm) whitish polypoid lesions, involving a discrete portion of the descending part of the duodenum with 3cm of extension.
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Affiliation(s)
| | - Rui Morais
- Gastrenterology, Centro Hospitalar Universitário de São João, Portugal
| | - Elsa Fonseca
- Gastroenterology, Hospital de São João, Portugal
| | - Guilherme Macedo
- Gastroenterology, Centro Hospitalar Universitário de São João, Portugal
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38
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Desai S, Moustafa MA, Wiseman G, Witzig T. Ibritumomab Tiuxetan Radioimmunotherapy for Primary Gastrointestinal Follicular Lymphoma. Oncologist 2021; 26:e2079-e2081. [PMID: 34227176 DOI: 10.1002/onco.13895] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/24/2021] [Accepted: 06/29/2021] [Indexed: 12/23/2022] Open
Abstract
Primary gastrointestinal follicular lymphoma (PGIFL) is characterized by localized involvement of the gastrointestinal (GI) tract. PGIFL usually runs an indolent course. External beam radiation therapy is curative in a substantial proportion of localized follicular lymphomas, but GI toxicities may discourage its use in PGIFL. Ibritumomab tiuxetan radioimmunotherapy (RIT) is a radioimmunoconjugate of anti-CD20 monoclonal antibody linked to chelator tiuxetan and radioisotope. RIT delivers confined high-intensity radiation with short path length specifically targeting lymphoma cells and sparing normal tissue. In this case series report, we included six cases of PGIFL treated with RIT. All patients had low-risk, localized, and nonbulky disease. All patients responded completely and were relapse-free for the duration of follow-up. Hematologic toxicities were seen, but none were serious. RIT is a potentially curative treatment option in PGIFL with a tolerable toxicity profile.
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Affiliation(s)
- Sanjal Desai
- Department of Hematology, Mayo Clinic, Rochester, Minnesota, USA
| | | | - Gregory Wiseman
- Department of Nuclear Medicine, Mayo Clinic, Rochester, Minnesota, USA
| | - Thomas Witzig
- Department of Hematology, Mayo Clinic, Rochester, Minnesota, USA
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39
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Quintanilla‐Martinez L. Recognizing but not harming. Borderline B‐cell lymphoid proliferations. Hematol Oncol 2021. [DOI: 10.1002/hon.2854] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/09/2022]
Affiliation(s)
- Leticia Quintanilla‐Martinez
- Institute of Pathology and Neuropathology Eberhard Karls University of Tübingen and Comprehensive Cancer Center Tübingen University Hospital Tübingen Germany
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40
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Toth L, Vasef MA. Molecular Pathogenesis of Primary Gastrointestinal Tract Lymphomas. Semin Diagn Pathol 2021; 38:46-52. [PMID: 33985832 DOI: 10.1053/j.semdp.2021.04.003] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/08/2021] [Revised: 04/07/2021] [Accepted: 04/21/2021] [Indexed: 12/15/2022]
Abstract
Primary gastrointestinal lymphomas are rare though the incidence is significantly increased among adult patients in recent years. The majority of the patients present with symptoms overlapping with other gastrointestinal disorders and imaging findings are not specific. Therefore, histologic examination is necessary to establish the diagnosis. Insight into etiologies, molecular pathogenesis and critical signaling pathways in lymphomas including gastrointestinal lymphomas has significantly expanded within the last 3 decades. Given the increasing demand for incorporation of genetic data, the appropriate handling and processing of small endoscopic gastrointestinal biopsy samples of suspected lymphoma is becoming extremely crucial and at times challenging. The use of next generation sequencing with analysis of genes relevant to diagnosis, prognosis, and therapeutic targets continues to have a significant promising impact on management of patients in lymphoid malignancies. In particular, the identification of constitutively activated pathways and the emergence of novel targeted medications predict that more effective therapies will be identified for these disorders in the coming years.
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Affiliation(s)
- Laura Toth
- Department of Pathology, University of New Mexico Health Sciences Center, Albuquerque, New Mexico, USA
| | - Mohammad A Vasef
- Department of Pathology, University of New Mexico Health Sciences Center, Albuquerque, New Mexico, USA.
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41
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Alvarez-Lesmes J, Chapman JR, Cassidy D, Zhou Y, Garcia-Buitrago M, Montgomery EA, Lossos IS, Sussman D, Poveda J. Gastrointestinal Tract Lymphomas: A Review of the Most Commonly Encountered Lymphomas. Arch Pathol Lab Med 2021; 145:1585-1596. [PMID: 33836528 DOI: 10.5858/arpa.2020-0661-ra] [Citation(s) in RCA: 16] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 01/04/2021] [Indexed: 11/06/2022]
Abstract
CONTEXT.— The gastrointestinal (GI) tract is the most common site of extranodal non-Hodgkin lymphoma, accounting for 20% to 40% of all extranodal lymphomas. The majority of these are systemic processes secondarily involving the GI tract. Primary GI lymphomas are less common, accounting for approximately 10% to 15% of all non-Hodgkin lymphoma. Most non-Hodgkin lymphoma involving the GI tract are of B-cell lineage, of which diffuse large B-cell lymphoma is the most common subtype, irrespective of location. OBJECTIVE.— To review the lymphoproliferative neoplasms of B-cell and T-cell lineage involving the luminal GI tract according to the most prevalent subtypes at each anatomic site. DATA SOURCE.— Systematic search of the PubMed database for updated literature on GI lymphomas epidemiology, subtypes, clinical, endoscopic, and genetic findings. Histologic images are derived from our collection of clinical cases. CONCLUSIONS.— The GI tract is the most common site of extranodal lymphoproliferative neoplasms. Recognition of the most frequently encountered GI lymphomas is imperative for patient management and treatment.
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Affiliation(s)
- Jessica Alvarez-Lesmes
- From the Department of Pathology, Division of Hematopathology, Division of Gastrointestinal Pathology, University of Miami Miller School of Medicine, Sylvester Comprehensive Cancer Center and Jackson Memorial Hospital, Miami, Florida (Alvarez-Lesmes, Chapman, Cassidy, Zhou, Garcia-Buitrago, Montgomery, Poveda)
| | - Jennifer R Chapman
- From the Department of Pathology, Division of Hematopathology, Division of Gastrointestinal Pathology, University of Miami Miller School of Medicine, Sylvester Comprehensive Cancer Center and Jackson Memorial Hospital, Miami, Florida (Alvarez-Lesmes, Chapman, Cassidy, Zhou, Garcia-Buitrago, Montgomery, Poveda)
| | - Daniel Cassidy
- From the Department of Pathology, Division of Hematopathology, Division of Gastrointestinal Pathology, University of Miami Miller School of Medicine, Sylvester Comprehensive Cancer Center and Jackson Memorial Hospital, Miami, Florida (Alvarez-Lesmes, Chapman, Cassidy, Zhou, Garcia-Buitrago, Montgomery, Poveda)
| | - Yi Zhou
- From the Department of Pathology, Division of Hematopathology, Division of Gastrointestinal Pathology, University of Miami Miller School of Medicine, Sylvester Comprehensive Cancer Center and Jackson Memorial Hospital, Miami, Florida (Alvarez-Lesmes, Chapman, Cassidy, Zhou, Garcia-Buitrago, Montgomery, Poveda)
| | - Monica Garcia-Buitrago
- From the Department of Pathology, Division of Hematopathology, Division of Gastrointestinal Pathology, University of Miami Miller School of Medicine, Sylvester Comprehensive Cancer Center and Jackson Memorial Hospital, Miami, Florida (Alvarez-Lesmes, Chapman, Cassidy, Zhou, Garcia-Buitrago, Montgomery, Poveda)
| | - Elizabeth A Montgomery
- From the Department of Pathology, Division of Hematopathology, Division of Gastrointestinal Pathology, University of Miami Miller School of Medicine, Sylvester Comprehensive Cancer Center and Jackson Memorial Hospital, Miami, Florida (Alvarez-Lesmes, Chapman, Cassidy, Zhou, Garcia-Buitrago, Montgomery, Poveda)
| | - Izidore S Lossos
- Division of Hematology, Department of Medicine, University of Miami Miller School of Medicine, Sylvester Comprehensive Cancer Center, Miami, Florida (Lossos).,Department of Molecular and Cellular Pharmacology (Lossos), University of Miami Miller School of Medicine, Miami, Florida
| | - Daniel Sussman
- Division of Digestive Health and Liver Diseases (Sussman), University of Miami Miller School of Medicine, Miami, Florida
| | - Julio Poveda
- From the Department of Pathology, Division of Hematopathology, Division of Gastrointestinal Pathology, University of Miami Miller School of Medicine, Sylvester Comprehensive Cancer Center and Jackson Memorial Hospital, Miami, Florida (Alvarez-Lesmes, Chapman, Cassidy, Zhou, Garcia-Buitrago, Montgomery, Poveda)
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42
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Plummer RM, Linden MA, Beckman AK. Update on B-cell lymphoproliferative disorders of the gastrointestinal tract. Semin Diagn Pathol 2021; 38:14-20. [PMID: 33863577 DOI: 10.1053/j.semdp.2021.03.006] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/31/2021] [Revised: 02/25/2021] [Accepted: 03/22/2021] [Indexed: 12/14/2022]
Abstract
The gastrointestinal (GI) tract is home to a significant portion of the immune system, which interacts daily with the antigenic milieu of its contents. Therefore, the presence of white blood cells within the walls of the GI tract upon histologic examination is a familiar sight on GI biopsies-both in health and disease. The GI tract is the most common site of extranodal lymphomas, most of which are B-cell neoplasms. Here, we review common and uncommon B-cell neoplasms of the GI tract - extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma), mantle cell lymphoma, duodenal-type follicular lymphoma, diffuse large B-cell lymphoma, plasmablastic lymphoma, EBV-positive mucocutaneous ulcer, and post-transplant lymphoproliferative disorders - with special focus on literature published during the past five years. Along with the other articles in this edition of Seminars in Diagnostic Pathology, it is the authors' hope that this review proves to be a useful resource in the workup of the array of hematopoietic processes that can involve the GI tract.
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Affiliation(s)
- Regina M Plummer
- Department of Pathology and Laboratory Medicine, University of Kansas Medical Center, Kansas City, USA
| | - Michael A Linden
- Department of Laboratory Medicine and Pathology, University of Minnesota Medical School, 420 Delaware St. SE, Minneapolis, MN 55455, USA
| | - Amy K Beckman
- Department of Laboratory Medicine and Pathology, University of Minnesota Medical School, 420 Delaware St. SE, Minneapolis, MN 55455, USA.
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43
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Dreyling M, Ghielmini M, Rule S, Salles G, Ladetto M, Tonino SH, Herfarth K, Seymour JF, Jerkeman M. Newly diagnosed and relapsed follicular lymphoma: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol 2021; 32:298-308. [PMID: 33249059 DOI: 10.1016/j.annonc.2020.11.008] [Citation(s) in RCA: 145] [Impact Index Per Article: 36.3] [Reference Citation Analysis] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/01/2020] [Revised: 11/10/2020] [Accepted: 11/10/2020] [Indexed: 12/25/2022] Open
Affiliation(s)
- M Dreyling
- Department of Medicine III, LMU Hospital Munich, Germany
| | - M Ghielmini
- Oncology Institute of Southern Switzerland, Ospedale San Giovanni, Bellinzona, Switzerland
| | - S Rule
- Haematology, Peninsula School of Medicine, Plymouth, UK
| | - G Salles
- Service d'Hématologie, Hospices Civils de Lyon, Centre Hospitalier Lyon-Sud, Lyon; Université Claude Bernard Lyon-1, Pierre-Benite, France
| | - M Ladetto
- Divisione di Ematologia, Azienda Ospedaliera Santi Antonio e Biagio e Cesare Arrigo, Alessandria, Italy
| | - S H Tonino
- Department of Hematology, Amsterdam UMC, University of Amsterdam, Amsterdam, the Netherlands
| | - K Herfarth
- Department of Radiation Oncology, University of Heidelberg, Germany
| | - J F Seymour
- Department of Haematology, Peter McCallum Cancer Center & Royal Melbourne Hospital, Melbourne, University of Melbourne, Parkville, Australia
| | - M Jerkeman
- Department of Oncology, Skåne University Hospital, Lund University, Lund, Sweden
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44
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Lumish M, Falchi L, Imber BS, Scordo M, von Keudell G, Joffe E. How we treat mature B-cell neoplasms (indolent B-cell lymphomas). J Hematol Oncol 2021; 14:5. [PMID: 33407745 PMCID: PMC7789477 DOI: 10.1186/s13045-020-01018-6] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/26/2020] [Accepted: 12/02/2020] [Indexed: 12/30/2022] Open
Abstract
Mature B cell neoplasms, previously indolent non-Hodgkin lymphomas (iNHLs), are a heterogeneous group of malignancies sharing similar disease courses and treatment paradigms. Most patients with iNHL have an excellent prognosis, and in many, treatment can be deferred for years. However, some patients will have an accelerated course and may experience transformation into aggressive lymphomas. In this review, we focus on management concepts shared across iNHLs, as well as histology-specific strategies. We address open questions in the field, including the influence of genomics and molecular pathway alterations on treatment decisions. In addition, we review the management of uncommon clinical entities including nodular lymphocyte-predominant Hodgkin lymphoma, hairy cell leukemia, splenic lymphoma and primary lymphoma of extranodal sites. Finally, we include a perspective on novel targeted therapies, antibodies, antibody-drug conjugates, bispecific T cell engagers and chimeric antigen receptor T cell therapy.
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Affiliation(s)
- Melissa Lumish
- Lymphoma Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, 1275 York Avenue, SR-441B, New York, NY, 10065, USA
| | - Lorenzo Falchi
- Lymphoma Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, 1275 York Avenue, SR-441B, New York, NY, 10065, USA
| | - Brandon S Imber
- Lymphoma Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, 1275 York Avenue, SR-441B, New York, NY, 10065, USA
| | - Michael Scordo
- Lymphoma Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, 1275 York Avenue, SR-441B, New York, NY, 10065, USA
| | - Gottfried von Keudell
- Lymphoma Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, 1275 York Avenue, SR-441B, New York, NY, 10065, USA
| | - Erel Joffe
- Lymphoma Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, 1275 York Avenue, SR-441B, New York, NY, 10065, USA.
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45
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Foukas PG, Bisig B, de Leval L. Recent advances upper gastrointestinal lymphomas: molecular updates and diagnostic implications. Histopathology 2020; 78:187-214. [PMID: 33382495 DOI: 10.1111/his.14289] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/06/2020] [Revised: 10/21/2020] [Accepted: 10/22/2020] [Indexed: 12/14/2022]
Abstract
Approximately one-third of extranodal non-Hodgkin lymphomas involve the gastrointestinal (GI) tract, with the vast majority being diagnosed in the stomach, duodenum, or proximal small intestine. A few entities, especially diffuse large B-cell lymphoma and extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue, represent the majority of cases. In addition, there are diseases specific to or characteristic of the GI tract, and any type of systemic lymphoma can present in or disseminate to these organs. The recent advances in the genetic and molecular characterisation of lymphoid neoplasms have translated into notable changes in the classification of primary GI T-cell neoplasms and the recommended diagnostic approach to aggressive B-cell tumours. In many instances, diagnoses rely on morphology and immunophenotype, but there is an increasing need to incorporate molecular genetic markers. Moreover, it is also important to take into consideration the endoscopic and clinical presentations. This review gives an update on the most recent developments in the pathology and molecular pathology of upper GI lymphoproliferative diseases.
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Affiliation(s)
- Periklis G Foukas
- Second Department of Pathology, National and Kapodistrian University of Athens, Attikon University Hospital, Athens, Greece
| | - Bettina Bisig
- Department of Laboratory Medicine and Pathology, Institute of Pathology, Lausanne University Hospital and Lausanne University, Lausanne, Switzerland
| | - Laurence de Leval
- Second Department of Pathology, National and Kapodistrian University of Athens, Attikon University Hospital, Athens, Greece
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46
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Abe R, Mori T, Tanigawa T, Koda Y, Hosoe N, Sakurai M, Kikuchi T, Kato J, Shimizu T, Ogata H, Kanai T, Sasaki A, Kameyama K, Okamoto S. Clinical characteristics and outcomes of duodenal-type follicular lymphoma. Leuk Lymphoma 2020; 61:3266-3268. [PMID: 32696685 DOI: 10.1080/10428194.2020.1795162] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/04/2022]
Affiliation(s)
- Ryohei Abe
- Division of Hematology, Department of Medicine, Keio University School of Medicine, Tokyo, Japan
| | - Takehiko Mori
- Division of Hematology, Department of Medicine, Keio University School of Medicine, Tokyo, Japan
| | - Tomohiko Tanigawa
- Division of Hematology, Department of Medicine, Keio University School of Medicine, Tokyo, Japan
| | - Yuya Koda
- Division of Hematology, Department of Medicine, Keio University School of Medicine, Tokyo, Japan
| | - Naoki Hosoe
- Center for Diagnostic and Therapeutic Endoscopy, Keio University Hospital, Tokyo, Japan
| | - Masatoshi Sakurai
- Division of Hematology, Department of Medicine, Keio University School of Medicine, Tokyo, Japan
| | - Taku Kikuchi
- Division of Hematology, Department of Medicine, Keio University School of Medicine, Tokyo, Japan
| | - Jun Kato
- Division of Hematology, Department of Medicine, Keio University School of Medicine, Tokyo, Japan
| | - Takayuki Shimizu
- Division of Hematology, Department of Medicine, Keio University School of Medicine, Tokyo, Japan
| | - Haruhiko Ogata
- Center for Diagnostic and Therapeutic Endoscopy, Keio University Hospital, Tokyo, Japan
| | - Takanori Kanai
- Division of Gastroenterology and Hepatology, Department of Medicine, Keio University School of Medicine, Tokyo, Japan
| | - Aya Sasaki
- Department of Diagnostic Pathology, Keio University Hospital, Tokyo, Japan
| | - Kaori Kameyama
- Department of Diagnostic Pathology, Keio University Hospital, Tokyo, Japan
| | - Shinichiro Okamoto
- Division of Hematology, Department of Medicine, Keio University School of Medicine, Tokyo, Japan
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47
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Li HN, Wang RC, Chen JP, Chang ST, Chuang SS. Density and size of lymphoid follicles are useful clues in differentiating primary intestinal follicular lymphoma from intestinal reactive lymphoid hyperplasia. Diagn Pathol 2020; 15:82. [PMID: 32635930 PMCID: PMC7341590 DOI: 10.1186/s13000-020-00991-3] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/11/2020] [Accepted: 06/17/2020] [Indexed: 12/27/2022] Open
Abstract
Background Primary intestinal follicular lymphoma (PI-FL) is a rare and indolent lymphoma and is challenging for diagnosis with endoscopic biopsy specimens. Whole slide imaging (WSI) has been increasingly used for assisting pathologic diagnosis, but not for lymphoma yet, probably because there are usually too many immunostained sections in a single case. In this study we attempted to identify morphological clues of PI-FL in the endoscopic biopsy specimens by measuring various parameters using WSI. Methods We retrospectively investigated 21 PI-FL cases, and scanned the HE sections from 17 of these cases with endoscopic biopsy specimens. Sections from 17 intestinal biopsies showing reactive lymphoid hyperplasia were scanned for comparison. The density and diameter of lymphoid follicles and the shortest distance of these follicles to the surface epithelia were measured on WSI. Comparisons of the aforementioned parameters were made between the neoplastic and reactive follicles. Results The density of follicles was significantly higher in PI-FL than that of reactive hyperplasia (median 0.5 vs. 0.2/mm2; p < 0.01). Furthermore, the neoplastic follicles were significantly larger (median diameter 756.9 vs. 479.7 μm; p < 0.01). The shortest distance of follicles to the surface epithelia tended to be closer in PI-FL (104.7 vs. 177.8 μm, p = 0.056), but not statistically significant. Conclusions In this study we found that in PI-FL the density and diameter of lymphoid follicles as measured from WSI were significantly different from that of intestinal reactive lymphoid hyperplasia. When facing the diagnostic challenge between these two entities in routine practice, pathologists might be alerted by these morphological clues and request for immunohistochemistry for differential diagnosis.
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Affiliation(s)
- Hsin-Ni Li
- Department of Pathology and Laboratory Medicine, Taichung Veterans General Hospital, Taichung, Taiwan
| | - Ren Ching Wang
- Department of Pathology and Laboratory Medicine, Taichung Veterans General Hospital, Taichung, Taiwan.,Department of Nursing, HungKuang University, Taichung, Taiwan
| | - Jun-Peng Chen
- Biostatistics Task Force, Taichung Veterans General Hospital, Taichung, Taiwan
| | - Sheng-Tsung Chang
- Department of Pathology, Chi-Mei Medical Center, 901 Chung-Hwa Road, Yong-Kang District, Tainan, 71004, Taiwan.,Department of Nursing, National Tainan Institute of Nursing, Tainan, Taiwan
| | - Shih-Sung Chuang
- Department of Pathology, Chi-Mei Medical Center, 901 Chung-Hwa Road, Yong-Kang District, Tainan, 71004, Taiwan. .,Department of Pathology, School of Medicine, College of Medicine, National Taiwan University, Taipei, Taiwan.
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48
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Scientific Advances and the Evolution of Diagnosis, Subclassification and Treatment of Lymphoma. Arch Med Res 2020; 51:749-764. [PMID: 32553461 DOI: 10.1016/j.arcmed.2020.05.022] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/12/2020] [Accepted: 05/26/2020] [Indexed: 12/11/2022]
Abstract
The diagnosis of lymphoma has evolved tremendously over time. Initially, diagnosis of lymphoma was largely based on morphology alone. Over time, immunophenotyping using flow cytometry and immunohistochemistry, and then in situ hybridization, have contributed dramatically to the pathologist's ability to recognize, diagnose and subclassify lymphomas more precisely. In recent years, cytogenetic and molecular genetic techniques have developed that allow evaluation of abnormalities in lymphomas, leading to an understanding of their pathogenesis and opening the door to targeted therapies that will lead to better outcomes for lymphoma patients.
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Katsurahara M, Umeda Y, Sakuno T, Tsuboi J, Yamada R, Nakamura M, Hamada Y, Inoue H, Tanaka K, Horiki N, Takei Y. Primary Colorectal Follicular Lymphoma Observed by Magnifying Endoscopy, with a Five-year Follow-up. Intern Med 2020; 59:1395-1399. [PMID: 32161216 PMCID: PMC7332620 DOI: 10.2169/internalmedicine.3995-19] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/30/2019] [Accepted: 01/23/2020] [Indexed: 11/30/2022] Open
Abstract
Colorectal involvement is very rare in cases of follicular lymphoma. Colonoscopy of a 69-year-old man revealed an aggregation of multiple whitish nodules in the sigmoid colon. Magnifying endoscopy with narrow-band imaging demonstrated a coiled and elongated microvascular pattern on the surface and crystal violet staining showed a type I pit pattern. A biopsy showed follicular lymphoma (Grade 1), and no other involvement of lymphoma was detected. Following a diagnosis of primary colorectal follicular lymphoma stage I (Lugano classification), the patient was monitored by watch-and-wait therapy. We documented the endoscopic images of colorectal follicular lymphoma in the present case.
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Affiliation(s)
- Masaki Katsurahara
- Department of Endoscopic Medicine, Mie University Graduate School of Medicine, Japan
| | - Yuhei Umeda
- Department of Gastroenterology and Hepatology, Mie University Graduate School of Medicine, Japan
| | - Takashi Sakuno
- Department of Gastroenterology and Hepatology, Mie University Graduate School of Medicine, Japan
| | - Jyunya Tsuboi
- Department of Gastroenterology and Hepatology, Mie University Graduate School of Medicine, Japan
| | - Reiko Yamada
- Department of Gastroenterology and Hepatology, Mie University Graduate School of Medicine, Japan
| | - Misaki Nakamura
- Department of Gastroenterology and Hepatology, Mie University Graduate School of Medicine, Japan
| | - Yasuhiko Hamada
- Department of Endoscopic Medicine, Mie University Graduate School of Medicine, Japan
| | - Hiroyuki Inoue
- Department of Gastroenterology and Hepatology, Mie University Graduate School of Medicine, Japan
| | - Kyosuke Tanaka
- Department of Endoscopic Medicine, Mie University Graduate School of Medicine, Japan
| | - Noriyuki Horiki
- Department of Endoscopic Medicine, Mie University Graduate School of Medicine, Japan
| | - Yoshiyuki Takei
- Department of Gastroenterology and Hepatology, Mie University Graduate School of Medicine, Japan
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Inoue H, Rai S, Tanaka H, Espinoza JL, Watatani Y, Kumode T, Serizawa K, Nakayama S, Taniguchi Y, Morita Y, Tatsumi Y, Ashida T, Matsumura I. Tumour-immune microenvironment in duodenal-type follicular lymphoma. Br J Haematol 2020; 191:243-252. [PMID: 32383789 DOI: 10.1111/bjh.16715] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/06/2020] [Revised: 04/04/2020] [Accepted: 04/12/2020] [Indexed: 01/29/2023]
Abstract
Despite duodenal-type follicular lymphoma (DTFL) being morphologically, immunophenotypically and genetically indistinguishable from nodal FL (nFL), this entity typically shows a significantly better prognosis. Here, we analysed the tumour immune microenvironments of diagnostic specimens from patients with DTFL (n = 30), limited-stage FL (LSFL; n = 19) and advanced-stage FL (ASFL; n = 31). The mean number of CD8+ tumour-infiltrating lymphocytes (TILs) in the neoplastic follicles was higher in DTFL (1,827/mm2 ) than in LSFL (1,150/mm2 ) and ASFL (1,188/mm2 ) (P = 0·002, P = 0·002, respectively). In addition, CD8+ PD1- T cells with non-exhausting phenotype were more abundant in the peripheral blood (PB) of DTFL than in LSFL and ASFL, indicating that DTFL may exhibit a better and longer-lasting T cell-mediated immune response. Moreover, whereas FOXP3+ CTLA-4+ effector regulatory T cells (eTregs) were rarely observed in the neoplastic follicles of DTFL (mean: 12/mm2 ), they were more abundant in LSFL (78/mm2 ) and ASFL (109/mm2 ) (P = 2·80 × 10-5 , P = 4·74 × 10-8 , respectively), and the numbers of eTregs correlated inversely with those of CD8+ TILs (r = -0267; P = 0·018). Furthermore, DTFL showed significantly fewer circulating FOXP3hi CD45RA- CD25hi eTregs (0·146%) than ASFL (0·497%) and healthy controls (0·639%) (P = 0·0003, P = 6·79 × 10-7 , respectively). These results suggest that the augmented anti-tumour immune reactions may contribute to a better prognosis on DTFL.
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Affiliation(s)
- Hiroaki Inoue
- Department of Hematology and Rheumatology, Faculty of Medicine, Kindai University Hospital, Osakasayama, Japan
| | - Shinya Rai
- Department of Hematology and Rheumatology, Faculty of Medicine, Kindai University Hospital, Osakasayama, Japan
| | - Hirokazu Tanaka
- Department of Hematology and Rheumatology, Faculty of Medicine, Kindai University Hospital, Osakasayama, Japan
| | - J Luis Espinoza
- Department of Hematology and Rheumatology, Faculty of Medicine, Kindai University Hospital, Osakasayama, Japan
| | - Yosaku Watatani
- Department of Hematology and Rheumatology, Faculty of Medicine, Kindai University Hospital, Osakasayama, Japan
| | - Takahiro Kumode
- Department of Hematology and Rheumatology, Faculty of Medicine, Kindai University Hospital, Osakasayama, Japan
| | - Kentaro Serizawa
- Department of Hematology and Rheumatology, Faculty of Medicine, Kindai University Hospital, Osakasayama, Japan
| | - Shoko Nakayama
- Department of Hematology and Rheumatology, Faculty of Medicine, Kindai University Hospital, Osakasayama, Japan
| | - Yasuhiro Taniguchi
- Department of Hematology and Rheumatology, Faculty of Medicine, Kindai University Hospital, Osakasayama, Japan
| | - Yasuyoshi Morita
- Department of Hematology and Rheumatology, Faculty of Medicine, Kindai University Hospital, Osakasayama, Japan
| | - Yoichi Tatsumi
- Department of Hematology and Rheumatology, Faculty of Medicine, Kindai University Hospital, Osakasayama, Japan
| | - Takashi Ashida
- Department of Hematology and Rheumatology, Faculty of Medicine, Kindai University Hospital, Osakasayama, Japan
| | - Itaru Matsumura
- Department of Hematology and Rheumatology, Faculty of Medicine, Kindai University Hospital, Osakasayama, Japan
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