Primary Esophageal Extranodal Marginal Zone Lymphoma: A Rare Tumor

Primary Esophageal Extranodal Marginal Zone Lymphoma: A Rare Tumor | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Case Report Primary Esophageal Extranodal Marginal Zone Lymphoma: A Rare Tumor Yekta Altemur KARAMUSTAFAOGLU, Nuray CAN, Fazli YANIK, Yener YORUK This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-7085475/v1 This work is licensed under a CC BY 4.0 License Status: Published Journal Publication published 25 Jul, 2025 Read the published version in Digestive Diseases and Sciences → Version 1 posted 4 You are reading this latest preprint version Abstract Primary esophageal extranodal marginal zone lymphoma (ENMZL) is a rare and challenging malignancy to diagnose. A case involving a 66-year-old woman with a 6-month history of worsening dysphagia, odynophagia, and weight loss is presented. Imaging studies revealed a large mediastinal mass and a distal esophageal mass causing partial obstruction, with no evidence of enlarged lymph nodes. Despite biopsies from esophagoscopy and endoscopic ultrasound not providing a definitive diagnosis, the patient underwent surgical excision of the middle esophageal mass. Histopathological analysis confirmed primary ENMZL. The patient was subsequently referred for chemotherapy as stage I ENMZL. Treatment strategies may vary, typically involving a combination of chemotherapy and radiotherapy, with surgical options considered as well. Raising awareness of this rare tumor among medical professionals is vital for improving patient outcomes. Esophagus mass lymphoma Figures Figure 1 Figure 2 Introduction Primary esophageal extranodal marginal zone lymphoma (ENMZL) is a rare malignancy with diagnostic challenges. Treatment typically involves a combination of chemotherapy and radiotherapy, with surgery as an alternative option depending on individual circumstances. Raising awareness of this uncommon tumor among medical professionals is essential for enhancing patient outcomes. While most esophageal cancers are squamous cell carcinoma or adenocarcinoma, lymphoma can occur anywhere in the gastrointestinal system, either as a primary or secondary event. Although rare, primary esophageal ENMZL presents significant diagnostic difficulties. This case report includes relevant imaging studies, esophagoscopy results, and surgical findings. Case Report The case describes a 66-year-old woman experiencing severe swallowing difficulties that significantly affected her daily life. She had no history of smoking or alcohol consumption, and her physical examination showed no notable findings. Her medical history included polyarthritis managed with etanercept for a decade. While routine blood tests and chest X-rays were unremarkable, thoracic computed tomography revealed two masses in the esophagus—one in the middle (27×21×48 mm) and another in the distal esophagus (22×22×30 mm)—without lymph node enlargement. A positron emission tomography scan reflected significant mass metabolic activity, suggesting malignancy. Endoscopy showed submucosal lesions protruding into the esophageal cavity, though biopsies failed to provide a definitive diagnosis, necessitating surgical intervention. During surgery, a right mini-thoracotomy was performed to excise the upper esophageal mass, with a complication of mucosal perforation that was successfully repaired. Post-surgery, the patient recovered well, and histopathological analysis confirmed ENMZL. The patient was subsequently referred to a hematology clinic for chemotherapy planning. There is no clinical trial number for a case report. Discussion The gastrointestinal system is a common site for extranodal non-Hodgkin’s lymphoma, accounting for 10% to 20% of all cases [1]. In clinical practice, gastrointestinal involvement is observed in approximately 4% of non-Hodgkin’s lymphoma cases [1,2]. Primary esophageal lymphoma is rare, with only 15 cases identified in a study of 785 articles published between 1963 and 2020 [3]. Dysphagia was the most frequently reported symptom in these cases, similar to the symptoms presented by our patient. Diagnosis criteria for primary esophageal lymphoma include the absence of superficial lymphadenopathy, definitive mediastinal lymph node enlargement, liver or spleen involvement, and predominant esophageal engagement. Our patient fulfilled all these criteria [4]. While the underlying causes of primary esophageal lymphoma are not clearly understood, it is believed to arise from the submucosal lymphoid tissue of the esophagus [5]. Endoscopic findings in previous cases have varied, including ulcers and submucosal masses. In this case, multiple submucosal masses were observed. A surgical procedure was required due to the inability to obtain a satisfactory diagnosis via endoscopy. Postoperative analysis confirmed B-cell type non-Hodgkin lymphoma. ENMZL usually affects the gastrointestinal tract, particularly the stomach, and is uncommon in the esophagus. Tumor cells resemble marginal zone B cells. Differentiating ENMZL from reactive follicular hyperplasia or other small B-cell lymphomas is crucial, with immunohistochemistry playing a key role in the diagnosis [6]. ENMZL cells express specific B-cell markers, requiring a specialized antibody panel for differentiation. Prognosis is generally favorable but depends on factors, including age, comorbidities, disease stage, and treatment response. Due to the rarity of esophageal ENMZL, individualized treatment plans are essential. Although data are limited, survival rates range from 26% to 73%. [7]. Standard treatment typically involves chemotherapy and radiotherapy, with surgery considered in some cases. However, radiotherapy presents challenges due to the proximity of vital organs [8]. Chemotherapy regimens are utilized for advanced cases or when surgical resection is not feasible. A collaborative approach involving various medical specialists assists in choosing the best treatment for each patient. The prognosis for ENMZL is generally favorable, reflecting its slow progression; however, an advanced-stage disease with local invasion or metastasis is associated with poorer outcomes. The complete removal of a tumor depends on its size and location. In this case, surgical intervention was limited due to multiple masses, leading to a referral for chemotherapy. No signs of disease recurrence were noted after one month; nonetheless, the patient opted to continue her chemotherapy in her home country. Effective management of this rare cancer requires close collaboration among specialists. The nonspecific early symptoms emphasize the need for increased awareness among healthcare providers when encountering unexplained esophageal symptoms, especially in older adults or those with chronic inflammatory conditions. The lack of focused research on esophageal ENMZL highlights the necessity for greater attention in this understudied area of oncology. Declarations Author contributions: Yekta Altemur Karamustafaoglu and Nuray Can contributed to the submitted manuscript's conception, drafting, and editing. Fazli Yanik and Yener Yoruk contributed to the literature review. All authors reviewed and approved the final version of the manuscript. Yekta Altemur Karamustafaoglu is the article guarantor. I declare that I have not sent my article to another journal for simultaneous review. Funding The author(s) received no financial support for this article's research, authorship, and/or publication. Data Availability The data and material related to this case report are available. Materials Availability The data and material related to this case report are available. Clinic Trial Number Not applicable Ethics declarations Conflict of interest The authors declare that they have no competing interests. Ethical approval This study was exempted from ethical approval in our institution. Consent to participate This study was exempted from ethical approval in our institution. Consent for publication Written informed consent to publication form was obtained from the patient. References Herrmann R, Panahon A, Barcos MP, Walsh D, Stutzman L. Gastrointestinal involvement in non-Hodgkin’s lymphoma. Cancer 1980;46:215-22. Soon MS, Yen HH, Soon A, Lin OS Primary esophageal B-cell lymphoma: evaluation by EUS. Gastrointest Endosc.2005;61(7):901-3. Qu J, Zhuang Y, Zheng D, Huang F, Zhang S. Primary Esophageal Lymphoma: Clinical Experience in Diagnosis and Treatment. Cureus. 2021;1;13(9):e17628. doi: 10.7759/cureus.17628. eCollection 2021 Sep. Dawson IM, Cornes JS, Morson BC. Primary malignant lymphoid tumors of the intestinal tract. Report of 37 cases with a study of factors influencing prognosis. Br J Surg 1961;49:80-9. Agha FP, Schnitzer B. Esophageal involvement in lymphoma. Am J Gastroenterol 1985;80:412-6. Tabibian JH, Kalani A, Moran AM, Panganamamula K. Extranodal Marginal Zone B Cell (MALT) Lymphoma of the Esophagus. J Gastrointest Cancer. 2019;50(4):1034-36. Inayat F, Munir A, Wahab A, Younus F, Zafar F, Ullah W. Primary Esophageal Diffuse Large B-Cell Lymphoma: A Comparative Review of 15 Cases. Journal of Investigative Medicine High Impact Case Reports Volume 2018; 6: 1–6. Freret ME, Tringale KR, Boe L, Imber BS, Joffe E, Yahalom J, Hajj C. "Very low-dose radiotherapy for extranodal marginal zone lymphoma of bronchus-associated lymphoid tissue. Leukemia and Lymphoma. 2023;64(13):2195-2201. Additional Declarations No competing interests reported. Cite Share Download PDF Status: Published Journal Publication published 25 Jul, 2025 Read the published version in Digestive Diseases and Sciences → Version 1 posted Editorial decision: Revision requested 13 Jul, 2025 Editor assigned by journal 13 Jul, 2025 Submission checks completed at journal 11 Jul, 2025 First submitted to journal 09 Jul, 2025 You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. As a division of Research Square Company, we’re committed to making research communication faster, fairer, and more useful. We do this by developing innovative software and high quality services for the global research community. Our growing team is made up of researchers and industry professionals working together to solve the most critical problems facing scientific publishing. Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-7085475","acceptedTermsAndConditions":true,"allowDirectSubmit":false,"archivedVersions":[],"articleType":"Case Report","associatedPublications":[],"authors":[{"id":483555037,"identity":"bc88bee7-4267-43ab-aee2-014aaa85841e","order_by":0,"name":"Yekta Altemur KARAMUSTAFAOGLU","email":"data:image/png;base64,iVBORw0KGgoAAAANSUhEUgAAAZAAAAAyAQMAAABI0h/eAAAABlBMVEX///8AAABVwtN+AAAACXBIWXMAAA7EAAAOxAGVKw4bAAAA2ElEQVRIiWNgGAWjYDACdghlwC8BpiVkCGthZoZokZzBwNgA1MJDvBaDG2AtDIS18DfzH3x0s+2esfHt5uOPbtRY8DCwHz66AZ8WicPMzMa5bcVmZneOJTbnHAM6jCct7QZeaw4zs0nntiXYmN3IMWzOYQNqkeAxw6tF/jAz+2+QFuMZIC3/iNBiALSFGajFzEACqCW3jQgthoeZjaVzziUYS9xIS5yd2yfBw0bIL3LHGx9+zilLMOyfkXzgc863Ojl+9sPH8HsfA7CRpnwUjIJRMApGATYAANKSQYVRdJrmAAAAAElFTkSuQmCC","orcid":"","institution":"Trakya University","correspondingAuthor":true,"prefix":"","firstName":"Yekta","middleName":"Altemur","lastName":"KARAMUSTAFAOGLU","suffix":""},{"id":483555038,"identity":"c2390f96-e636-4da3-89b6-316ffcaad381","order_by":1,"name":"Nuray CAN","email":"","orcid":"","institution":"Trakya University","correspondingAuthor":false,"prefix":"","firstName":"Nuray","middleName":"","lastName":"CAN","suffix":""},{"id":483555039,"identity":"fc7922f7-0c40-4bbf-9b5b-624329b288ef","order_by":2,"name":"Fazli YANIK","email":"","orcid":"","institution":"Trakya University","correspondingAuthor":false,"prefix":"","firstName":"Fazli","middleName":"","lastName":"YANIK","suffix":""},{"id":483555040,"identity":"c9dabbb4-37b5-4dc9-a512-4f13f0a6d8ef","order_by":3,"name":"Yener YORUK","email":"","orcid":"","institution":"Trakya University","correspondingAuthor":false,"prefix":"","firstName":"Yener","middleName":"","lastName":"YORUK","suffix":""}],"badges":[],"createdAt":"2025-07-09 15:38:25","currentVersionCode":1,"declarations":"","doi":"10.21203/rs.3.rs-7085475/v1","doiUrl":"https://doi.org/10.21203/rs.3.rs-7085475/v1","draftVersion":[],"editorialEvents":[{"content":"https://doi.org/10.1007/s10620-025-09273-8","type":"published","date":"2025-07-25T15:57:56+00:00"}],"editorialNote":"","failedWorkflow":false,"files":[{"id":86666755,"identity":"2f923d04-8460-46df-b6f0-e839b3851ea4","added_by":"auto","created_at":"2025-07-14 11:09:18","extension":"png","order_by":1,"title":"Figure 1","display":"","copyAsset":false,"role":"figure","size":2623994,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cstrong\u003eA:\u003c/strong\u003eMediastinal mass in the middle and distal esophagus encompassing the lumen of the esophagus in thorax computed tomography \u003cstrong\u003e1B, C:\u003c/strong\u003eEsophagoscopy shows multiple endoluminal mass.\u003c/p\u003e","description":"","filename":"Figure1.png","url":"https://assets-eu.researchsquare.com/files/rs-7085475/v1/7d206a560784f90ee84a582d.png"},{"id":86667933,"identity":"992aca76-c65d-4aed-942a-daa1d089da27","added_by":"auto","created_at":"2025-07-14 11:17:19","extension":"png","order_by":2,"title":"Figure 2","display":"","copyAsset":false,"role":"figure","size":706090,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cstrong\u003eA:\u003c/strong\u003e Subepithelial nodular infiltration of lymphoid cells with pale cytoplasm (H\u0026amp;EX 40).\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eFigure 2B:\u003c/strong\u003eNeoplastic have small-medium sized nuclei with irregular contours and relatively abundant pale cytoplasms (H\u0026amp;EX 200, thumbnail; H\u0026amp;EX400)\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eFigure 2C:\u003c/strong\u003e Immune expression of CD20 in neoplastic lymphoid cells with pale cytoplasm (H\u0026amp;EX 40).\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eFigure 2D:\u003c/strong\u003e Low Ki-67 proliferating index in neoplastic lymphoid cells (H\u0026amp;EX 40).\u003c/p\u003e","description":"","filename":"Figure2.png","url":"https://assets-eu.researchsquare.com/files/rs-7085475/v1/0f220d6ae7f6850594f83eb5.png"},{"id":88506155,"identity":"4166d1fc-db10-4188-a2e8-aeb5b88b95a5","added_by":"auto","created_at":"2025-08-07 07:31:57","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":3622411,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-7085475/v1/8d792874-8f87-461c-ae76-8648a92cbc2c.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"\u003cp\u003ePrimary Esophageal Extranodal Marginal Zone Lymphoma: A Rare Tumor\u003c/p\u003e","fulltext":[{"header":"Introduction","content":"\u003cp\u003ePrimary esophageal extranodal marginal zone lymphoma (ENMZL) is a rare malignancy with diagnostic challenges. Treatment typically involves a combination of chemotherapy and radiotherapy, with surgery as an alternative option depending on individual circumstances. Raising awareness of this uncommon tumor among medical professionals is essential for enhancing patient outcomes. While most esophageal cancers are squamous cell carcinoma or adenocarcinoma, lymphoma can occur anywhere in the gastrointestinal system, either as a primary or secondary event. Although rare, primary esophageal ENMZL presents significant diagnostic difficulties. This case report includes relevant imaging studies, esophagoscopy results, and surgical findings.\u0026nbsp;\u003c/p\u003e"},{"header":"Case Report","content":"\u003cp\u003eThe case describes a 66-year-old woman experiencing severe swallowing difficulties that significantly affected her daily life. She had no history of smoking or alcohol consumption, and her physical examination showed no notable findings. Her medical history included polyarthritis managed with etanercept for a decade. While routine blood tests and chest X-rays were unremarkable, thoracic computed tomography revealed two masses in the esophagus—one in the middle (27×21×48 mm) and another in the distal esophagus (22×22×30 mm)—without lymph node enlargement. A positron emission tomography scan reflected significant mass metabolic activity, suggesting malignancy. Endoscopy showed submucosal lesions protruding into the esophageal cavity, though biopsies failed to provide a definitive diagnosis, necessitating surgical intervention. During surgery, a right mini-thoracotomy was performed to excise the upper esophageal mass, with a complication of mucosal perforation that was successfully repaired. Post-surgery, the patient recovered well, and histopathological analysis confirmed ENMZL. The patient was subsequently referred to a hematology clinic for chemotherapy planning. There is no clinical trial number for a case report.\u003c/p\u003e\n\n\n"},{"header":"Discussion","content":"\u003cp\u003eThe gastrointestinal system is a common site for extranodal non-Hodgkin’s lymphoma, accounting for 10% to 20% of all cases [1]. In clinical practice, gastrointestinal involvement is observed in approximately 4% of non-Hodgkin’s lymphoma cases [1,2]. Primary esophageal lymphoma is rare, with only 15 cases identified in a study of 785 articles published between 1963 and 2020 [3]. Dysphagia was the most frequently reported symptom in these cases, similar to the symptoms presented by our patient. Diagnosis criteria for primary esophageal lymphoma include the absence of superficial lymphadenopathy, definitive mediastinal lymph node enlargement, liver or spleen involvement, and predominant esophageal engagement. Our patient fulfilled all these criteria [4]. While the underlying causes of primary esophageal lymphoma are not clearly understood, it is believed to arise from the submucosal lymphoid tissue of the esophagus [5]. Endoscopic findings in previous cases have varied, including ulcers and submucosal masses. In this case, multiple submucosal masses were observed. A surgical procedure was required due to the inability to obtain a satisfactory diagnosis via endoscopy. Postoperative analysis confirmed B-cell type non-Hodgkin lymphoma. ENMZL usually affects the gastrointestinal tract, particularly the stomach, and is uncommon in the esophagus. Tumor cells resemble marginal zone B cells. Differentiating ENMZL from reactive follicular hyperplasia or other small B-cell lymphomas is crucial, with immunohistochemistry playing a key role in the diagnosis [6]. ENMZL cells express specific B-cell markers, requiring a specialized antibody panel for differentiation. Prognosis is generally favorable but depends on factors, including age, comorbidities, disease stage, and treatment response. Due to the rarity of esophageal ENMZL, individualized treatment plans are essential.\u0026nbsp;Although data are limited, survival rates range from 26% to 73%. [7].\u0026nbsp;Standard treatment typically involves chemotherapy and radiotherapy, with surgery considered in some cases. However, radiotherapy presents challenges due to the proximity of vital organs [8]. Chemotherapy regimens are utilized for advanced cases or when surgical resection is not feasible. A collaborative approach involving various medical specialists assists in choosing the best treatment for each patient. The prognosis for ENMZL is generally favorable, reflecting its slow progression; however, an advanced-stage disease with local invasion or metastasis is associated with poorer outcomes.\u003c/p\u003e\u003cp\u003eThe complete removal of a tumor depends on its size and location. In this case, surgical intervention was limited due to multiple masses, leading to a referral for chemotherapy. No signs of disease recurrence were noted after one month; nonetheless, the patient opted to continue her chemotherapy in her home country. Effective management of this rare cancer requires close collaboration among specialists. The nonspecific early symptoms emphasize the need for increased awareness among healthcare providers when encountering unexplained esophageal symptoms, especially in older adults or those with chronic inflammatory conditions. The lack of focused research on esophageal ENMZL highlights the necessity for greater attention in this understudied area of oncology.\u003c/p\u003e"},{"header":"Declarations","content":"\u003cp\u003eAuthor contributions: Yekta Altemur Karamustafaoglu and Nuray Can contributed to the submitted manuscript's conception, drafting, and editing. Fazli Yanik and Yener Yoruk contributed to the literature review. All authors reviewed and approved the final version of the manuscript. Yekta Altemur Karamustafaoglu is the article guarantor.\u003c/p\u003e\n\u003cp\u003e\u0026nbsp;I declare that I have not sent my article to another journal for simultaneous review.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eFunding\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe author(s) received no financial support for this article's research, authorship, and/or publication.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eData Availability\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe data and material related to this case report are available.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eMaterials Availability\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe data and material related to this case report are available.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eClinic Trial Number\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eNot applicable\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eEthics declarations\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConflict of interest\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThe authors declare that they have no competing interests.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eEthical approval\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThis study was exempted from ethical approval in our institution.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConsent to participate\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eThis study was exempted from ethical approval in our institution.\u003c/p\u003e\n\u003cp\u003e\u003cstrong\u003eConsent for publication\u003c/strong\u003e\u003c/p\u003e\n\u003cp\u003eWritten informed consent to publication form was obtained from the patient.\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\n\u003cli\u003eHerrmann R, Panahon A, Barcos MP, Walsh D, Stutzman L. Gastrointestinal involvement in non-Hodgkin\u0026rsquo;s lymphoma. Cancer 1980;46:215-22.\u003c/li\u003e\n\u003cli\u003eSoon MS, Yen HH, Soon A, Lin OS Primary esophageal B-cell lymphoma: evaluation by EUS. Gastrointest Endosc.2005;61(7):901-3.\u003c/li\u003e\n\u003cli\u003eQu J, Zhuang Y, Zheng D, Huang F, Zhang S. Primary Esophageal Lymphoma: Clinical Experience in Diagnosis and Treatment. Cureus. 2021;1;13(9):e17628. doi: 10.7759/cureus.17628. eCollection 2021 Sep.\u003c/li\u003e\n\u003cli\u003eDawson IM, Cornes JS, Morson BC. Primary malignant lymphoid tumors of the intestinal tract. Report of 37 cases with a study of factors influencing prognosis. Br J Surg 1961;49:80-9.\u003c/li\u003e\n\u003cli\u003eAgha FP, Schnitzer B. Esophageal involvement in lymphoma. Am J Gastroenterol 1985;80:412-6.\u003c/li\u003e\n\u003cli\u003eTabibian JH, Kalani A, Moran AM, Panganamamula K. Extranodal Marginal Zone B Cell (MALT) Lymphoma of the Esophagus. J Gastrointest Cancer. 2019;50(4):1034-36.\u003c/li\u003e\n\u003cli\u003eInayat F, Munir A, Wahab A, Younus F, Zafar F, Ullah W. Primary Esophageal Diffuse Large B-Cell Lymphoma: A Comparative Review of 15 Cases. Journal of Investigative Medicine High Impact Case Reports Volume 2018; 6: 1\u0026ndash;6.\u003c/li\u003e\n\u003cli\u003eFreret ME, Tringale KR, Boe L, Imber BS, Joffe E, Yahalom J, Hajj C. \u0026quot;Very low-dose radiotherapy for extranodal marginal zone lymphoma of bronchus-associated lymphoid tissue. Leukemia and Lymphoma. 2023;64(13):2195-2201.\u003c/li\u003e\n\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":true,"hideJournal":false,"highlight":"","institution":"","isAcceptedByJournal":true,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"digestive-diseases-and-sciences","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"ddsj","sideBox":"Learn more about [Digestive Diseases and Sciences](http://link.springer.com/journal/10620)","snPcode":"10620","submissionUrl":"https://submission.nature.com/new-submission/10620/3","title":"Digestive Diseases and Sciences","twitterHandle":"","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"stoa","reportingPortfolio":"Springer Hybrid","inReviewEnabled":true,"inReviewRevisionsEnabled":false},"keywords":"Esophagus, mass, lymphoma","lastPublishedDoi":"10.21203/rs.3.rs-7085475/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-7085475/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"Primary esophageal extranodal marginal zone lymphoma (ENMZL) is a rare and challenging malignancy to diagnose. A case involving a 66-year-old woman with a 6-month history of worsening dysphagia, odynophagia, and weight loss is presented. Imaging studies revealed a large mediastinal mass and a distal esophageal mass causing partial obstruction, with no evidence of enlarged lymph nodes. Despite biopsies from esophagoscopy and endoscopic ultrasound not providing a definitive diagnosis, the patient underwent surgical excision of the middle esophageal mass. Histopathological analysis confirmed primary ENMZL. The patient was subsequently referred for chemotherapy as stage I ENMZL. Treatment strategies may vary, typically involving a combination of chemotherapy and radiotherapy, with surgical options considered as well. Raising awareness of this rare tumor among medical professionals is vital for improving patient outcomes.","manuscriptTitle":"Primary Esophageal Extranodal Marginal Zone Lymphoma: A Rare Tumor","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2025-07-14 11:09:00","doi":"10.21203/rs.3.rs-7085475/v1","editorialEvents":[{"type":"communityComments","content":0},{"type":"decision","content":"Revision requested","date":"2025-07-14T01:51:30+00:00","index":"","fulltext":""},{"type":"editorAssigned","content":"","date":"2025-07-14T01:50:16+00:00","index":"","fulltext":""},{"type":"checksComplete","content":"","date":"2025-07-11T04:43:50+00:00","index":"","fulltext":""},{"type":"submitted","content":"Digestive Diseases and Sciences","date":"2025-07-09T15:36:30+00:00","index":"","fulltext":""}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"digestive-diseases-and-sciences","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":false,"externalIdentity":"ddsj","sideBox":"Learn more about [Digestive Diseases and Sciences](http://link.springer.com/journal/10620)","snPcode":"10620","submissionUrl":"https://submission.nature.com/new-submission/10620/3","title":"Digestive Diseases and Sciences","twitterHandle":"","acdcEnabled":true,"dfaEnabled":true,"editorialSystem":"stoa","reportingPortfolio":"Springer Hybrid","inReviewEnabled":true,"inReviewRevisionsEnabled":false}}],"origin":"","ownerIdentity":"bb23606a-c660-42ce-ad7a-220f1958ad27","owner":[],"postedDate":"July 14th, 2025","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"published-in-journal","subjectAreas":[],"tags":[],"updatedAt":"2025-08-07T07:18:54+00:00","versionOfRecord":{"articleIdentity":"rs-7085475","link":"https://doi.org/10.1007/s10620-025-09273-8","journal":{"identity":"digestive-diseases-and-sciences","isVorOnly":false,"title":"Digestive Diseases and Sciences"},"publishedOn":"2025-07-25 15:57:56","publishedOnDateReadable":"July 25th, 2025"},"versionCreatedAt":"2025-07-14 11:09:00","video":"","vorDoi":"10.1007/s10620-025-09273-8","vorDoiUrl":"https://doi.org/10.1007/s10620-025-09273-8","workflowStages":[]},"version":"v1","identity":"rs-7085475","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-7085475","identity":"rs-7085475","version":["v1"]},"buildId":"8U1c8b4HqxoKbykW_rLl7","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}