Primary Esophageal Lymphoma Research Articles

S3350 Successful Endoscopic Mucosal Resection of Primary Esophageal MALT Lymphoma and Subsequent Surveillance

S3350 Successful Endoscopic Mucosal Resection of Primary Esophageal MALT Lymphoma and Subsequent Surveillance

500. PRIMARY ESOPHAGEAL LYMPHOMA: UNDERSTANDING PROGNOSTIC FACTORS AND OUTCOMES

Abstract Background Primary Esophageal Lymphoma (PEL) represents a rare subtype of gastrointestinal lymphomas, posing unique diagnostic and therapeutic challenges. Given its rarity, comprehensive insights into its prognostic factors and outcomes remain limited. This study examines the epidemiology, treatment modalities, and outcomes of PEL, aiming to identify significant prognostic factors that influence patient survival. Methods A systematic review of the literature was conducted across PubMed/Medline and Scopus databases, focusing on cases of adult PEL. Data extraction included demographic information, treatment modality, and patient outcomes. Survival rates were calculated, and prognostic factors were analyzed through unifactorial analysis. Results Out of 56 studies, 69 cases of PEL were analyzed, with a male-to-female ratio of 2.8:1 and mean age of 55.5 years. Patients were predominantly treated with chemotherapy (52.1%) while 20% underwent surgical treatment. The mean survival reported was 49.3 months with one-, three-, and five-year survival rates of 84.5%, 72.3%, and 28.9%, respectively, exhibiting a less favorable prognosis compared to common lymphoma types. Survival analysis revealed that local lymphadenopathy had a significant impact on prognosis (p=0.008), while gender, age, treatment approach, and type of lymphoma were not significant determinants of survival. Immunocompromised status showed a marginally significant effect on survival (p= 0.053). Discussion The study highlights the less favorable prognosis of PEL compared to more common lymphomas, emphasizing the importance of recognizing patients with unfavorable prognostic factors such as local lymphadenopathy and immunocompromised status. The findings advocate for the establishment of an international patient registry to better understand and manage PEL, aiming to standardize diagnostic criteria and treatment strategies.

Primary Esophageal Lymphoma: Clinical Experience in Diagnosis and Treatment

Primary Esophageal Lymphoma: Clinical Experience in Diagnosis and Treatment

Primary Esophageal Mucosa-associated Lymphoid Tissue Lymphoma: A Rare Case Report and Review of Other Published Data

The gastrointestinal tract is the most common extranodal site for lymphomas, and mucosa-associated lymphoid tissue lymphoma is the second most common histological lymphoma subtype. However, primary esophageal mucosa-associated lymphoid tissue lymphomas are extremely rare. Few such cases are documented, and the reports demonstrate inconsistent diagnostic and therapeutic strategies. Herein, a 54-year-old man was referred to our hospital for treatment of dysphagia. Esophagogastroduodenoscopy revealed a large, horseshoe-shaped subepithelial mass in the upper esophagus. Endoscopic ultrasonography and computed tomography revealed that the mass was well-demarcated and confined to the muscularis mucosa, with no abnormalities in other organs or lymph nodes. The mass was presumptively diagnosed as benign, and the patient underwent endoscopic mucosal dissection for pathological confirmation and symptom relief. Pathological examination of the dissection specimen revealed that it was a primary esophageal mucosa-associated lymphoid tissue lymphoma. As the patient had an elevated immunoglobulin G level and <i>Helicobacter pylori</i> infection, we administered adjuvant eradication therapy. The patient remains under surveillance and is free of lymphoma recurrence 36 months postoperatively. This case report demonstrates that endoscopic resection and <i>H. pylori</i> eradication are effective treatment strategies for early-stage esophageal mucosa-associated lymphoid tissue lymphoma.

Primary Esophageal Lymphoma: A Histopathological Experience from Two Tertiary Hospitals, Western Saudi Arabia.

Primary esophageal lymphoma (PEL) is a rare disorder. The objective of this study was to document the clinicopathological features of PEL at two tertiary hospitals in the western region of the Kingdom of Saudi Arabia. All PELs diagnosed between May 2002 and June 2022 were retrieved. Histopathological and immunohistochemical slides were reviewed. Additional immunohistochemistry stains were performed in selected cases. Follow-up data were collected. There were only eight cases of PEL in the records of the two hospitals. The age of the patients ranged between 50 and 74 years (median 62 years and mean 62.5 years). There were six males (80%) and two females (20%). None of the patients were immunocompromised or had human immunodeficiency virus (HIV) infection. The clinical manifestation included dysphagia and loss of weight. Six cases were diffuse large B-cell lymphoma (DLBCL), and two were low-grade mucosa-associated lymphoid tissue lymphoma. PEL is an extremely rare disease with male predominance. DLBCL is the most common pathological type in our community. There was no relation to immune status or HIV infection in this series. Clinical presentations were typically dysphagia with weight loss. Further reporting of PEL cases might help explain this disease and improve its diagnosis and management.

Primary esophageal diffuse large B-cell lymphoma.

A 76-year-old man presented with dysphagia, epigastric pain and weight loss for the last two months. Heavy sweating was also presented. Past medical conditions included type 2 diabetes. He had no evidence of any immunosupressive disease including HIV infection. Physical examination only revealed low-grade fever. Laboratory data showed leukocytosis. Gastroscopy evidenced a complete esophageal stenosis starting at 30 cm, with a severely friable mucosa of malignant appearance. The results of biopsies were insufficient for diagnosis of malignancy. Computed tomography demonstrated a 10-cm irregular tumor located in the distal and middle thirds of the esophagus, which resulted in narrowing of the lumen. Involving tracheal carina, bronchus, and descending aorta were observed. Perforation signs were also seen. Distant metastases were not found. Empirical treatment with piperacillin/tazobactan was started. A surgical gastrostomy to allows nutritional support was performed. Two other gastroscopies were performed resulting in an inconclusive diagnosis. Finally, flow cytometry performed in samples obtained by endobronchial ultrasound-guided biopsy evidenced prominent clonal B-cell populations consistent with extranodal diffuse large B-cell lymphoma exhibing CD10 expression. A treatment with Rituximab in combination with cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) was started. Primary esophageal diffuse large B-cell lymphoma (DLBCL), a variant of non-Hodgkin's lymphoma, accounts for less than 1% of all cases of gastrointestinal lymphomas.

S2051 Primary Esophageal Lymphoma or Lymphoma Presenting as an Esophageal Mass: Rare Disease or Rare Presentation of a Disease?

Introduction: GI tract is the most common site of extra nodal non Hodgkin lymphoma (NHL) with the usual sites being stomach, small intestine and colon. Esophageal involvement has been reported in < 1% of cases and mostly occurs as an extension of mediastinal or gastric lymphoma. Here, we present a case of diffuse large B cell lymphoma (DLBCL) presenting with obstructive esophageal symptoms and diagnosed on esophageal ulcer biopsy. Case Description/Methods: A 72-year-old White male with history of 84 pack years smoking, GERD, presented with a month of shortness of breath, dysphagia to solids, odynophagia, loss of appetite, and 20 lbs. weight loss. Labs revealed WBC count 7000/µL. CT chest without contrast showed a large mediastinal mass with hilar extension, causing esophageal obstruction and two masses within the right lobe of liver. Endoscopy showed a mid-esophageal ulcer without bleeding, and atypical lymphoid cells on biopsy. Immunohistochemistry was positive for CD20, PAX5, BCL2 and BCL6, MUM1 and negative for CD10, cyclinD1 with MIB1 90%, thus consistent with DLBCL. Biopsy of liver mass showed similar histology and immunohistochemistry. HIV testing was negative. He was started on EPOCH based chemotherapy for the DLBCL. On chart review, CT Lung screening a year ago showed several filling defects in mid esophagus, however the filling defects were not evaluated as he was lost to follow up. Discussion: Primary esophageal DLBCL is a rare NHL with only handful of cases reported in literature to date. Our patient did not satisfy Dawson’s criteria for diagnosis of primary GI lymphoma which requires absence of peripheral and mediastinal lymphadenopathy, lack of liver and spleen involvement, and normal peripheral blood count. However, the utility of Dawson’s criteria for diagnosis of primary esophageal lymphoma has been questioned. Esophagus is a mediastinal structure and has rich lymphatic network, thus, predisposing to early mediastinal lymph nodes involvement. Also, esophageal cancers have been known to spread to liver via venous channels. Our patient had several mid esophageal filling defects a year ago and now presented with a large mediastinal mass encasing esophagus, thus, supporting the diagnosis of primary esophageal lymphoma. This case highlights the importance of endoscopic evaluation of esophageal filling defects seen on imaging.Figure 1.: Low dose CT lung screening performed a year prior shows filling defects in mid esophagus (red arrow) as seen in (A) axial section and (B) sagittal section. (C) CT chest without contrast on presentation showing large mediastinal mass, at the same level as image A causing esophageal obliteration. (D) Endoscopic biopsy of esophageal ulcer showing atypical lymphoid cells.

Primary Esophageal Lymphoma: Clinical Experience in Diagnosis and Treatment.

Primary esophageal lymphoma is a rare malignant tumor, which is often misdiagnosed. To improve the diagnosis and treatment of this disease, we presented one case admitted at our institution and a literature review of primary esophageal lymphoma cases, including the clinical data, features of imaging, endoscopy and pathology, treatment, and prognosis. The symptoms were non-specific. Under endoscopy, most of the lesions were located in the middle and lower segment of the esophagus, behaving as ulcers, polyps, or submucosal masses, always accompanying with esophageal stricture. The diagnosis of primary esophageal lymphoma was highly dependent on pathological and immunohistochemical examination, hence stacked sampling was suggested to improve the positive rate of mucosal biopsy. Combination of chemotherapy and radiotherapy may be the first choice of treatment, surgical and endoscopic resections may be an alternative solution as well. The therapeutic effect and prognosis were slightly better than those of other esophageal malignant tumors.

Primary esophageal non-Hodgkin's lymphoma: demographics, clinical characteristics, histopathologic types, and survival in 179 patients from the SEER program and systematic review of the literature.

The most frequent site for the extranodal appearance of primary non-Hodgkin's lymphomas (NHL) is the gastrointestinal (G.I.) tract. However, primary esophageal lymphoma is extremely rare. The purpose of the present study was to describe and analyze the demographics, clinical characteristics, histopathologic types, and long-term survival of patients with primary esophageal NHL registered in the surveillance, epidemiology, and end results (SEER) database. Retrospective cohort study. Individuals with primary esophageal lymphoma (PEL) were identified using the international classification of disease for oncology, third edition histology codes. Patients were excluded if there was no microscopic confirmation of the neoplasm or if the diagnosis was made by autopsy or death certificate. Data on demographics, clinical characteristics, histopathology and survival were analyzed using the Kaplan-Meier method, life table, and cox proportional hazard models. 179 patients were included (68% males, median age 66years [IQR 46-79]). The overall survival at 1, 5 and 10years was 65% (95% CI 57.9-72.3%), 49% (95% CI 42.1-57.3%), and 31% (95% CI 24.5-38.6%), respectively. On univariate analyses, individuals with extranodal marginal zone lymphoma (MZL) had a significantly higher overall survival when compared to patients with diffuse large B cell lymphoma (HR 0.29; 95% CI 0.11-0.73. p = 0.008). Furthermore, patients whose cancer was diagnosed after 1997 showed an improved overall survival (HR 0.40; 95% CI 0.26-0.61. p < 0.001) when compared to those diagnosed before 1997. In this large population-based series, diagnosis after 1997 (year of rituximab approval by the FDA) and MZL subtype were associated with improved survival outcomes in patients with PEL.

Primary Esophageal Diffuse Large B-Cell Lymphoma With Barrettʼs Esophagus: A Case Report and Literature Review

Diffuse large B-cell lymphoma (DLBCL) is the most common type of NHL in United States and accounts for 40% of NHL worldwide. GI lymphomas are rare and primarily located in the stomach, small intestine, and colon with esophageal being the rarest location. We present a rare case of rapidly growing esophageal lymphoma arising in the background of long segment Barrett's esophagus. A 76-year-old Caucasian male presented to our facility for poor oral intake and weight loss of 40 pounds in four months. Abdominal CT demonstrated circumferential distal esophageal wall thickening (Figure 1). EGD demonstrated erythematous and circumferentially nodular mucosa in the distal esophagus.1739_A Figure 1. CT scan showing distal esophageal thickening.Biopsies revealed Barrett's esophagus with erosive esophagitis but was negative for malignancy. Esophageal brushings showed candida esophagus. A 3-week course of fluconazole was prescribed. Repeat EGD in 4 weeks demonstrated salmon colored mucosa without nodules. Distal esophagus had ulcerated, granular and friable mucosa covered with exudates. (Figure 2). Biopsies from this region returned positive for high grade B-Cell lymphoma with C-MYC and BCL-2 rearrangements. Immunoperoxidase studies showed neoplastic B cells positive for CD20, PAX-5, BCL-6 and CD10. C-MYC was positive in 60% cells and KI-67 positive in 80% cells. PET scan demonstrated marked uptake in the distal esophagus without any evidence of regional or distal metastatic disease. Bendamustine and rituximab was initiated for treatment of diffuse large B cell lymphoma. The patient had an uneventful hospital course and was discharge home to continue chemotherapy. He continues to do well on follow up clinic visit.1739_B Figure 2. Distal Esophagus: Ulcerated, granular and friable mucosa with exudatesGastrointestinal lymphoma accounts less than 20% of extra nodal lymphomas with primary esophageal lymphoma accounting for less than 1% of GI lymphomas. Our patient satisfies Dawson's criterion of primary GI lymphoma which includes an absence of peripheral lymphadenopathy, absence of mediastinal adenopathy, lack of liver or spleen involvement, and normal peripheral blood count. Primary esophageal lymphomas are most commonly found in the distal esophagus, may present with dysphagia, weight loss, odynophagia or symptoms of upper gastrointestinal bleeding. To our knowledge this is the first reported case of esophageal lymphoma arising in background of long segment Barrett's esophagus. We hope that our description of this patient's esophageal DLBCL contributes to the limited data available.

Isolated Primary non‑Hodgkin’s Lymphoma of the Esophagus

AbstractIsolated primary esophageal lymphoma, defined as a lymphoma developing in the esophageal wall, is a distinctly rare presentation and accounts for &lt;1% of all gastrointestinal lymphomas and &lt;0.1% of all malignant lymphomas. The clinical and biological characteristics of the disease are not well known due to its rarity, compounded with a low index of suspicion during diagnosis. We report a rare case of an immunocompetent patient diagnosed with an isolated primary esophageal non-Hodgkin's lymphoma of diffuse large B-cell type. Clinicoradiological and histopathological features, treatment, and outcome, along with a brief review of the literature, are discussed herewith.

Первинна В-клітинна лімфома стравоходу, некласифікована, з проміжними характеристиками між дифузною В-великоклітинною лімфомою і класичною лімфомою Годжкіна у пацієнта зі синдромом Дауна

Первинна В-клітинна лімфома стравоходу, некласифікована, з проміжними характеристиками між дифузною В-великоклітинною лімфомою і класичною лімфомою Годжкіна у пацієнта зі синдромом Дауна

Two Cases of Primary Esophageal Diffuse Large B Cell Lymphoma: Therapeutic Considerations and a Literature Review

Primary esophageal lymphoma is very rare, and most reported cases are histologically mucosa-associated lymphoid tissue lymphoma. Therefore, the principle treatment strategy for primary esophageal lymphoma focuses on local treatments, such as endoscopic mucosal resection or radiation therapy, but systemic chemotherapy plays the central role in the treatment of diffuse large B cell lymphoma (DLBCL). Generally, standard treatment for DLBCL is six or three cycles of R-CHOP chemotherapy followed by involved field radiation therapy according to stage. However, the optimal treatment strategy for primary esophageal DLBCL, and the role of additional radiation is not settled, due to a paucity of cases. Moreover, the clinical characteristics related to the etiology and natural course are also unknown. Here, we present two cases of primary esophageal DLBCL with a literature review. (Korean J Med 2015;88:224-230)

Primary esophageal mucosa‐associated lymphoid tissue lymphoma treated by endoscopic submucosal dissection

Primary esophageal mucosa-associated lymphoid tissue (MALT) lymphoma is rare. There have been few reports about early primary esophageal MALT lymphoma being treated endoscopically. The clinical profile of primary esophageal MALT lymphoma is currently unclear, so it is important to accumulate more information about early esophageal MALT lymphoma. To achieve early detection of esophageal MALT lymphoma, we need more accurate knowledge and information about the macroscopic and morphological features of this tumor. Endoscopic resection is one of the most effective treatments. With respect to the lateral and vertical margins of the resected specimen, endoscopic submucosal dissection (ESD) may be superior to endoscopic mucosal resection for treating early esophageal MALT lymphoma. Here we report the macroscopic appearance of the tumor which is the first successful case of ESD for early esophageal MALT lymphoma.

Primary Mucosa-associated Lymphoid Tissue Lymphoma of the Esophagus, Manifesting as a Submucosal Tumor

We report a case of primary mucosa-associated lymphoid tissue (MALT) lymphoma in the esophagus that manifested as a large submucosal tumor (SMT). Primary esophageal lymphoma is very rare, occurring in less than 1% of all patients with gastrointestinal lymphoma. Only a few cases of MALT lymphoma in the esophagus have been reported in the English literature. A 53-year-old man was referred to Dongguk University Ilsan Hospital (Goyang, Korea) in July 2012 for further evaluation and treatment of an esophageal SMT. Endoscopy showed a cylindrically elongated submucosal mass with normal overlying mucosa in the mid esophagus, 25-30 cm from the incisor teeth. He underwent surgery to confirm the diagnosis. Pathologic findings showed diffuse small atypical lymphoid cells which were stained with Bcl-2, CD20, but not with CD3, CD5, CD23, Bcl-6, or cyclin D1. These cells showed a positive monoclonal band for immunoglobulin heavy chain gene rearrangement. Based on the pathological, immunohistochemical, and molecular biological features, the esophageal mass was diagnosed as extranodal marginal zone B-cell lymphoma of the MALT type.

Extremely rare case of primary esophageal mucous associated lymphoid tissue lymphoma

SJ is a 37-year-old male who presented with one year history of dysphagia, odynophagia and 15 pounds weight loss. He underwent endoscopic evaluation which showed mid esophageal ulcers. It was thought that the cause of the ulcer was the multivitamins and the patient was asked to stop them. Furthermore Esomeprazole therapy was also initiated. Patient's symptoms persisted but he did not seek any medical attention until about one year later. Meanwhile the patient reported additional 15 pounds of weight loss. We repeated upper endoscopy again which showed evidence of two chronic non bleeding irregular friable ulcerations seen in the mid esophagus, 31 cm from the incisors. Biopsies and frozen section were taken and sent for assessment to the Pathology lab. Immunoperoxidase studies on frozen sections showed the presence of IgM and for most plasma cells IgG. The microscopic and histologic findings were consistent with mucous associated lymphoid tissue lymphoma with plasmocytic differentiation. Computed tomographic scan done showed no evidence of spread to adjacent structures. The patient was referred to oncology and several cycles of radiation and Rituximab therapy were initiated which cured the disease. Subsequent endoscopies with blind biopsies were done which were negative for any neoplastic process.

Primary Esophageal Lymphoma in an Immunocompetent Patient Presenting as Dysphagia: An Extremely Rare Case and Often Difficult Diagnosis

Primary Esophageal Lymphoma in an Immunocompetent Patient Presenting as Dysphagia: An Extremely Rare Case and Often Difficult Diagnosis

Primary Esophageal Lymphoma in an Immunocompetent Patient Presenting as Dysphagia: An Extremely Rare Case and Often Difficult Diagnosis

Primary Esophageal Lymphoma in an Immunocompetent Patient Presenting as Dysphagia: An Extremely Rare Case and Often Difficult Diagnosis

Linfoma esofágico primário

We describe the case of a 54 year old woman seen with an esophageal mass diagnosed as a primary esophageal lymphoma. The main symptom was dysphagia of seven months duration. The treatment consisted in resection of the tumor, and reconstruction of the defect with a reversed pleural flap, followed by a chemotherapy regimen that consisted of five drugs, cyclophosphamid, prednisone, doxorubicin, rituximab and vincristine (R-CHOP). The patient developed an esophageal pleural fistula treated with pleural drainage and irrigation that closed in 45 days. Two and one half years later she is doing well and disease free.

A very elderly case of complete remission by chemotherapy in a patient of primary esophageal non-Hodgkin lymphoma

A very elderly case of complete remission by chemotherapy in a patient of primary esophageal non-Hodgkin lymphoma