Primary Intestinal Lymphoma Research Articles

Effect of Clinical, Endoscopic, Radiological Findings, and Complications on Survival in Patients with Primary Gastrointestinal Lymphoma.

The purpose of this study was to evaluate the clinical, endoscopic, and radiological characteristics, complications, survival outcomes, and prognostic factors of patients with primary gastrointestinal lymphoma. This study retrospectively analyzed the demographic, laboratory, endoscopic, and radiological characteristics and treatment outcomes of 43 patients with newly diagnosed primary gastrointestinal lymphoma. The median age was 62 years (range: 26-83). The primary lesion location was the gastric in 33 (77%) patients and the intestinal in 10 (23%) patients. The most common lesions were the corpus (33%) and corpus+antrum (24%) in primary gastric lymphoma and the ileum (60%) in primary intestinal lymphoma. The most common endoscopic findings were diffuse infiltrative lesion (23%) and massforming (33%), while the most common computed tomography finding was wall thickening (53%). Wall thickening and mass-forming at computed tomography were greater in primary intestinal lymphoma than in primary gastric lymphoma (P = .034). Complications were observed in 9 (21%) patients and 13 (31%) patients who underwent surgery. Complication and surgery rates were higher in primary intestinal lymphoma than in primary gastric lymphoma (P = .003 and P = .014, respectively). Five-year overall survival and 5-year eventfree survival rates were 75% and 72%, respectively. Univariate analysis showed that intestinal involvement, advanced clinical stage, a high International Prognostic Index score, mass-forming and wall thickening at computed tomography, extranodal involvement, and complication were found to adversely affect survival. Multivariate analysis revealed that intestinal involvement and a high International Prognostic Index score were independent prognostic factors for overall survival and event-free survival. Patients with primary gastrointestinal lymphoma with intestinal involvement and high International Prognostic Index score should be followed closely.

S3463 Primary Intestinal Follicular Lymphoma

Introduction: Follicular Lymphoma (FL) mostly presents as a nodal lymphoma, however primary extranodal involvement is rare. The gastrointestinal tract is the most common site of extranodal FL, and with rates of FL increasing it remains important to recognize the characteristic endoscopic features of the disease. It typically carries an indolent course that may not even require treatment. We present a case of primary intestinal FL that involved the entire small intestine. Case Description/Methods: A 53-year-old Hispanic male with a history of gastrointestinal reflux disease (GERD), presented with refractory GERD symptoms despite maximal PPI therapy. An upper endoscopy was performed and revealed an irregular Z line, gastritis and numerous, patchy, small (< 1cm), white nodules in the second and third portion of the duodenum. Multiple biopsies were taken from the nodules. Pathology revealed Grade I-II FL. Capsule endoscopy was performed and revealed numerous similar lesions throughout the entire small intestine. He was referred to Oncology and underwent PET-CT scan which revealed multiple FDG-avid abdominopelvic lymph nodes. After discussion with Oncology, the patient elected for a watch and wait approach. Repeat PET-CT scan 6 months later revealed multiple abdominopelvic lymph nodes that are no longer FDG-avid. He remains asymptomatic and will be monitored closely off therapy. (Figure) Discussion: The most common primary intestinal lymphomas are diffuse large B-cell lymphoma (DLBCL) and mucosa associated lymphoid tissue (MALT) Lymphoma. FL only comprises approximately 1-2% of primary intestinal lymphomas. The small intestine is the most common location, with the second portion of the duodenum as the most likely site of involvement. It is rarely seen in the colon and stomach and there are no reported cases of esophageal involvement. Rates of diagnosis are on the rise, thus highlighting the importance of endoscopic recognition as it may often be seen in the second portion of the duodenum on routine upper endoscopy. Given the indolent course of FL, a watch and wait approach may be reasonable. Other options for therapy include chemotherapy, Rituximab, or radiation.Figure 1.: White nodular mass in duodenum visualized on capsule endoscopy consistent with patient's known diagnosis of Follicular Lymphoma.

Clinical Features Analysis and Survival Nomogram of Primary Small Intestinal Diffuse Large B-Cell Lymphoma

PurposeThis study aimed to analyze the clinical features and survival of primary small intestinal diffuse large B-cell lymphoma (PsI-DLBCL), and establish and independently validate a prognostic nomogram for individual risk prediction.Patients and methodsData for 24 patients from the Renmin Hospital of Wuhan University were used as an independent validation cohort, data for 1144 patients with PsI-DLBCL from the SEER database were randomly assigned to training (N=817) and internal validation (N=327) sets. The survival nomogram was constructed with the most significant factors associated with OS using Univariate and multivariate analyses on the training set. Decision curve analysis (DCA) was conducted. Internal validation was SEER validation set. Our cancer center cohort was used as an external validation set to further verify the survival nomogram.ResultsFive clinicopathological feature factors associated with OS of the training set yielded (age, marital status, Ann Arbor stage, surgery for primary site and chemotherapy), which were used to create a survival nomogram. Additionally, the calibration curves of the prognostic nomogram revealed good agreement between the predicted survival probabilities and the ground truth values. The stability of our survival nomogram was explained by internal and external validation data.ConclusionOur nomogram proposes the clinical and therapeutic factors affecting OS for patients with PsI-DLBCL. It shows that chemotherapy and surgery are beneficial to patients in the choice of treatment options. These results suggest that a survival nomogram may be better at predicting OS for PsI-DLBCL patients.

Epidemiology and Determinants of Survival for Primary Intestinal Non-Hodgkin Lymphoma: A Population-Based Study.

BackgroundGastrointestinal tract is the most common site of extranodal non-Hodgkin lymphoma (EN-NHL). Most of the published data have been on gastric NHL with limited studies on primary intestinal non-Hodgkin lymphoma (PI-NHL) considering rare incidence. We performed epidemiological and survival analysis for PI-NHL from the Surveillance, Epidemiology, and End Results (SEER) 18 database.MethodsA total of 9,143 PI-NHL cases of age ≥ 18 years were identified from the SEER 18 database for the period 2000 - 2015. Totally, 8,568 patients were included for survival analysis. Cause-specific survival (CSS) and overall survival (OS) analysis were done for PI-NHL and PI-diffuse large B-cell lymphoma (PI-DLBCL) using sex, age of onset, treatment, histology, stage, and year of diagnosis. Survival analysis was done by using Cox proportional hazard model and Kaplan-Meier plot with log-rank test.ResultsThe percentage of PI-NHL of all the intestinal cancers and EN-NHL were 1.35%, and 10.52%, respectively. The age-adjusted incidence was 0.9145/100,000 population for the study population. PI-NHL was more common among patients aged ≥ 60 years, male and non-Hispanics Whites. Majority of patients were diagnosed at stage 1 and 2 (74%), and DLBCL (44.8%) was the most common histology. Overall median survival was 111 (95% confidence interval (CI): 105 - 117) months. In OS analysis, significant increased risk of mortality was seen with T-cell NHLs vs. DLBCL (hazard ratio (HR): 2.56), patients aged ≥ 60 vs. < 60 years (HR: 2.87), stage 4 vs. stage 1 (HR: 1.93), male vs. female (HR: 1.17), with best outcome seen in patient treated with combination of chemotherapy and surgery vs. none (HR: 0.45). Similar results were seen in CSS and for PI-DLBCL as well. Significant improvement in outcomes was observed for PI-DLBCL patients receiving chemotherapy with/without surgery.ConclusionsFindings from our large, population-based study reveal PI-NHL is a rare type of intestinal malignancy with significant difference in survival based on histological and epidemiological characteristics.

Primary Intestinal Lymphoma in Rabbits

Neoplasms of the intestinal tract are uncommon in rabbits and primary lymphoma of the intestinal tract is rare. This case series is the first detailed description of primary intestinal lymphoma in rabbits. We reviewed four cases of primary intestinal lymphoma in rabbits aged 5-9.5 years old with an average age of 7.8 years. Neoplastic cells in three cases were large (8μm diameter) while one case had intermediate cells (5μm diameter). Neoplastic lymphocytes were of B-cell lineage and characterized by intense, multifocal, membranous immunoreactivity for CD79a and no immunoreactivity to CD3. Based on the Revised European-American Classification of Lymphoid Neoplasms/World Health Organization classification, three of the cases were consistent with diffuse large B-cell lymphoma and the case with intermediate-sized neoplastic cells was consistent with lymphoblastic lymphoma.

CD30 + Primary intestinal T-cell lymphoma (unclassified) masquerading as chronic inflammation: a case report

BackgroundPrimary intestinal T-cell lymphomas are uncommon malignancies that pose a diagnostic dilemma, because the clinical features and imaging findings commonly overlap with those encountered in inflammatory bowel diseases.Case presentationThe current clinical case report describes the clinical history, laboratory findings and histopathological analysis from a patient with non-specific gastrointestinal symptoms with a presumptive clinical diagnosis of inflammatory bowel disease, and two intestinal biopsy specimens with non-specific findings. Due to the persistent symptoms a third biopsy was consistent with primary intestinal T-cell lymphoma, a diagnosis that was elusive for months after the initial presentation. Clinical correlation with laboratory and histopathological findings is required to establish a definitive diagnosis and to further stratify the patients. In addition, the neoplastic cells featured partial expression of CD30, which had relevant therapeutic implications.ConclusionsSuspicion for an intestinal T-cell lymphoproliferative disorder should always exist in patients with persistent abdominal symptoms with no clear etiology. The current discussion provides a summary and review of the key diagnostic histological features for the classification of primary intestinal T-cell lymphomas. In addition, the discussion describes how specific the histological findings are relevant for the clinical management decisions.

Targeted Next-generation Sequencing Reveals a Wide Morphologic and Immunophenotypic Spectrum of Monomorphic Epitheliotropic Intestinal T-Cell Lymphoma.

Primary intestinal T-cell lymphoma (PITL) is highly aggressive and includes celiac disease-related enteropathy-associated T-cell lymphoma (EATL), monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL), and primary intestinal peripheral T-cell lymphoma, not otherwise specified (ITCL-NOS). MEITL is the most common PITL in Asia, comprising of monomorphic medium-sized cells typically expressing CD8, CD56, and cytotoxic granules. Occasional cases with intermediate features between MEITL and ITCL-NOS are difficult to be classified and warrant further investigation. We collected 54 surgically resected PITLs from Taiwan, with 80% presenting with bowel perforation. The overall outcome was poor with a median survival of 7 months. Based on histopathology (monomorphic vs. pleomorphic) and immunophenotype, we classified these cases into 4 groups: MEITL with typical immunophenotype (n=34), MEITL with atypical immunophenotype (n=5), pleomorphic PITL with MEITL-like immunophenotype (n=6), and ITCL-NOS (n=9). There was no EATL in our cohort. Targeted next-generation sequencing of the first 3 groups showed highly prevalent loss-of-function mutations for SETD2 (85%, 80%, and 83%, respectively) and frequent activating mutations for STAT5B (64%, 60%, and 50%, respectively) and JAK3 (38%, 20%, and 50%, respectively). In contrast, ITCL-NOS cases had less frequent mutations of SETD2 (56%) and STAT5B (11%) and rare JAK3 mutations (11%). Our results suggest that there is a wider morphologic and immunophenotypic spectrum of MEITL as currently defined in the 2017 WHO classification. MEITL with atypical immunophenotype and PITL with MEITL-like immunophenotype shared clinicopathologic and molecular features similar to MEITL but distinct from ITCL-NOS, indicating that such cases may be considered as immunophenotypic or histopathologic variants of MEITL.

Differential Diagnosis of Crohn's Disease and Ulcerative Primary Intestinal Lymphoma: A Scoring Model Based on a Multicenter Study.

BackgroundDifferential diagnosis of Crohn’s disease (CD) and ulcerative primary intestinal lymphoma (UPIL) is a tough problem in clinical practice.AimsOur study identified key differences between CD and UPIL patients and aimed to further establish a scoring model for differential diagnosis.MethodsA total of 91 CD and 50 UPIL patients from 9 tertiary inflammatory bowel disease centers were included. Univariate and multivariate analyses were used to determine significant markers for differentiating CD and UPIL. A differential scoring model was established by logistic regression analysis.ResultsThe differential model was based on clinical symptoms, endoscopic and imaging features that were assigned different scores: intestinal bleeding (−2 points), extraintestinal manifestation (2 points), segmental lesions (1 point), cobblestone sign (2 points), homogeneous enhancement (−1 point), mild enhancement (−1 point), engorged vasa recta (1 point). A total score of ≥1 point indicates CD, otherwise UPIL was indicated. This model produced an accuracy of 83.66% and an area under the ROC curve of 0.947. The area under the ROC curve for validation using the 10-fold validation method was 0.901.ConclusionThis study provided a convenient and useful model to differentiate CD from UPIL.

Successful management of primary intestinal diffuse large B-cell lymphoma complicated with chylothorax

Introduction: Primary intestinal non-Hodgkin’s lymphoma is a part of extranodal non-Hodgkin’s lymphoma which is rare in incidence, moreover when complicated with chylothorax. Case Report: A 26-year-old female patient was referred to our hospit

Clinical Features and Prognosis of Patients with Primary Intestinal B-cell Lymphoma Treated with Chemotherapy with or without Surgery.

This multicenter study reviewed the clinical features and prognosis according to the primary site of involvement and the treatment modality in patients with B-cell primary intestinal lymphoma (PIL). Among 125 consecutive patients diagnosed with PIL, 100 patients were analyzed. The median age was 59 years, and the male to female ratio was 1.86:1. Diffuse large B-cell lymphoma (66/100, 66.0%) was the most common histological subtype. The estimated 5-year survival rate (5-YSR) was 48.5%. The 5-YSR was similar regardless of the type of primary treatment (chemotherapy alone vs. surgery/chemotherapy, 50.7 vs. 45.3%, p=0.582). A comparison of the survival according to the primary site of involvement revealed a 5-YSR of 32.5% (p=0.027), 64.3% (reference), 46.5% (p=0.113), and 49.8% (p=0.024) for the small intestine, ileocecal region, large intestine, and multiple sites, respectively. Multivariate analysis, however, revealed a low hemoglobin level, advanced Ann Arbor stage, and aggressive histological type to be independent prognostic factors for shorter survival but not ileocecal region involvement. The Ann Arbor stage, hemoglobin level, and histological type were independent prognostic factors for survival, while the primary site of involvement and treatment modality did not affect the prognosis in patients with B-cell PIL.

Primary Small Intestinal Lymphoma: A Rare Case Report of Large B Cell Lymphoma Presentation as Intestinal Obstruction

Primary Gastrointestinal Lymphoma (PGIL) is the commonest Extranodal Lymphoma (ENL), which accounts for 30%-40%. It is important to understand them and keep them in mind in differential diagnosis in daily clinical practice. Men are more frequently affected than are women. The clinical presentation of gastrointestinal lymphoma is frequently associated with pain, masses, perforations and obstructions. We are reporting the case of a young woman with intestinal obstruction diagnosed as Diffuse Large B Cell Lymphoma (DLBCL).

Development and External Validation of a Nomogram to Predict Cancer-Specific Survival in Patients with Primary Intestinal Non-Hodgkin Lymphomas.

PurposePrimary intestinal non-Hodgkin lymphoma (PINHL) is a biologically and clinically heterogeneous disease. Few individual prediction models are available to establish prognoses for PINHL patients. Herein, a novel nomogram was developed and verified to predict long-term cancer-specific survival (CSS) rates in PINHL patients, and a convenient online risk calculator was created using the nomogram.Materials and MethodsData on PINHL patients from January 1, 2004, to December 31, 2015, obtained from the Surveillance, Epidemiology, and End Results (SEER) database (n = 2372; training cohort), were analyzed by Cox regression to identify independent prognostic parameters for CSS. The nomogram was internally and externally validated in a SEER cohort (n = 1014) and a First Affiliated Hospital of Guangzhou University of Chinese Medicine (FAHGUCM) cohort (n = 37), respectively. Area under the receiver operating characteristic curve (AUC), calibration curves, and decision curve analysis (DCA) were used to evaluate nomogram performance.ResultsFive independent predictors were identified, namely, age, marital status, Ann Arbor Stage, B symptoms, and histologic type. The nomogram showed good performance in discrimination and calibration, with C-indices of 0.772 (95% CI: 0.754–0.790), 0.763 (95% CI: 0.734–0.792), and 0.851 (95% CI: 0.755–0.947) in the training, internal validation, and external validation cohorts, respectively. The calibration curve indicated that the nomogram was accurate, and DCA showed that the nomogram had a high clinical application value. AUC values indicated that the prediction accuracy of the nomogram was higher than that of Ann Arbor Stage (training cohort: 0.804 vs 0.630; internal validation cohort: 0.800 vs 0.637; external validation cohort: 0.811 vs 0.598), and Kaplan–Meier curves indicated the same.ConclusionA nomogram was developed to assist clinicians in predicting the survival of PINHL patients and in making optimal treatment decisions. An online calculator based on the nomogram was made available at https://cuifenzhang.shinyapps.io/DynNomapp/.

The impact of surgery on long-term survival of patients with primary intestinal non-Hodgkin lymphomas based on SEER database

Evidence regarding the need for surgery for primary intestinal non-Hodgkin lymphoma (PINHL) patients with chemotherapy is limited and controversial. We aimed to investigate the specific impact of surgery on survival of PINHL patients. Data from PINHL patients (aged > 18 years) with chemotherapy between 1983 and 2015 were extracted from the Surveillance, Epidemiology, and End Results (SEER) database. We concerned about overall survival (OS) and improved cancer-specific survival (CSS). Propensity score matching (PSM) analysis was also used to explore the reliability of the results to further control for confounding factors. Finally, we screened 3537 patients. Multivariate regression analysis showed that patients with surgery and chemotherapy had better OS (hazard ratio [HR] 0.83; 95% confidence interval [CI] 0.75–0.93; p = 0.0009) and CSS (HR 0.87; 95% CI 0.77–0.99; p = 0.0404) compared with the non-operation group after adjusting for confounding factors. After PSM analysis, compared with non-surgery, surgery remained associated with improved OS (HR 0.77; 95% CI 0.68–0.87; p < 0.0001) and improved CSS (HR 0.82; 95% CI 0.72–0.95; p = 0.008) adjusted for baseline differences. In the large cohort of PINHL patients with chemotherapy older than 18 years, surgery was associated with significantly improved OS and CSS before and after PSM analysis.

CD70 Expression in Mature T-Cell Lymphomas

IntroductionMature T-cell lymphomas (TCLs) represent about 15% of the non-Hodgkin lymphomas in U.S. and are a heterogeneous group of neoplasms. TCLs usually have poor outcomes and are resistant to conventional chemotherapy regimens. Therefore, there is a need for identifying biomarkers that could translate into effective novel treatments. CD70 is the TNF superfamily ligand of CD27 and it is being explored as a potential target in CAR-T therapy against TCLs and other neoplasms. Although CD70 expression has been documented in TCL cell lines, thus providing a potential rationale for its use as a therapeutic target, the expression of CD70 among the most frequent TCL subtypes in patient samples has not been previously evaluated.MethodsPatients with de novo and/or relapsed mature TCLs diagnosed between 01/2010 and 06/2020 and with available tissue sections were included in the study. The assessment of CD70 expression was performed by immunohistochemistry (IHC) using a novel proprietary antibody developed by CRISPR Therapeutics. CD70 expression was scored using % of expression, intensity (negative, +1, +2, and +3) and H-score {H-score = [(%positive cells intensity 1+) x 1] + [(%positive cells intensity 2+) x 2] + [(%positive cells intensity 3+) x 3]} in neoplastic cells (Figure 1). Clinicopathologic characteristics were collected retrospectively and included age, sex, staging, biopsy site, WHO pathologic classification, immunophenotype, presence of other malignancies, treatment status, disease progression/relapsed, and follow-up. These characteristics were compared with CD70 expression using ANOVA or non-parametric analysis. Overall-survival (OS) was estimated using Kaplan-Meier method and compared using log-rank. A p< 0.05 was considered statistically significant.ResultsOne hundred thirty-six patient samples representing the major subtypes of the 4 categories of mature TCLs were included [nodal TCLs (n=64; including PTCL Th1, and Th2 subtypes and angioimmunoblastic T cell lymphoma - AITL), extranodal (n=35; including primary intestinal lymphomas), cutaneous (n=24; including mycosis fungoides (MF) with large cell transformation), and leukemic (n=13; including adult T cell leukemia/lymphoma)]. Most patients were male (58.8%), in the 6 th or 7 th decade (47.1%), with advanced stage IV (55.7%), no previous malignancies (69.9%) and previously treated (57.2%); 59 of which (88%) received two or more lines of treatment. The median expression of CD70 was 40% (ranged from 0 to 100%) and the median H-score was 110 (ranged from 0 to 300) and was significantly higher (p= 0.006) in peripheral T-cell lymphoma, NOS, primary cutaneous TCLs (non-mycosis fungoides), and AITL (180, 150, and 150, respectively) (Figure 1). The expression of CD70 was more frequent in nodal and extranodal subtypes (including skin), compared to leukemic TCLs (p= 0.005). The expression of CD70 was associated with advanced age at the diagnosis (p= 0.003), CD2 expression (p= 0.01), CD3 expression (p= 0.001), CD16 negativity (p= 0.03), and absence of ALK-1 expression (p= 0.005). After a median follow-up of 19 months (range: 1 - 300 months), 42% of the patients died of disease. The median OS was 76 months (CI95, 38.5 - 113.4 months) and it was not associated with CD70 expression.ConclusionsCD70 was highly expressed in most mature TCLs and it was negative in most ALK-positive ALCL. Its expression was associated with nodal and extranodal subtypes, advanced age, expression of CD2, CD3, and negativity for CD16. Therefore, CD70 can be used as a potential biomarker and a target in clinical trials of patients with mature TCLs. [Display omitted] DisclosuresIyer: CRISPRX: Research Funding; Seattle Genetics: Research Funding; Rhizen: Research Funding; Merck: Research Funding; Legend: Research Funding; Innate: Research Funding; Spectrum: Research Funding; Trillium: Research Funding; Astra Zeneca: Research Funding; Yingli: Research Funding; Cyclacel: Research Funding. Sagert: CRISPR Therapeutics: Current Employment. Pham: CRISPR Therapeutics: Current Employment. Tipton: CRISPR Therapeutics: Current Employment. Vega: i3Health, Elsevier, America Registry of Pathology, Congressionally Directed Medical Research Program, and the Society of Hematology Oncology: Research Funding; CRISPR Therapeutics and Geron: Research Funding.

Surgery plus chemotherapy versus chemotherapy alone in primary intestinal lymphoma: a meta-analysis.

ObjectivePrimary intestinal lymphomas (PILs) are uncommon tumors, but their incidence is increasing. Currently, their management is centered around systemic treatments, such as chemotherapy and radiotherapy, whereas surgery is restricted to selected indications. This meta-analysis aimed to evaluate the role of surgery in PIL treatment.MethodsWe collected publications comparing surgery plus chemotherapy versus chemotherapy alone in patients with PIL from 2000 to 2021. All trials analyzed the summary odds ratios (ORs) of endpoints, including the 5-year overall survival (OS), 3-year OS, and 3-year progression-free survival rates. Combined pooled ORs were analyzed using fixed- or random-effects models according to heterogeneity.ResultsSix studies were included. Compared with chemotherapy alone, surgery plus chemotherapy was associated with significantly higher 5-year OS [OR = 4.88, 95%confidence interval (CI) = 1.91–12.44, Z = 3.32], 3-year OS (OR = 3.83, 95%CI = 2.33–6.30, Z = 5.30), and 3-year progression-free survival (OR = 3.51, 95%CI = 2.20–5.58, Z = 5.29).ConclusionsSurgery plus chemotherapy was associated with better outcomes than chemotherapy alone, especially in the early stages. Therefore, surgery plus chemotherapy may be the preferred strategy for appropriately selected patients with PIL.The protocol for this systematic review was registered at INPLASY (INPLASY202180102) and is available in full (https: //doi.org/10.37766/inplasy2021.8.0102).

Surgery and Chemotherapy versus Chemotherapy Only in Older Persons with Primary Intestinal Diffuse Large B-Cell Lymphoma.

BackgroundThe management of primary intestinal diffuse large B cell lymphoma (PI-DLBCL) in elderly patients (aged >60 years) remains controversial. We conducted a retrospective study to assess the efficacy of different treatment strategies and prognostic factors for elderly Chinese patients with PI-DLBCL.Patients and MethodsForty-six untreated elderly patients with PI-DLBCL were included in this retrospective study. Twenty-four patients were treated with surgery (prior to chemotherapy) plus chemotherapy (SCT). The other 22 patients did not undergo surgery before chemotherapy (CT).ResultsPatients treated with SCT had a higher overall response rate of 91.7% than patients receiving CT, but the difference between groups was not significant (P=0.581). Regarding survival, SCT resulted in a greater 3-year overall survival (OS) rate (87.3% vs 56.9%, P=0.130) and significantly higher 3-year event-free survival (EFS) rate (74.1% vs 27.3%, P=0.002) than CT. The univariate analysis showed that male sex, advanced Lugano stage, poor performance status and chemotherapy alone were associated with a shorter EFS. Only the male sex was correlated with a shorter OS. The multivariate analysis showed that sex (P=0.040) and treatment strategy (P=0.022) were independent prognostic factors for EFS.ConclusionSurgery plus chemotherapy produced a better outcome for EFS, but not OS, than chemotherapy alone in elderly Chinese patients with PI-DLBCL.

Machine Learning Based on Morphological Features Enables Classification of Primary Intestinal T-Cell Lymphomas.

Simple SummaryWe presented a machine learning approach for accurate quantification of nuclear morphometrics and differential diagnosis of primary intestinal T-cell lymphomas. The human interpretable machine learning approach can be easily applied to other lymphomas and potentially even broader disease categories. This approach not only brings deeper insights into lymphoma phenotypes, but also paves the way for future discoveries concerning their relationship with disease classification and outcome.The aim of this study was to investigate the feasibility of using machine learning techniques based on morphological features in classifying two subtypes of primary intestinal T-cell lymphomas (PITLs) defined according to the WHO criteria: monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) versus intestinal T-cell lymphoma, not otherwise specified (ITCL-NOS), which is considered a major challenge for pathological diagnosis. A total of 40 histopathological whole-slide images (WSIs) from 40 surgically resected PITL cases were used as the dataset for model training and testing. A deep neural network was trained to detect and segment the nuclei of lymphocytes. Quantitative nuclear morphometrics were further computed from these predicted contours. A decision-tree-based machine learning algorithm, XGBoost, was then trained to classify PITL cases into two disease subtypes using these nuclear morphometric features. The deep neural network achieved an average precision of 0.881 in the cell segmentation work. In terms of classifying MEITL versus ITCL-NOS, the XGBoost model achieved an area under receiver operating characteristic curve (AUC) of 0.966. Our research demonstrated an accurate, human-interpretable approach to using machine learning algorithms for reducing the high dimensionality of image features and classifying T cell lymphomas that present challenges in morphologic diagnosis. The quantitative nuclear morphometric features may lead to further discoveries concerning the relationship between cellular phenotype and disease status.

Primary Intestinal Lymphoma: Clinicopathological Characteristics of 55 Patients.

IntroductionGastrointestinal (GI) tract is the most common site of extranodal lymphoma accounting for 30–40% of the cases. In Western countries, stomach is the most common site of GI lymphoma, whereas in the Middle East and Mediterranean countries, small intestine is commonly involved. Studies about primary intestinal lymphoma (PIL) are heterogeneous in anatomical distribution, presentation, and histological subtypes. The present study was aimed at studying the anatomical distribution, histological subtypes, and clinical characteristics at tertiary care centers.Materials and methodsThe present study was retrospective, conducted between 2006 and 2020. Patient's data were collected from institutional medical records. PIL was diagnosed by Lewin's criteria. After histological diagnosis, PIL was classified as per the World Health Organization (WHO) criteria and staging was done according to the Ann Arbor classification as modified by Musshoff.ResultsA total of 941 lymphoma cases were diagnosed during the study period between 2006 and 2020 consisting of 238 Hodgkin's lymphoma and 703 non-Hodgkin's lymphoma (NHL) cases. PIL constituted 5.8% of all lymphoma cases (55 out of 941) and 50.9% (55 of 108) of all primary GI lymphoma. Median age at diagnosis was 44 years and comprised predominantly males (85.45%). Diffuse large B-cell lymphoma (DLBCL) and mucosa-associated lymphoid tissue (MALT) lymphoma were the most common histological subtype (78%) seen. Two patients with primary Hodgkin's lymphoma involving the intestine were seen. T-cell lymphoma was seen in three (5.4%) patients. Ileocecal region was the most common site involved (27%). The common presenting complaints were intestinal obstruction (40%) requiring surgical resection and abdominal pain (32%). Majority of the patients presented in the early stages (I and II).ConclusionOur study demonstrates the pattern of distribution and various histological subtypes of PIL including the rare variants like primary intestinal Hodgkin's lymphoma. Relatively more number of patients presented with intestinal obstruction requiring surgery in comparison with other studies.How to cite this articleMalipatel R, Patil M, Rout P, et al. Primary Intestinal Lymphoma: Clinicopathological Characteristics of 55 Patients. Euroasian J Hepato-Gastroenterol 2021;11(2):71–75.

Development and validation of prognostic scoring in primary intestinal diffuse large B-cell lymphoma: a single-institution study of 184 patients.

BackgroundThe incidence of primary intestinal diffuse large B-cell lymphoma (PI-DLBCL) is much lower than primary gastric DLBCL, and large-scale analyses on the clinical characteristics, molecular features, therapeutic strategies, and risk stratification have been seldomly performed in PI-DLBCL.MethodsTo assess prognostic model development, 107 PI-DLBCL patients diagnosed before 2014 were studied for prognosis factors including different primary involved sites and treatment strategies. For internal validation, a non-random split sample set with 77 PI-DLBCL patients after 2014 was included for validation of the prognosis factors.ResultsPatients with an ileocecal lesion presented with better survival time than those with non-ileocecal sites, with surgical resection significantly influencing the prognosis. Non-ileocecal patients who underwent surgery with lymphadenectomy had superior overall survival (OS) and progression-free survival (PFS) compared to those receiving surgery without lymphadenectomy or those not receiving (without) surgery. For ileocecal patients, surgery with or without lymphadenectomy resulted in better OS and PFS than those without surgery. For biomarker analysis, only BCL-2 >50% or Ki67 >80% on tumor cells indicated poor clinical outcome. In multivariate analysis, age, Eastern Cooperative Oncology Group (ECOG) score, and site of origin were independent prognostic factors for inferior OS in PI-DLBCL. A prognosis model was set up based on age, ECOG score, and site of origin, and validated well.ConclusionsThe prognosis in patients with PI-DLBCL with ileocecal involvement showed was better than those with non-ileocecal involvement. Surgical strategy can impact the clinical outcome of PI-DLBCL patients.

Clinical diagnosis and treatment of primary small intestinal lymphoma

Objective: To report experiences in the diagnosis and treatment of primary lymphoma of the small intestine (PSIL). Method: The clinical data of 15 patients with PSIL treated from January 2015 to July 2019 at Guangzhou First People’s Hospital were investigated retrospectively. Among the 15 patients, 9 were male, and 6 were female, with ages ranging from 18 to 73 years, with a median age of 51.6 years. Data relating to gender, age, clinical manifestation, laboratory examination, imaging, diagnosis, and treatment of the patients were reviewed. Results: The most common clinical manifestations were abdominal pain, abdominal lump, bowel obstruction, gastrointestinal hemorrhage, and athrepsy. Serum tumor markers were checked and found to be normal. In all 15 cases, tumors were found by spiral computed tomography (CT), and 12 cases were diagnosed as PSIL. Eleven cases were given barium meal examinations, and positive results were found in 4 cases, with only 1 case considered to be PSIL. All 15 patients underwent surgery. All patients were diagnosed as having nonHodgkin lymphoma by postoperative pathology (8 patients with diffuse large B-cell lymphoma, 5 with mucosa associated lymphoid tissue type B-cell lymphoma and 2 with enteropathy-type intestinal T-cell lymphoma). There were no cases of perioperative deaths. Ten patients received adjuvant chemotherapy with the CHOP (cyclophosphamide, epirubicin, vincristine, and prednisone) regimen after the operation. Fourteen cases were followed up for a mean duration of 30 months (range of 6-52 months). The 1- and 3-year survival rates were 85.7% and 57.1%, respectively. Conclusions: PSIL has no specific clinical manifestations. The diagnostic rate with barium study is low, whereas spiral CT is a promising diagnostic method for PSIL. Surgery combined with chemotherapy is important for the treatment of PSIL