Non-Hodgkin's Lymphoma Presenting Research Articles

Non-Hodgkin lymphoma presenting with spinal cord compression: A population-based analysis of the NHL-BFM study group.

Spinal cord compression is a rare presentation of non-Hodgkin lymphoma (NHL) in children. We aimed to describe the prevalence, histological subtypes, clinical presentation, therapy, and outcome of those children in a population-based cohort. The chemotherapy regimen remained comparable over time. We retrospectively identified all children and adolescents with paresis as initial manifestations of the NHL between January 1990 and December 2020 from the NHL-BFM database. Characteristics, therapy, and outcome data were gathered from the database and patient files. Fifty-seven of 4779 children (1.2%) presented with initial paresis due to spinal cord compression. The median age was 10.3 years (range, 3.1-18.0 years), and 33% were female. Initial symptoms were paresis/weakness (n=50, 88%), back pain (n=33, 58%), paresthesia (n=23, 40%), and bladder dysfunction and/or constipation (n=22, 39%), persisting for a median of 14 days before diagnosis. Subtype distribution was mature B-NHL (n=41, 72%), precursor B-lymphoblastic lymphoma (LBL) (n=12, 21%), anaplastic large cell lymphoma (ALCL) (n=3, 5%), and T-LBL (n=1, 2%). Initial emergency therapy included surgery (70%) and/or chemotherapy/steroids (63%). Five-year event-free survival and overall survival (80%±5% and 82%±5%, respectively) were comparable with all other NHL patients. Neurological symptoms persisted in approximately one-third of surviving patients at the last follow-up. 1.2% of pediatric NHL patients presented with paresis from spinal cord compression mainly due to B-cell lymphomas. Neurological sequelae were observed in one-third of surviving patients.

Non‑Hodgkin's lymphomas of the lacrimal sac: Current insights and future directions (Review).

Non-Hodgkin's lymphoma (NHL) of the lacrimal sac is a rare, yet clinically significant entity within the spectrum of ocular malignancies. While primary lacrimal sac lymphoma is uncommon, it poses unique diagnostic and therapeutic challenges due to its anatomical location and potential for aggressive behavior. Despite advancements being made in the current understanding and treatment of NHL, research that specifically addresses the involvement of the lacrimal sac is currently lacking. Thus, the present review aimed to provide insight into the epidemiology, clinical presentation, diagnostic modalities, histopathological features, treatment strategies and prognosis of lacrimal sac NHL. Through a methodical analysis of previous literature, the present review highlights the diverse spectrum of NHL subtypes that affect the lacrimal sac, including diffuse large B-cell lymphoma, extranodal marginal zone lymphoma, mantle cell lymphoma and follicular lymphoma. Moreover, the present review discusses the role of advanced imaging techniques in accurate staging and treatment planning, including computed tomography (CT), magnetic resonance imaging and positron emission tomography-CT. The present review also discusses evolving treatment approaches, such as surgical intervention, chemotherapy, radiotherapy, immunotherapy, combinations of the aforementioned treatments and targeted therapy. In addition, the present review highlights the significance of multidisciplinary collaboration in attaining optimal outcomes for individuals with lacrimal sac NHL. The present review aimed to provide a basis for 'further investigations into novel treatment modalities and prognostic markers that may aid in guiding personalized management strategies, ultimately improving outcomes for patients with NHL.

Colonic non-hodgkin lymphoma presenting as splenomegaly splenocolic fistula in person living with HIV: A case report.

Primary colonic lymphoma is an infrequent malignancy among other large bowel malignancies, and the risk of the spread of tumor cells through a spleno-colic fistula is a unique finding and hence noteworthy. We report a case of a 55-year-old man living with HIV on anti-retroviral treatment for 12 years, who presented to the emergency room with complaints of generalized weakness and left-sided abdominal discomfort. Further examination and evaluation revealed massive splenomegaly with a thickened splenic flexure of the colon and spleno-colic fistula. The diagnosis of lymphoma with spread was made following laparotomy and histopathological examination of the colon and spleen.

A Case of Childhood Non-Hodgkin's Lymphoma Presenting as Isolated Inguinal Lymphadenopathy

A Case of Childhood Non-Hodgkin's Lymphoma Presenting as Isolated Inguinal Lymphadenopathy

Case report of rare gastrointestinal non-Hodgkin's lymphoma presenting as intussusception in adults

Gastrointestinal lymphomas comprise a group of distinct clinicopathological entities of B- or T- cell type, with primary gastrointestinal Hodgkin’s lymphoma being extremely uncommon. The gastrointestinal tract is the predominant site of extra-nodal non-Hodgkin's lymphoma accounting for 30-40% of all extra- nodal lymphomas.1 Most common non-Hodgkin’s lymphoma causing intussusception is diffuse large B-cell lymphoma (DLBCL). We herein report a rare case of ileo-colic Intussusception due to diffuse large B-cell lymphoma in a 42-year-old female. Computed tomography showed ileo-colic intussusception. Open hemicolectomy with ileo-colic anastomosis was done with postoperative chemotherapy.

Intussusception as clinical presentation of non-Hodgkin lymphoma in a 5-year-old patient

Intussusception as clinical presentation of non-Hodgkin lymphoma in a 5-year-old patient

Late Presentation of a Primary Spinal Epidural Non-Hodgkin Lymphoma

Primary spinal epidural lymphomas (PSELs) constitute a unique subset of epidural tumors characterized by the exclusive presence of lymphoma within the epidural space at initial presentation, with no concurrent lymphoma in other sites. We aim to present a unique of PSEL and compare it with the present literature This case report presents a 65-year-old female with delayed-onset primary spinal epidural non-Hodgkin lymphoma (NHL). The patient exhibited paraplegia and walking difficulties, following a year of lower-extremity weakness and tingling sensations. Magnetic resonance imaging revealed a mass in the L1 through L3. Surgical decompression, stabilization, and tumor resection were performed. Microscopic examination confirmed NHL. Chemotherapy with the CHOP regimen was administered postsurgery. Subsequent follow-up revealed motoric and sensory improvements. PSEL primarily affects the thoracic spine and is more common in males. Patient age and gender impact prognosis. PSEL typically exhibits two phases – prodromal and rapid neurological deterioration. Surgical intervention coupled with chemotherapy is recommended, but the optimal surgical approach varies. Combined therapy could lead to improved disease-free survival. Cases of PSEL have shown potential for local and systemic relapse. Late presentation can still yield improvements with surgical and prompt chemotherapeutic intervention. Even without a prior history of malignancy or significant disease, PSEL should still be considered in patients presenting with spinal compression symptoms. Cases of delayed presentation, like the one presented here, can still benefit from surgical and prompt chemotherapeutic intervention.

Lymphoma Masquerading as Thyroid Mass

Abstract Lymphomas are divided into Hodgkin’s and non-Hodgkin’s lymphoma (NHL). NHL arises usually from lymph nodes, but can rarely involve extranodal sites. Primary thyroid lymphomas are very rare, accounting for 2% of thyroid malignancies. Usually, primary thyroid malignancies manifest as a rapidly enlarging neck mass with pressure symptoms and cervical lymphadenopathy. Early diagnosis is crucial as it could alter the course of treatment and improve the prognosis. Here, we depict a rare case of NHL presenting as a thyroid mass; which appeared as frank pus intraoperatively.

Primary Effusion Lymphoma Prognostic Score (PEL-PS): A Validated International Prognostic Score in HIV-Associated Primary Effusion Lymphoma

Background: Primary effusion lymphoma (PEL) is a rare, B cell non-Hodgkin lymphoma (NHL) caused by Kaposi sarcoma herpesvirus (KSHV), also known as human herpesvirus 8, that is most common in people with HIV. It has a unique natural history among NHL presenting as malignant body cavity effusions, accompanying inflammatory signs and symptoms, and 80% of tumors are also EBV positive. PEL has poor survival compared with other HIV-associated lymphomas with fewer than 50% of patients alive 3 years after diagnosis. The International Prognostic Index (IPI) has not been validated in PEL due to its rarity. ECOG performance status, levels of interleukin (IL)-6, IL-10, and EBV positivity of the tumor have been shown to be prognostic in small cohorts of patients with PEL, but these factors have not been validated. In a large international cohort, we sought to develop and validate a prognostic model in HIV-associated PEL. Methods: We collected demographic and clinical data from patients with HIV-associated PEL who received first-line anthracycline-containing chemotherapy. The training set used to develop the predictive model was identified from patients treated in the HIV and AIDS Malignancy Branch at the National Cancer Institute (NCI) in the United States and Chelsea and Westminster Hospital (CWH) in the United Kingdom from 2000-2022. We performed univariate Kaplan-Meier analyses to determine prognostic factors of overall survival (OS). Factors with unadjusted p-values <0.10 were candidates for inclusion in the Cox proportional hazards model. We applied backward and stepwise selection to determine independent factors for inclusion in the final Cox model. The factors identified in the Cox model were able to form two groups of patients who were predicted to have good or poor survival. The factors included in the Cox model and the groupings formed were then applied to a validation set identified from patients with HIV-associated PEL treated with first-line anthracycline-containing chemotherapy at the Hôpital Saint-Louis in France over the same period. T cell counts and peripheral blood KSHV viral loads were not measured uniformly across institutions and could not be evaluated in the Cox model. Results: For the training cohort, we identified 59 patients with PEL treated with first-line anthracycline-containing chemotherapy at NCI and CWH. The median OS was 10.6 years and not statistically different between institutions (P=0.27). In univariate analysis, 5 factors were statistically negative prognostic factors: ECOG 3-4, hemoglobin <8 g/dL, albumin ≤2 g/dL, platelets <135 K/L, and IPI 4-5. Two factors were statistically positive prognostic factors: extracavitary only disease and EBV + tumors. In the multivariable Cox model, only 2 factors, ECOG ≥3 [P=0.007; hazard ratio (HR)=4.0 (95% CI: 1.5-11.1)] and hemoglobin <8 g/dL [P=0.006; HR=3.8 (95% CI: 1.5-9.7)] were jointly associated with lower survival probability. After forming groups based on the permutations of hemoglobin and ECOG, a scoring system was devised using patients with no negative prognostic factors (score 0: hemoglobin ≥8 g/dL and ECOG ≤2) versus patients with 1-2 negative prognostic factors (score 1: hemoglobin <8 g/dL and/or ECOG ≥3) resulting in a median OS of 10.6 years (95% CI: 10.6-not estimable) versus 0.8 years (95% CI: 0.3-2.8 years), p<0.0001, respectively. The validation cohort was very similar to the training cohort in terms of patient and disease characteristics. When we applied the multivariable model to the 58 patients from the validation cohort, ECOG and hemoglobin jointly predicted OS, validating the prognostic model [ECOG ≥3: P=0.0005; HR=15.6 (95% CI: 3.3-73.5) and hemoglobin <8 g/dL: P=0.04; HR=2.5 (95% CI: 1.05-5.8)]. Median OS in patients with score 0 was 16.9 years (95% CI:8.7-16.9) versus 0.6 years (95% CI: 0.3-1.0) in those with score 1. Conclusion: Using the largest international cohort of patients with HIV-associated PEL ever evaluated, we developed and validated the PEL-PS, which is easily implementable in any clinical setting using ECOG and hemoglobin. Low hemoglobin may be a surrogate marker for KSHV-associated inflammation and elevated IL-6 levels, and this association should be evaluated further. Patients with poor prognosis identified by the PEL-PS may benefit from novel therapies currently under investigation in PEL, such as immunomodulatory drugs, anti-CD38 monoclonal antibodies, and anti-PD-1 agents.

Case of non-hodgkin lymphoma presenting as adrenal mass

Case of non-hodgkin lymphoma presenting as adrenal mass

The Great Imitator: Aggressive Non-Hodgkin Lymphoma Presenting as a Cervical Mass

The Great Imitator: Aggressive Non-Hodgkin Lymphoma Presenting as a Cervical Mass

Primary non-Hodgking lymphoma of Coledoco, which simulates cholangiocarcinoma diagnosed by endoscopic ultrasonography: Case report and literature review

We present the second report in Latin America, and the first case in our country, Colombia, on the presentation of primary Coledoco non-Hodgkin lymphoma, which mimics cholangiocarcinoma diagnosed by endoscopic ultrasonography.

Primary Ovarian Non-Hodgkin's Lymphoma: An 18-year Retrospective Institutional Study and Review of Literature from India.

Primary lymphomas of the female reproductive tract are rare and the ovarian extranodal presentation of non-Hodgkin's lymphoma (NHL) accounts for only 0.5% of all NHLs and 1.5% of all ovarian malignancies. We retrospectively reviewed the institutional medical oncology database for newly diagnosed NHL cases between 1999 and 2017. We aimed to study the clinical characteristics, pathology, and outcome of primary ovarian non-Hodgkin's lymphoma (NHL) cases presented to our institution. Weidentified three patients (3.7% of extranodal NHLs and 0.85% of all NHL patients) with primary ovarian NHL from 350 NHL patient records. They underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy followed by six to eight cycles of (rituximab, adriamycin, cyclophosphamide, vincristine, prednisolone (R-CHOP/CHOP), and they attained complete remission. Given the heterogeneity of cancer incidence in India and the absence of state-wise cancer registries, our study argues a pressing need to develop a national representative registry for NHL for accurate incidence, mortality, and survival data. Additionally, fertility preservation is an important issue that must be discussed with women of fertile age and the parents of children.

Abstract 6750: Primary effusion lymphoma descriptors and clinicopathologic determinants of survival: Analysis of a pooled database

Abstract Background Primary effusion lymphoma (PEL) is a rare and aggressive type of B-cell non-Hodgkin lymphoma presenting primarily as lymphomatous effusions in body cavities and less often as solid extracavitary masses. It is typically associated with human herpesvirus 8 (HHV8) infection and occasionally with Epstein-Barr virus (EBV) co-infection. It is commonly seen in immunocompromised states, such as human immunodeficiency virus (HIV) infection, organ transplantation, immunosuppressive therapy, or advanced age. Due to our limited understanding of this disease, we conducted this pooled database analysis to delineate key clinicopathological characteristics, prognostic indicators, and treatment modalities that affect survival in this rare and unique lymphoma subtype. Methods To study the demographic characteristics, molecular and immunohistochemical signatures, therapeutic interventions, survival, and prognostic factors, we compiled a pooled database of 303 cases of PEL. All cases with an unequivocal expression of CD19 and CD20 expression were excluded. Kaplan-Meier survival curves were constructed. Cox proportional hazards model and Log-rank tests were used to assess the influence of demographic and clinicopathologic factors on overall survival (OS). Result A total of 304 patients with confirmed PEL were identified. The median age was 49, with a peak incidence between 42 and 56 years. There was a male preponderance with M:F of 10. The majority (71%) was associated with HIV infection, with a median CD4 count of 108. Sixty-eight percent were cavitary with the pleural, pericardial, and peritoneal cavities involved in 56%, 20%, and 30%. The median duration of symptoms before diagnosis was 1.5 months. Patients presented with constitutional symptoms, hepatosplenomegaly, lymphadenopathy, and bone marrow involvement in 31%, 10%, 20%, and 16%. The median OS of the whole group was 6.5 months. Presentation with constitutional symptoms (p=0.026), extracavitary/solid tumors (p=0.03), and normal platelet counts (p=0.04) were associated with better median OS. Cavitary disease with effusions restricted to the thoracic cavity had also better median OS (p<0.0001). PEL occurrence in the context of immunosuppression due to transplantation had poor median OS (p=0.004). While having Kaposi sarcoma tended to impact OS negatively (p=0.08), Castleman’s disease did not. Compared to no treatment, combination chemotherapy alone, chemotherapy with anti-retroviral therapy, and stem cell transplant were statistically superior with a median OS of 1.5, 8, 30, and 16 months, respectively (p<0.0001). Conclusion This study presents updated clinicopathologic data from a pooled cohort of patients with PEL. It identifies the type of clinical setting contributing to immunodeficiency, disease presentation, the primary location of cavitary disease, and treatment approach as key determinants of OS. Citation Format: Supriya Gupta, Christopher Graham, Philip A. Haddad. Primary effusion lymphoma descriptors and clinicopathologic determinants of survival: Analysis of a pooled database. [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2023; Part 1 (Regular and Invited Abstracts); 2023 Apr 14-19; Orlando, FL. Philadelphia (PA): AACR; Cancer Res 2023;83(7_Suppl):Abstract nr 6750.

Abstract 6751: Provisional prognostic score for primary effusion lymphoma

Abstract Background Primary effusion lymphoma (PEL) is a rare and aggressive type of B-cell non-Hodgkin lymphoma presenting primarily as lymphomatous effusions in body cavities and less often as solid extracavitary masses. It is typically associated with human herpesvirus 8 (HHV8) infection and occasionally with Epstein-Barr virus (EBV) co-infection. It is commonly seen in immunocompromised states, such as human immunodeficiency virus (HIV) infection, organ transplantation, immunosuppressive therapy, or advanced age. Survival remains poor, and an optimal regimen has yet to be identified. However, the disease presents a diverse spectrum of survival outcomes. We conducted this study to develop a provisional PEL prognostic score (PPS) for this rare disease. Methods We used our constructed PEL database, which contains retrospective data on 302 cases. Such data included demographics such as sex, age, and race. It also included disease presentation symptoms, duration of symptoms before diagnosis, site(s) of the disease, blood counts, coexisting comorbidities, disease immunohistochemical and molecular phenotype, types of treatment, and survival outcomes. Of the 302 cases, only 238 had complete survival and outcomes data, the sample chosen for this study. Cox proportional-hazards model and Log-rank tests were used to assess the influence of clinicopathologic factors on overall survival (OS). Due to the relatively small training cohort, we included factors that statistically impacted OS and those that numerically trended that way. Results The median OS of the cohort was 6.5 months. The following dichotomous variables were identified as impactful prognostic factors in this cohort: the absence of constitutional symptoms (5 vs. 18 months), cavitary effusion disease (6 vs. 66 months), peritoneal cavity disease (3.5 vs. 11 months), transplant context (4 vs. 7 months), male sex (6 vs. 40 months), and detectable HHV8 viremia (7 vs. 66 months). A prognostic model was devised using these variables to identify different levels of risk. Each variable was assigned a score of 1 when present, except for peritoneal cavity disease and transplant context, which were assigned 2 points as they had the highest hazard ratio of all the other variables. In this exploratory cohort, low risk was assigned a score of 0-1, intermediate risk a score of 2-3, and high risk a score of 4-8. This prognostic score system led to our cohort's most optimal risk discriminatory model, where low, intermediate, and high risk had a median OS of 66, 28, and 4 months, respectively (p<0.0001). Conclusion This PPS is a promising new tool for risk-stratifying patients with PEL. However, it still needs prospective validation. Citation Format: Philip A. Haddad, Supriya Gupta, Christopher Graham. Provisional prognostic score for primary effusion lymphoma. [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2023; Part 1 (Regular and Invited Abstracts); 2023 Apr 14-19; Orlando, FL. Philadelphia (PA): AACR; Cancer Res 2023;83(7_Suppl):Abstract nr 6751.

Delayed diagnosis of oral lymphoma: a case series.

Lymphomas are the second most common neoplasm in the head and neck. The clinical and radiographic presentation of non-Hodgkin lymphoma in the oral cavity is non-specific and can be hard to differentiate from other common infectious or inflammatory conditions. We report four cases of lymphoma of the head and neck, which presented as odontogenic infection, osteomyelitis, or cutaneous infection, and subsequently led to a delay in provision of appropriate treatment. Correlation between clinical, radiographic and histological findings is essential in reaching an accurate diagnosis. It is important for clinicians to consider malignant lesions, such as lymphoma, in the differential diagnosis of pain, swelling, tooth mobility or radiographic radiolucencies. Clinicians should maintain a high level of suspicion for malignancy when inflammatory lesions fail to respond to normal modes of treatment.

NON-HODGKIN LYMPHOMA PRESENTING AS RECURRENT PLEUROPERICARDIAL EFFUSION- A CASE REPORT

Non-Hodgkin lymphoma is a lymphoproliferative disorder usually affecting lymphoid organs. 20% patients with non-Hodgkin lymphoma may present with pleural effusion but pericardial involvement is rare. Pleural & pericardial effusion in lymphoma are usually due to direct seeding of malignant cells or by obstruction to the lymphatic drainage of pleura & pericardium. We report a case of non-Hodgkin lymphoma presenting as recurrent pleuro-pericardial effusion. A middle aged male with chief complaints of progressive dyspnea, palpitation & generalized pufness for past 2 months was found to have bilateral pleural effusion, pericardial effusion, ascites & generalized lymphadenopathy. Right supraclavicular lymph was node was evaluated using FNAC & excisional biopsy. Pleural & pericardial uids were analyzed. He was diagnosed as Non-Hodgkin lymphoma and started with Rituximab, Prednisolone, Cyclophosphamide, Vincristine & Doxorubicin. Patient improved on subsequent follow up

A An Unusual Presentation of Primary Extra Nodal Non-Hodgkin Lymphoma

Patients presented with soft tissue lesions without any β symptoms can be a case of Primary extranodal Non- non-Hodgkins lymphoma. According to the World Health Organization classification system, there are two types of Lymphoma with various sub-types of Hodgkins Lymphoma and Non-Hodgkin Lymphoma are classified to assess the disease burden Progression and metastasis Imaging modalities are helpful. Commonest site involvement in Non-Hodgkin Lymphoma in the gastrointestinal tract (44%) followed by Head and Neck and may Involve bone (8%) and Central Nervous System 5%. Diffuse large β-Cell Lymphoma is the most common subtype representing 30%-35% of all Non-Hodgkin Lymphoma Cases. Oral and Para oral sites were involved in 2.5% of the cases. A 27-year-old Male noticed a swelling in the flank which was progressively Increasing in size one year back later it was associated with pain which reduced mobility and pain was increased during walking. Due to the advancement of diagnostic techniques, it is easy to get the radiological picture and correlation with clinical presentation and pathological reports, but these facilities are not available at various centers. Usually, patients are present in very advanced stages because of a lack of awareness about the disease, and how to proceed for diagnosis and treatment resulting in poor outcomes for these patients. This presentation and clinical funding are rare in the case of Lymphoma. This case report allows us to think of another diagnosis rather than usual.

153. Contrasting oral presentations of non-Hodgkin's lymphoma

Non-Hodgkin's Lymphoma (NHL) is a broad term for malignancies of the lymphoreticular system, although extra nodal involvement does occur. There are approximately 60 different types of non-Hodgkin's lymphoma with a World Health Organisation classification system. NHL of the oral cavity is relatively rare. We present two contrasting cases of patients who initially presented with oral lesions.

ODP059 Torn Between Two Adrenal Cancers: A Case Report

Abstract The adrenals are affected by Non-Hodgkin's lymphoma (NHL) in only 1 percent of cases. We report a rare case of NHL presenting as a large irregular mass at the adrenal gland in an elderly male. A 62-year-old male with hypertension and diabetes presented with 6-month history of vague right sided abdominal pain with recent extension to the left flank accompanied by body malaise and unintentional weight loss. Abdominal ultrasound revealed a 9-cm left suprarenal mass in addition to bilateral nephrolithiases and prostate enlargement. There was also left-sided minimal pleural effusion. CT scan and MRI contrast studies both confirmed a large left adrenal mass with infiltration of the spleen, pancreatic tail, and left kidney. High-Resolution CT scan of the chest confirmed left-sided pleural effusion with subsegmental atelectasis. Hormonal tests showed normal 24-hour urinary metanephrines, 24-hour urinary free cortisol, aldosterone-renin ratio, and serum DHEAS. Intact PTH and calcium were also normal. Multidisciplinary consultations involving endocrinology, medical oncology, hematology, pulmonology, surgical oncology, urology, thoracic cardiovascular surgery, anesthesiology, radiology, and pathology were done. He underwent exploratory laparotomy with resection of left adrenal mass and en bloc left nephrectomy, splenectomy and distal pancreatectomy. Histopathologic findings and immunostaining were consistent with diffuse large B-cell lymphoma. He completed R-CHOP chemotherapy and adjuvant radiotherapy with good clinical response and he has been on remission for two years. Presentation: No date and time listed