Primary non-Hodgkin Lymphoma Research Articles

Two Cases of The Follicular Lymphoma of The Breast: An Exceedingly Rare Presentation

Abstract Introduction/Objective The primary Non-Hodgkin lymphoma of the breast is a very rare entity, which comprises only less than 0.5% of all breast malignancies. The follicular lymphoma of the breast is still rarer as the most common type is diffuse large B cell lymphoma (DLBCL). We are reporting two cases of follicular lymphoma of the breast. Methods/Case Report Case 1: A 67-year-old female with a history of low-grade follicular lymphoma of the lacrimal gland and cervical lymph node was presented with 1.7 cm irregular soft tissue mass in the upper inner quadrant of left breast identified on CT scan. Microscopic examination showed diffuse infiltration of medium-sized lymphocytes with focal follicular architecture. These lymphocytes were positive for CD20, CD10, BCL2, BCL6, and Ki67 (10%); while negative for CD5, CD3, cyclin D1, and EBER. These findings are consistent with low-grade follicular lymphoma. Subsequently, a biopsy of the right breast mass revealed similar findings. The patient was followed up with a PET scan every six months and doing well. Case 2: A 52-year-old female with a history of low-grade follicular lymphoma of the right hard/soft palate junction was presented with bilateral breast masses identified on mammography. The biopsy was performed and microscopic examination showed vaguely follicular architecture with small to medium-sized lymphocyte infiltration. The flow cytometric analysis revealed CD10 positive B cell population with kappa light chain restriction. Fluorescence in situ hybridization (FISH) analysis showed 96% of cells with BCL2 and BCL6 rearrangement. These findings were consistent with low-grade follicular lymphoma. The patient was started on chemotherapy with good outcomes. Results (if a Case Study enter NA) N/A Conclusion By reviewing two cases, we emphasize the rarity of follicular lymphoma in the breast. The most important differential diagnosis is extranodal marginal zone lymphoma and DLBCL. In absence of flow cytometric analysis FISH analysis for BCL-2 rearrangement or other molecular analysis is key to diagnose follicular lymphoma.

Anaplastic T-Cell Non-Hodgkin’s Lymphoma: Mucocutaneous Presentation

A 41-year-old male presented with a nodular, vegetating and ulcerated lesion of the upper lip, with two weeks of evolution and rapid growth. Biopsy revealed Primary Cutaneous Anaplastic T-Cell Non-Hodgkin's Lymphoma (pc-ALCL). ALCL represents mature T-cell neoplasms that express the lymphocyte activation marker CD30. This can be grouped based on the expression of the ALK marker (positive or negative) and by clinical presentation (systemic or localized). Localized forms of ALCL include primary cutaneous (pc) ALCL and breast implant-associated ALCL (BIA-ALCL). c-ALCL makes up 8% of cutaneous T-cell lymphoma; it is seen mostly in male adults. Clinically, pc-ALCL presents with nodular cutaneous lesions; the majority of the patients present with solitary or localised nodules or papules. Patients with pc-ALCL have an excellent prognosis, with a 5-year disease-specific survival over 90%. [...]

Primary gastric non-Hodgkin lymphomas: Recent advances regarding disease pathogenesis and treatment.

Primary gastric lymphomas (PGLs) are distinct lymphoproliferative neoplasms described as heterogeneous entities clinically and molecularly. Their main histological types are diffuse large B-cell lymphoma (DLBCL) or mucosa-associated lymphoma tissue. PGL has been one of the main fields of clinical research of our group in recent years. Although gastric DLBCLs are frequent, sufficient data to guide optimal care are scarce. Until today, a multidisciplinary approach has been applied, including chemotherapy, surgery, radiotherapy or a combination of these treatments. In this minireview article, we provide an overview of the clinical manifestations, diagnosis and staging of these diseases, along with their molecular pathogenesis and the most important related clinical published series. We then discuss the scientific gaps, perils and pitfalls that exist regarding the aforementioned studies, in parallel with the unmet need for future research and comment on the proper methodology for such retrospective studies. Aiming to fill this gap, we retrospectively evaluated the trends in clinical presentation, management and outcome among 165 patients with DLBCL PGL who were seen in our institutions in 1980-2014. The study cohort was divided into two subgroups, comparing the main 2 therapeutic options [cyclophosphamide doxorubicin vincristine prednisone (CHOP) vs rituximab-CHOP (R-CHOP)]. A better outcome with immunochemotherapy (R-CHOP) was observed. In the next 2 mo, we will present the update of our study with the same basic conclusion.

ABCL-193: Insights into Diagnosis and Treatment of Nasopharyngeal Non-Hodgkin Lymphomas

Context Non-Hodgkin lymphomas (NHL) are heterogeneous lymphoproliferative tumors with different disease burdens (DB) and responses to treatment. Objective Identification of the diagnosis patterns and assessment of the short-term results of treatment in primary nasopharyngeal NHL. Design This analytical and cohort study included 66 patients with nasopharyngeal NHL who were managed at the Institute of Oncology from Moldova between 2014–2020. The diagnosis was proved by cytological, histopathological, and immunohistochemical examinations. Setting The patients’ follow-up and study were performed at the comprehensive cancer center. Patients The patients age ranged between 19–86 years (average age: 58.4±2,14 years). Stage I was diagnosed in 10 (15.2%) cases, stage II in 36 (54.5%), stage III in 8 (12.1), and stage IV in 12 (18.2). Main Outcomes Measures The ECOG-WHO score and complete response (CR) rate should estimate the short-term results of combined chemotherapy (CChT) and CChT followed by locoregional radiotherapy (LRT). Interventions The eligible patients underwent mostly CVChlP, R-CVChlP, CHOP, and R-CHOP regimens combined with LRT on bulky or residual tumors. Results The aggressive NHL were diagnosed mostly (76.1%) at stage I and II due to the earlier developed DB. The ECOG-WHO score accounted for 1–3. CR was achieved in 10 (100%) stage I cases after CChT and RT. CR occurred in 21 (67.7%) and partial response (PR) in 7 (22.6%) in stage II NHL after CChT and RT. In stage II NHL treated with CChT also, CR was achieved in 1 (25%) and PR in 2 (50%) cases. In stage III treated with CChT and RT, CR was registered in 1 (20%) and PR in 2 (40%) cases. Only PR occurred in 2 (66.7%) stage III cases and 1 (33.3%) stage IV case after CChT alone. In stage IV NHL, CR was obtained in 1 (11.1%) case and PR in 5 (55.6%) after CChT and RT. The ECOG-WHO score reached 0–1 as a result of CChT and RT in cases with CR and PR. Conclusions Aggressive NHLs were diagnosed mostly in localized stages due to the earlier developed DB. Regardless of stage, the highest response rates were achieved after R-CHOP regimen combined with LRT.

A Life-Threatening Presentation of Primary Small Cell Colorectal Non-Hodgkin Lymphoma as Cardiac Tamponade and Its Challenging Management.

Colorectal small cell NHL is a quite rare malignancy that may present initially with pericardial metastasis.Cardiac tamponade secondary to colorectal NHL is a life-threatening presentation. It can be managed by timely chemotherapy alone whenever the usual drainage procedures are not safe.A multidisciplinary approach is a cornerstone in the management of unstable lymphoma patients. It helps the rapid diagnosis and initiation of appropriate chemotherapy.

Tumor characteristics and survival in patients with extranodal non-Hodgkin lymphoma of the female reproductive tract

<h3>Objectives:</h3> Primary extranodal non-Hodgkin lymphoma (NHL) of the female reproductive tract is rare, comprising approximately 1% of all NHL. Studies to date have been limited to case reports without longitudinal follow-up. The objective of this study is to describe tumor characteristics and survival in patients with primary vulvar, vaginal, cervical, uterine and ovarian NHL using the Surveillance, Epidemiology and End Results (SEER) cancer database. <h3>Methods:</h3> Adults with a histologic diagnosis of non-Hodgkin lymphoma (ICD-O-3 9590-9823) and a primary site coded as vulva (ICD-10-CM C51.0-51.9), vagina (C52.9), cervix (C53.0-53.9), uterus (C54.0-C55.9), or ovary (C56.9-C57.4) diagnosed between 1973 and 2015 were included in the analysis. Data was abstracted on variables of interest including age at diagnosis, year of diagnosis, demographics, Ann Arbor stage, histologic subtype, treatment regimens and disease-specific survival (DSS). Descriptive statistics were used to compare characteristics between primary sites. Kaplan-Meier survival curves and multivariable Cox proportional hazards survival analysis were performed using SPSS. <h3>Results:</h3> A total of 793 patients with primary gynecologic NHL were identified, including vulva (n=60), vagina (n=101), cervix (n=187), uterus (n=142) and ovary (n=303). The median age at diagnosis was 55 years old and ranged from 18 to 94 years. Vulvar, vaginal and cervical primary cancers were more likely to be diagnosed at an early stage compared to uterus and ovary (p<0.001) and receive radiation (p<0.001). Ovarian primary NHL was most likely to be diagnosed at an advanced stage (65% stage III or IV) and 88% of patients were treated with surgery. There was no significant difference in chemotherapy administration between the primary sites. The median DSS was not reached. The 5-year disease-specific survival rate was 83.2% for cervical, 81.8% for vaginal, 72.3% for ovarian, 70.9% for vulvar and 69.1% for uterine primary sites (p=0.019). On multivariate analysis, compared to primary ovarian NHL, DSS was significantly improved if the primary site was cervix (hazard ratio 0.61, 95% CI 0.39-0.97) or vagina (HR 0.52, 95% CI 0.29-0.92) with no difference in survival with primary vulvar NHL (HR 0.76, 95% CI 0.43-1.35) or primary uterine NHL (HR 0.90, 95% CI 0.59-1.38). Age at diagnosis (HR 1.04, 95% CI 1.03-1.05), histologic subtype (Burkitt's lymphoma vs other NHL, HR 3.26, 95% CI 1.82-5.84), and advanced stage (Stage II vs I: HR 2.16, 95% CI 1.42-3.28; III vs I: 1.34, 0.60-2.98; IV vs I: 3.50, 2.43-5.06) were associated with worse survival and more recent diagnosis (HR 0.98, 95% CI 0.96-1.00) and treatment with surgery (HR 0.52, 95% CI 0.36-0.73)) were associated with improved survival. <h3>Conclusions:</h3> Overall, extranodal NHL of the female reproductive tract has an excellent prognosis. The clinical characteristics, treatment regimens and survival differ between primary sites.

A Rare Case of Bilateral Primary B-Cell Non-Hodgkin Lymphoma of Breast

Primary B-Cell Non-Hodgkin Lymphoma of Breast is a rare presentation of breast malignancy and it is necessary to differentiate it from secondary breast lymphoma as prognosis and treatment varies based on the diagnosis. We report a case of 47-year-old female with bilateral PBNHL of Breast which is rare due to younger age of presentation than earlier reported cases of this malignancy, lump with “peau de orange” appearance seen in both the breasts which is usually seen with high grade lymphoma and complete recovery of the patient while bilateral PBL outlines poor prognosis. Mammosonography findings included dilated ducts with hypoechoiec areas. MRI showed altered signal intensity. Findings of the imaging studies are non-specific and vary widely from case to case which is why biopsy which is gold standard was done to confirm the diagnosis

Primary non-Hodgkin's lymphoma of the temporal bone

We present a 6-year-old child, hospitalized in MSPI Institute of Mother and Child, Emilian Coţaga Clinic, Pediatric Otorhinolaryngology Department, Chisinau, Republic of Moldova, with primary non-Hodgkin’s lymphoma (NHL) of the temporal bone, with paresis of facial nerve.

Primary advanced non-Hodgkin’s lymphoma of bladder with bilateral hydroureteronephrosis: A case report

Primary lymphoma of bladder is a very rare entity with a 0.2% incidence of all bladder malignancies. Secondary bladder involvement in malignant lymphoma is more common (10-25%) with preponderance in middle aged females. A non-Hodgkin lymphoma of the bladder can be managed conservatively without surgery and has a better prognosis. Because the presenting symptoms and radiological imaging cannot conclusively rule out a primary bladder lymphoma from other malignant causes, cystoscopy and biopsy with immune-histochemical staining is essential. We present to you a 72-year-old male with complaints of irritative lower urinary tract symptoms and asymptomatic microhematuria. On evaluation computerized tomography (CT) was s/o a large bladder wall mass with bilateral mild hydroureteronephrosis and enlarged pelvic lymphnodes. Cystoscopy and biopsy was done and the report came out to be a non-Hodgkins lymphoma. Whole body positron emission tomography (PET) scan was done which confirmed it to be a primary bladder NHL. Patient was treated with chemotherapy and is now doing well on follow up. We would like to share our experience in managing this case.

CD19-Chimeric Antigen Receptor (CAR) Engineered Natural Killer (NK) Cell Therapy: Novel “Off the Shelf” Immunotherapy in CD20 Resistant B-Cell Non-Hodgkin Lymphoma (NHL) Cell Lines, Primary NHL Cells, and a Human Lymphoma Xenograft Model

Background: NK-92 is a continuously growing cell line consisting of “pure” (100%) activated NK cells. These cells were subsequently bioengineered to express human anti-CD19 CAR recognizing CD19 B cells (ie, CD19-CAR NK). The goal of this study was to establish the mechanistic rationale for NK-based therapy in B cell NHL (bNHL) and to determine the potency of this targeted “off the shelf” therapy in a host of in vitro bNHL cell lines (including anti-CD20 antibody resistant cells), primary NHL patient derived cells, and an in vivo NHL xenograft model.Methods: bNHL cell lines SUDHL10, SUDHL4, SUDHL2 (diffuse large B cell lymphoma, DLBCL), HF1 (follicular) and Raji (Burkitt) were used. Rituximab- (R) and obinutuzumab- (O) resistant bNHL cell lines (SUDHL2, SUDHL4, SUDHL10) were also established. Patient derived cell lines (EL-5 and KSC) were obtained from primary DLBCL patients via the Tufts Tissue Biorepository. CD19-CAR NK cellular therapy was supplied by NantKwest. In vitro NK cell mediated cytotoxicity was determined using G6PD release (aCella-tox assay), apoptosis by Annexin V & flow cytometry; 10,000 target bNHL cells were co-cultured with effector NK cells at clinically relevant effector to target ratios (E:T 1:1-10:1) for 4 hours. Further, the dynamic efficacy of CD19-CAR NK was determined utilzing droplet based microfluidics & live cell imaging. Global transcriptome analysis was used to establish the biological characteristics of R- & O- sensitive and -resistant bNHL cells & for expression of NK regulatory ligands. Key pathways and drivers were determined using established systems biology approaches (Beheshti A. Cancer Informatics 2015; Dashnamoorthy R. Cancer Res. 2016). Additional functional in vitro ELISA based assays (IFNγ & TNFα) were done. Finally, in vivo studies in SCID mice with a SUDHL10 xenograft model was studied with subcutaneous injections of CD19-CAR NK therapy.Results: We identified that untreated bNHL cell lines expressed high levels of NK activation ligands (CD70, CD86, ITGAL, MICB, etc) and expression of inhibitory ligands were minimal. Profiling analyses also showed that anti-CD20 resistant bNHL cell lines had unique biological properties suggestive of defective IFNγ & TNFα signaling components, while expression of NK regulatory ligands remained mostly intact in both sensitive & resistant bNHL cells. In microfluidics based analyses, we observed >90% death in SUDHL10 and SUDHL4 as well as in the corresponding anti-CD20 resistant cell lines. Global transcriptome analysis revealed that activation of IFNγ signaling was the key transcriptional response to CD19-CAR NK activity, confirmed by increased IFNγ secretion. Gene expression profiling showed activation of inflammatory responses via upregulated immunoregulatory interactions, IFNγ signaling and induction of apoptosis as conserved mechanisms in both parental and anti-CD20 resistant bNHL cells treated with CD19-CAR NK therapy (Fig 1A-B). Cytotoxicity studies showed significantly increased cytolytic activity at E:T ratios (1:1-10:1) via LDH release in all bNHL cell lines (Raji, SUDHL4 and SUDHL10) at 4 hours vs L428 (Hodgkin) or Jurkat (T cell lymphoma) cells (Fig 1C). Increased apoptosis 40-70% (E:T ratios 0.25:1-2:1) was also noted in Raji cells with CD19 CAR NK vs 20% cell death with E:T ratios (0.25:1-2:1) in parental NK-92. Furthermore, CD19-CAR NK co-culture E:T ratio (1:1) resulted in >75% death in O-resistant SUDHL4 (SUDHL4-OR) and 66% death in R-resistant HF1 (HF1-RR) cells demonstrating that anti-CD20 resistance did not appear to impact the effectiveness of cell kill with CD19-CAR NK therapy. Finally, results from studies in SCID mice using a SUDHL10 xenograft tumor model showed significant reduction in tumor volume with CD19-CAR NK therapy vs control cells (P <0.001) (Fig 1D).Conclusion: Altogether, we identified that most bNHL cell lines expressed high levels of NK activation ligands and that their biological properties are suggestive of IFNγ and TNFα signaling signaling suggesting a conserved mechanistic response to CD19-CAR NK cell therapy. Furthermore, CD19-CAR NK cell therapy induced prominent single-agent cytolytic activity against a spectrum of bNHL cells, including primary DLBCL cells, cells resistant to standard anti-CD20 antibody, and also a DLBCL in vivo xenograft model with cell death occurring via immunoregulatory pathways with prominent IFNγ signaling & induction of apoptosis. [Display omitted] DisclosuresBoissel:NantKwest, Inc.: Employment. Evens:Pharmacyclics: Consultancy; Merck: Consultancy; Affimed: Consultancy; Amgen: Consultancy; Seattle Genetics: Consultancy; Celgene: Consultancy; AbbVie: Consultancy; • Spectrum Pharmaceuticals: Consultancy; Kite Pharma: Consultancy; Millennium: Consultancy; Novartis: Consultancy.

Lympho- and Myeloproliferative Malignancies Occurring in the Same Host: Description of a Nationwide Discovery Cohort

Background: So far, myeloid and lymphoid malignancies have been considered as diseases with distinct pathogenetic mechanisms. However, recent studies (Frederiksen H et al, Blood 2011) have reported an increased risk of lymphoid malignancies in patients with chronic myeloproliferative neoplasms (MPN). Moreover, genomic studies in patients with angioimmunoblastic T-cell lymphoma (AITL) have revealed mutations in epigenetic modifier genes, such as IDH-2, TET2, and DNMT3A4, which are usually not seen in lymphomas, while they are commonly found in patients with myeloid diseases.Aim: The aim of our work was to identify and validate a nationwide cohort of patients, who were diagnosed with MPN as well as a lymphoid malignancy either simultaneously or sequentially. The establishment of this cohort is currently serving as a discovery platform for possible shared pathogenetic pathways in myeloid and lymphoid disease entities.Methods: Patients diagnosed with both MPN and a lymphoid malignancy within the period 1990-2015 were identified through the National Danish Pathology Registry. In order to minimize the occurrence of therapy related secondary myelodysplasias, only patients diagnosed either with both diseases simultaneously (i.e. within maximum half a year from each other) or with MPN first and later with a lymphoid malignancy were selected for further histopathological revision. Formalin-fixed paraffin-embedded tissue specimens from 16 different pathology departments throughout Denmark were collected. All specimens were reviewed by an experienced hematopathologist (TLP) at a tertiary referral center.Results: The algorithm leading to the establishment of the study cohort is shown in Figure 1. Ninety-seven patients fulfilled the described inclusion criteria. Demographically, 48.5% of the patients were females. The age at diagnosis ranged between 18.5-93.7 years and the median age at MPN diagnosis was 70.8 years (interquartile range 62.8-79.9 years). The average time between the diagnosis of MPN and that of lymphoid malignancy was 1.5 years and in the majority of the patients (90%) both diagnoses were established within 5 years from each other. The myeloid and lymphoid diagnoses for each individual patient are shown in Table 1. If one looks at mature, non-primary leukemic non-Hodgkin lymphomas, i.e. excluding lymphoblastic lymphoma, chronic lymphocytic leukemia, Waldenström macroglobulinemia, and Hodgkin lymphoma, an excess of peripheral T-cell lymphomas (PTCL) was observed (17%), at a frequency almost double as high as the one expected in a Caucasian population-based non-Hodgkin lymphoma cohort (8-10%). Interestingly, most PTCL diagnoses were of AITL type.Conclusion: The establishment of this unique series of tissue specimens from patients with co-existing myelo- and lymphoid malignancies revealed, among primary systemic non-Hodgkin lymphomas, an excess of PTCL in general and AITL in particular. Genomic studies are ongoing to search for shared pathogenetic steps explaining the co-occurrence of MPN and lymphoid malignancies in the same host. [Display omitted] Disclosuresd'Amore:Nordic Nanovector: Other: Advisory Board; Les Laboratoires Servier: Honoraria, Other: Advisory Board; Takeda Pharma: Honoraria, Other: Advisory Board, Research Funding; Sanofi: Research Funding.

Ovarian dlbcl presenting as a very large ovarian mass in a young woman – a case report (and potential frozen section pitfall)

While ovarian involvement as a late manifestation of disseminated non-Hodgkin lymphoma (NHL) is not uncommon, primary ovarian NHL, or NHL presenting for the first time as an ovarian mass, is rare, having a reported incidence of around 0.5% of all untreated NHL and 1.5% of untreated ovarian neoplasms. This case study reports a 27-year-old woman who underwent a unilateral salpingo-ophorectomy and staging for a very large, rapidly enlarging, right ovarian mass, suspected clinically of being a dysgerminoma. On frozen section, the tumour was favoured to be a germ cell tumour, with juvenile granulosa cell tumour also not excluded. On permanent section, however, the tumour more clearly demonstrated morphological features and immunological features in keeping with a large B-cell non-Hodgkin lymphoma. Coupled with FISH analysis showing no evidence of a MYC gene rearrangement, a diagnosis of DLBCL, NOS, double expressor phenotype, was ultimately made. This case serves as a reminder that, although rare as an initial presentation in the ovary, lymphoma should be considered by pathologists in the differential diagnosis of ovarian tumours, including at frozen section, where consideration should also be given to obtaining fresh tissue for flow cytometry. 1.Dimopoulos MA, Daliani D, Pugh W, et al. Primary ovarian non-Hodgkin's lymphoma: outcome after treatment with combination chemotherapy. Gynecol Oncol 1997; 64: 446–50.

Imaging features of a rare case of mandibular lymphoma

The primary osseous non-Hodgkin lymphoma infrequently involves the mandible. Only few cases of non-Hodgkin’s lymphomas (NHL) of mandible have been reported. Malignant lymphoma is classified into 2 types: non-Hodgkin lymphoma and Hodgkin lymphoma (HL). 20%-30% of NHLs are seen in extranodal sites. Waldeyer’s ring is the most common site of NHL in head and neck, and other sites include orbit, paranasal sinus, oral cavity, salivary gland and thyroid. However, NHL involvement of the mandible is very rarely reported and there is no detailed evaluation of its imaging features. The present case was of a 27 year old woman with exclusive mandibular involvement. Early detection of the lesion and differentiating it from other neoplastic or inflammatory lesions is important, as higher the clinical stage & histopathologic grade, poorer is the prognosis. In our case an ill-defined radiolucent lesion in mandible with associated pain is important feature of a malignant neoplasm including follicular lymphoma despite its rare presentation. Keywords: Lymphoma, Mandibular lymphoma, Non-Hodgkin’s lymphoma.

Multiple Intraventricular Non-Hodgkin Lymphoma – A Rare Case Report

Lymphoma of the central nervous system can present with a wide variety of clinical symptoms and imaging characteristics which can be challenging for radiologist as well as neurosurgeons to deal with. Primary CNS lymphoma (PCNSL) accounts for 1% of all primary brain tumors which is a rare variant of extranodal non-Hodgkin’s lymphoma. We report a case primary central nervous system Non-Hodgkins lymphoma presenting a multiple intraventricular and periventricular masses in a 14 years young man with severe headache, convulsion and unsteady gait. The diagnosis was obtained by image guided surgical biopsy. This is an unusual presentation of primary central nervous system lymphoma involving lateral ventricle and 3rd ventricle while the majority of the case presenting as solitary masses.&#x0D; Bang. J Neurosurgery 2021; 10(2): 206-209

Primary Extranodal Follicular T-Cell Lymphoma and Ductal Breast Carcinoma Diagnosed by a Magnetic Resonance Imaging-Guided Vacuum-Assisted Biopsy: A Case Report

Patient: Female, 62-year-oldFinal Diagnosis: Primary non-Hodgkin lymphoma and ductal carcinoma in-situSymptoms: No specific symptomsMedication:—Clinical Procedure: —Specialty: RadiologyObjective:Rare co-existance of disease or pathologyBackground:Extranodal non-Hodgkin’s lymphomas (NHLs) are most commonly low-grade B-cell lymphomas, and the breast is not an usual site for this condition. This case report describes a 62-year-old woman with a primary NHL and ductal carcinoma in situ (DCIS) of the left breast diagnosed by a magnetic resonance imaging (MRI)-guided vacuum-assisted breast biopsy. The simultaneous diagnosis of breast cancer and NHL is rare, with few cases described in the literature. Primary breast lymphomas account only for 0.04% to 0.5% of breast malignancies.Case Report:In November 2016, a 62-year-old woman was treated for a peripheral T-cell lymphoma (follicular helper T-cell phenotype) of the left upper central breast; later she underwent periodic breast imaging follow-ups. In October 2018, MRI revealed a focal 33-mm non-mass contrast enhancement (according to the Breast Imaging Reporting and Data System –MRI lexicon of the American College of Radiology) in the lower external quadrant of the left breast. Neither mammography nor ultrasonography demonstrated any suspicious features. The multidisciplinary medical team performed an MRI-guided vacuum-assisted breast biopsy and the histological analysis confirmed the diagnosis of a DCIS. Subsequently, she underwent surgery resulting in eradication of the disease and has had regular follow-ups, including mammography, ultrasonography, and MRI.Conclusions:This is a rare case of both a primary NHL of the breast and DCIS, which was detected only by MRI. It highlights the role of an MRI-guided vacuum-assisted breast biopsy, which allows an accurate and economic diagnosis in case of suspicious findings on MRI. We recommend the use of MRI in follow-ups for patients with previous breast lymphomas (high-risk patients).

Association of the Affordable Care Act with survival among adolescents and young adults with lymphomas in California.

6532 Background: Our recent study showed that the implementation of the Affordable Care Act (ACA) was associated with increased health insurance coverage among adolescents and young adults (AYAs, 15–39 years) diagnosed with lymphomas in California and decreased likelihood of late stage at diagnosis. However, AYAs of Black or Hispanic race/ethnicity (vs Whites) and those living in lower socioeconomic (SES) neighborhoods were at higher risk of presenting with advanced stage. We aimed to determine whether the increased insurance coverage under the ACA was associated with improved survival, and to identify the main predictors of survival among AYAs with lymphomas. Methods: We used data from the California Cancer Registry linked to Medicaid enrollment files on AYAs diagnosed with a primary non-Hodgkin (NHL) or Hodgkin (HL) lymphoma during March 2005–September 2010 (pre-ACA), October 2010–December 2013 (early ACA) or 2014–2017 (full ACA). Patients were followed from lymphoma diagnosis until death, loss to follow-up or end of the study (12/31/2018). Health insurance was categorized as continuous Medicaid, discontinuous Medicaid, Medicaid enrollment at diagnosis/uninsured, other public or private. We used multivariable Cox proportional regression to examine the associations between all-cause survival and era of diagnosis, adjusting for sex, age and stage at diagnosis, health insurance, race/ethnicity, neighborhood SES, treatment facility, comorbidities, and marital status. Results: Of 11,221 AYAs, 5,878 were diagnosed with NHL and 5,343 with HL. Most patients were male (56%), White (45%), presented with earlier stage (I/II, 56%), and had private insurance (57%). The proportion of AYAs who received initial care at National Cancer Institute-Designated Cancer Centers (NCI-CCs) increased from 24% pre-ACA to 31% after full ACA implementation (p &lt; 0.001). AYAs diagnosed in the early (aHR = 0.76, 95% CI 0.67–0.88) and full ACA (aHR = 0.55, 95%CI 0.47–0.64) eras had better survival than those diagnosed pre-ACA. Compared to those with private insurance, survival was worse among patients with no insurance (HR = 2.13, 95% CI 1.83–2.49), discontinuous Medicaid (HR = 2.17, 95% CI 1.83–2.56) and continuous Medicaid (HR = 1.93, 95% CI 1.63–2.29) at diagnosis. Regardless of their insurance, older AYAs, males, unmarried, those with later stage (II–IV), residents in lower SES neighborhoods, and those of Black, Hispanic, Asian/Pacific Islander, and American Indian/Alaskan Native race/ethnicity experienced worse survival. Conclusions: Following the ACA implementation in California, AYAs diagnosed with lymphomas experienced increased access to care at NCI-CCs and improved survival. Yet, racial/ethnic and socioeconomic survival disparities persisted. Moving forward, policy actions are required to mitigate structural and social determinants of health disparities in this population.

Primary T-cell non-Hodgkin's lymphoma of the larynx: A case report

Lymphoma is a malignant disease of lymphatic cells. Despite numerous sub-types, the disease is generally divided into two main groups: Hodgkin and non-Hodgkin lymphoma. Primary laryngeal non-Hodgkin lymphoma accounts for less than 1% of all laryngeal tumors. We herein present a 43-year-old male patient with difficulty swallowing due to a laryngeal mass. Morphological and immunohistochemical examination revealed T cell lymphoma according to the World Health Organization classification. The patient was referred for chemo-radiotherapy. Primary lymphoma of the larynx is a rare, and primary T-cell laryngeal lymphoma is an even rarer tumor.

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.

Role of Surgical Treatment in Primary Intestinal Non-Hodgkin Lymphomas: A SEER Population-Based Survival Analysis

Background: Evidence regarding the need for surgery for primary intestinal non-Hodgkin lymphoma (PINHL) patients with chemotherapy is limited and controversial. However, there is a lack of large-scale multicenter studies for the specific analysis of this population. We aimed to investigate the specific impact of surgery on survival in a large cohort of PINHL patients (aged >18 years) with chemotherapy. Methods: For this retrospective cohort study, data from PINHL patients (aged >18 years) with chemotherapy between 1983 and 2015 were extracted from the Surveillance, Epidemiology, and End Results (SEER) database through strict acceptance and discharge standards. The primary and secondary objectives were to access improved overall survival (OS) and improved cancer-specific survival (CSS) with surgery, respectively. In addition to multiple regression analysis and stratification analysis adjusted for confounding factors, propensity score matching (PSM) analysis was used to explore the reliability of the results to further control for confounding factors. Findings: Data were collected from the SEER database for 8398 patients; 3537 met inclusion criteria. The median survival was 53 months. Multivariate regression analysis showed that patients with surgery and chemotherapy had significantly 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 age, gender, race, marital status, year of diagnosis, Ann Arbor stage, histology, tumor size, and radiation. In particular, histological subgroup analyses showed that patients with primary intestinal diffuse large B-cell lymphoma had a better prognosis with chemotherapy plus surgery compared with the non-operation group (HR 0·71, 95% CI 0·60–0·84; P for interaction=0·0067). 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. Interpretation: 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. Funding: Supported by grants from the National Natural Science Foundation of China (grant no. 81873147) and the Natural Science Foundation of Guangdong Province (grant no. 2020A1515011177). Declaration of Interest: All the authors declared that they have no conflict of interest.

Uterine non-Hodgkin lymphoma: a case report and review of literatures

&lt;p&gt;&lt;span&gt;Primary extra nodal non-Hodgkin lymphomas of the genital tract are uncommon, and involvement of the uterine corpus is not only extremely rare but lacks pathognomonic signs and symptoms, resulting in delayed diagnosis and treatment. This prompted this case report of a multiparous lady who presented with recurrent bleeding per vaginam and progressive abdominal swelling that was initially suspicious of being uterine sarcoma, but histological analysis of excised sample was diagnostic of non-Hodgkin lymphoma of the uterus. The report also highlights the challenges to diagnosis and management in a resource poor setting where advanced diagnostic aids are not readily accessible. The diagnosis of uterine non-Hodgkin lymphoma, though uncommon, should be considered in any patient with an abdominal/pelvic mass and/or abnormal bleeding per vaginam. &lt;/span&gt;&lt;/p&gt;