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Mantle cell lymphoma (MCL) is frequently diagnosed at advanced stages and is characterized by multiple extranodal sites of disease, most notably the bone marrow, peripheral blood, and gastrointestinal tract. Historically the prognosis of mantle cell lymphoma has been poor with median survival of four to five years. With new treatment regimens, however, patients have been able to achieve prolonged remissions and require special attention while being evaluated for relapse. This report describes four patients treated for stage IV mantle cell lymphoma at the University of Miami who developed soft tissue relapse presenting as non-tender large masses of the extremities, including one patient who presented without associated nodal involvement. Average time to soft tissue relapse was 99 months (range: 28–240) following initial diagnosis. Providers who care for patients with mantle cell lymphoma should be aware of soft tissue lesions as a presentation of mantle cell lymphoma that merits evaluation for disease relapse. [ABSTRACT FROM AUTHOR]

Background: Primary breast lymphoma (PBL) is a rare type of extranodal lymphoma, the diagnostic process for which presents significant challenges owing to an overlap in clinical and pathological features with those observed in triple-negative breast cancer (TNBC). However, the current literature reveals a paucity of information regarding the ramifications of potential diagnostic errors, particularly in the context of emergent therapeutic strategies for TNBC. Thus, we present a unique report of a case of PBL. Case presentation: A 76-year-old female with no past medical or family history presented to the hospital with the chief complaint of a mass in the right breast. Two masses were palpated in the right breast: one 56 mm mass (No. 1) located at 10 o'clock, and a 21 mm large, elastic, hard mass (No. 2) at 4 o'clock. Needle biopsy was performed only on the larger 56 mm mass (No. 1). The results showed invasive carcinoma that was negative for estrogen receptor, progesterone receptor, and human epidermal growth factor receptor-2. The preoperative diagnosis was right breast cancer (T3N0M0 Stage IIB) of the TNBC subtype. The patient refused the preoperative chemotherapy recommended by the treatment team; therefore, right breast mastectomy and sentinel lymph-node biopsy were performed instead. The histopathological diagnosis of the first mass was diffuse large B-cell lymphoma (DLBCL); that of the second mass (No. 2) was an invasive breast carcinoma of no special type. Postoperative treatment consisted of endocrine therapy (letrozole) for breast cancer, while the DLBCL was treated with chemotherapy and three courses of intrathecal chemotherapy. At the time of this report, the patient is still living, and neither tumor had recurred in the 2 years following surgery. Conclusions: On rare occasions, PBL can preoperatively mimic TNBC. While this case did not lead to serious consequences, because surgery was eventually selected as the first therapy, clinicians should be aware that the diagnosis of PBL is challenging using only a core-needle biopsy and can often be misdiagnosed as TNBC. [ABSTRACT FROM AUTHOR]

A 51-year-old female with menorrhagia was found to have a cervical polyp. Polypectomy and endometrial curettage showed an atypical lymphoid infiltrate. Hysterectomy was performed, showing extensive myometrial infiltration by small, cytologically bland CD3-positive αβ T cells with a non-activated cytotoxic phenotype and a low proliferative rate. PCR showed clonal TCR-β gene rearrangement. Lymph nodes were uninvolved. PET-CT was negative. A diagnosis of CD8-positive T-cell lymphoproliferative disorder (T-LPD) was made. At 6 months, the patient was asymptomatic with a negative repeat PET-CT. A critical recent advance in the classification of lymphoid neoplasms is the recognition of indolent extranodal T-LPDs, including those of the gastrointestinal tract (T-cell and NK-cell types) and skin (small/medium CD4-positive and acral CD8-positive). However, T-LPDs of the uterus are rare. Two indolent T-LPDs of the uterus have been reported, both showing a CD8-positive, nonactivated cytotoxic phenotype, low proliferative rate, and clonal TCR rearrangement. Neither developed systemic disease nor recurrence. The etiology of indolent T-LPDs and their relationship to overt T-cell lymphomas remain poorly understood. T-LPDs of the uterus may arise from effector memory T-cells that establish long-term, tissueresident immunologic memory following exposure to fetal extravillous trophoblastic cell alloantigens during a previous pregnancy. Neither our patient nor the 2 previously reported had a current pregnancy or a known recent infection or toxic exposure, and the event(s) triggering evolution into T-LPD are unknown. Indolent T-LPDs can be encountered at new and unusual extranodal sites; knowledge of their clinicopathological features will help avoid unnecessary cytotoxic chemotherapy and improve understanding of this group of disorders. [ABSTRACT FROM AUTHOR]

Background: Extranodal Non-Hodgkin lymphoma (NHL) is more prevalent in the gastrointestinal (GI) tract than in other sites. This study aimed to explore the clinical features and prognostic factors of primary intestinal diffuse large B-cell lymphoma (PI-DLBCL), in order to provide new references for basic research and clinical diagnosis and treatment of the rare extranodal malignant lymphoma. Methods: The clinical data of 88 patients with PI-DLBCL admitted to Fujian Medical University Union Hospital from June 2011 to June 2022 were retrospectively studied, the clinical and pathological features, diagnosis and treatment process and prognosis of PI-DLBCL were analyzed, and univariate and multivariate analysis of prognostic factors was carried out. The Kaplan-Meier method was used for survival analysis. Meanwhile, the latest literature from PubMed was retrieved to systematically discuss the research progress in the diagnosis and treatment of PI-DLBCL. Results: Among the 88 patients with PI-DLBCL included in this study, 60 cases were males (68.18%), 28 cases were females (31.82%), and 62 patients (70.45%) were complaining of abdominal pain, and the second most common clinical manifestation was changes in bowel habits in 16 (18.18%), with a median age of onset of 57 (17-82) years. The first-line treatment regimen was surgery combined with R-CHOP chemotherapy (56.82%). The median follow-up time was 72 (1-148) months, 51 (57.95%) of 88 patients with PI-DLBCL survived, 30 patients (34.09%) died, 7 patients (7.95%) were lost to follow-up, and the PFS rates of 1-year, 3-year and 5-year were 57.95%, 29.55% and 15.91%, and the OS rates of 1-year, 3-year and 5-year were 79.55%, 45.45% and 28.41%, respectively. The results of univariate Cox regression analysis showed that ECOG score, Lugano stage, B symptoms, IPI score, white blood cells, serum LDH, albumin, β2 microglobulin were the influencing factors of OS in PI-DLBCL patients, and ECOG score, Lugano stage, B symptoms, IPI score, white blood cells, serum LDH, albumin, b2 microglobulin were all the influencing factors of PFS in PI-DLBCL patients. The results of multivariate Cox analysis showed that Lugano stage may be an independent prognostic factor affecting OS and PFS in PI-DLBCL patients. Conclusion: PI-DLBCL is more common in middle-aged and elderly men, clinical manifestations lack specificity, first-line treatment is mainly surgery combined with standard chemotherapy regimens. The Lugano stage may be an independent prognostic factor affecting OS and PFS in PI-DLBCL patients. [ABSTRACT FROM AUTHOR]

Summary: Primary gastric diffuse large B‐cell lymphoma (PG‐DLBCL) accounts for the majority of extra‐nodal DLBCL. Even so, literature is lacking on early, localised presentations. We studied a cohort of patients with stage I disease, diagnosed between 2006 and 2018, from six centres between Australia, Canada and Denmark. Our goal was to characterise outcomes, review treatment and investigate the role of interim positron emission tomography (iPET). Thirty‐seven eligible patients were identified. The median duration of follow‐up was 42.2 months. All received chemoimmunotherapy with 91.9% (n = 34) given rituximab, cyclophosphamide, doxorubicin, vincristine and prednisolone (R‐CHOP). 35.1% (n = 13) underwent consolidative radiotherapy. Eighteen patients were H. pylori positive and 11 had the documentation of H. pylori eradication therapy. The 4‐year progression‐free survival and overall survival of R‐CHOP was 88% (95% CI: 71–95) and 91% (95% CI: 75–97) respectively. All patients who achieved a partial metabolic response or complete metabolic response on iPET went on to achieve complete response at the end of treatment. R‐CHOP‐based therapy with iPET assessment appears to offer favourable outcomes, with radiotherapy and H. pylori eradication therapy implemented on a case‐by‐case basis. [ABSTRACT FROM AUTHOR]

Background: Combined PET/CT using 18F could be very effective with minimal hazards as well as false findings compared to the use one of these components alone in assessment of lymphoma. The purpose of this study was to review the usefulness of FDG PET/CT in diagnosis and staging of lymphoma, assessment of response to therapy, follow up and assessment of remissions and relapses among patients who had extranodal lymphoma. Methods: This cross-sectional study involved 36 patients who had pathologically confirmed lymphoma who underwent PET/CT study. A PET/CT in-line system was used to collect all of the data (General Electric PET CT Discovery). Prior to conducting a diagnostic enhanced whole-body CT scan, we conducted a low-dose enhanced CT scan, then a whole-body PET study. Results: The study compared the efficacy of contrast-enhanced CT and PET/CT in detecting lymphoma manifestations in 36 patients. PET/CT showed high sensitivity (100%) in detecting lymph node involvement and other extranodal manifestations, with varying specificity. Treatment response was evaluated using International Harmonization Project (IHP) and visual assessment based on the Déauville Criteria, showing a high level of agreement between the two methods. Conclusion: One promising oncologic imaging modality that could play a significant role in lymphoma care is a PET/CT combination that using 18F-FDG. In the early interim and after treatment ended, response assessments based on the Deauville criteria seemed to agree with IHP classifications to a reasonable degree. [ABSTRACT FROM AUTHOR]

Extranodal diffuse large B-cell lymphoma (DLBCL) is a heterogeneous disease process and an aggressive form of non-Hodgkin's lymphoma. We present a case of multiorgan involvement of DLBCL in a patient with documented risk factors, including [ 18 F] fluorodeoxyglucose positron emission tomography/magnetic resonance imaging findings highlighting striking perineural spread involving intracranial and extracranial segments of the bilateral trigeminal nerves. [ABSTRACT FROM AUTHOR]

Follicular lymphoma (FL) is one of the frequent varieties of non-Hodgkin's lymphoma (NHL). Secondary skeletal muscle FL is an uncommon extra-nodal lymphoma that is more challenging to identify. Spectral CT imaging can aid in the diagnosis of it. A 52-year-old man who had a bulge in his left inguinal area for two months in 2021. Following a mass aspiration biopsy, cervical lymph node dissection and a bone marrow aspiration biopsy, the patient was diagnosed as follicular lymphoma grade I with bone marrow involvement. Later, the patient had progressive worsening of low back pain for 2 month and underwent a lumbar spectral enhanced-CT scan, which demonstrated the right psoas major muscle and the retroperitoneal swollen lymph nodes were discovered to be homologous lesions, indicating the lymphoma infiltration of the right psoas major muscle. Spectral enhanced-CT is a non-invasive, practical, and reliable alternative technique. By reconstructing and comparing the energy spectrum curves of different tissues to shed light on the nature of the mass, it can aid in the diagnosis of extra-nodal lymphoma, which is of great value in determining the extent of the lesion and guiding the clinical treatment plan.

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A 76-year-old female with a history of multiple falls on the patella presented with worsening knee pain, swelling, and reduced range of motion. Radiographs revealed tricompartmental osteoarthritis and subtle erosion of the posterior cortex of the patella that was missed by the referring orthopedist and radiologist. Her persistent pain prompted an MRI, revealing a mass later confirmed to be primary diffuse large B-cell lymphoma of the patella after biopsy and appropriate metastatic workup. Primary B-cell patellar lymphoma is a rarely described cause of knee pain requiring broad differential diagnosis and multidisciplinary workup in order to avoid erroneous treatment and ensure prompt oncologic treatment. [ABSTRACT FROM AUTHOR]

Extranodal lymphoma occurs in one-third of lymphoma patients and is a key indicator of the international prognostic index, associated with unfavorable outcomes. Due to the lack of ideal models, the causes and characteristics of extranodal lymphoma are greatly underexplored. Recently, we observed a high incidence of extranodal lymphoma in two types of mouse models with tropism for the brain and kidneys. These findings prompt us to rethink the pathological progression of lymphoma colonization in lymph nodes and non-lymphoid organs. Nodal lymphoma, primary extranodal lymphoma and secondary extranodal lymphoma should be biologically and clinically distinctive scenarios. Based on the observations in mouse models with extranodal lymphoma, we propose that lymphoma dissemination can be seen as lymphoma losing the ability to home to lymph nodes. The pathological process of nodal lymphoma should be referred to as lymphoma homing to distinguish it from benign hyperplasia. Lymphoma dissemination, defined as a pathological process that lymphoma can occur in almost any part of the body, is a key pathological hallmark for malignant progression of B-cell lymphoma. Reshaping cellular plasticity is a promising strategy to allow transformed cells to homing back to lymph nodes and re-sensitize tumor cells to treatment. From this perspective, we provide new insights into the pathological progression of lymphoma dissemination and its inspiration on therapeutic interventions. We believe that establishing extranodal lymphoma mouse models, identifying molecular mechanism governing lymphoma dissemination, and developing therapies to prevent lymphoma dissemination will become emerging topics for fighting relapsed and refractory lymphoma. [ABSTRACT FROM AUTHOR]

In the sinonasal tract, diffuse large B-cell lymphomas are the predominant type non-Hodgkin's lymphoma while natural killer (NK) cell T-cell lymphoma, the nasal type, constitute only 3%-12% of NHLs in this region and is the more aggressive subtype. NK cell T-cell lymphoma mostly occurs in male at the median age of 50 years and has got a poor prognosis with a 3-years overall survival (OS) of 46.3% and a 5-years OS of 42%. We present a case of a 37 year old lady with a mass in nasal cavity extending to the nasopharynx and upper cervical lymphadenopathy. Biopsy from the nasal mass showed features of NHL, NK cell T-cell type although immunohistochemistry was not available for the definite diagnosis. For localized diseases, chemo-radiotherapy forms the mainstay of treatment with complete remission in up to 50% of cases while chemotherapy alone is given to patients with disseminated disease with a very poor outcome (5-year survival of 10%-45% only). In our case, the patient received first dose of chemotherapy and then died at home before receiving the second dose showing the aggressive nature of the disease. Timely diagnosis with typical imaging features and histological diagnosis can improve the outcome with complete cure in almost half of the cases with localized disease.

Abstract Approximately 30% of lymphomas occur outside the lymph nodes, spleen, or bone marrow, and the incidence of extranodal lymphoma has been rising in the past decade. While traditional chemotherapy and radiation therapy can improve survival outcomes for certain patients, the prognosis for extranodal lymphoma patients remains unsatisfactory. Extranodal lymphomas in different anatomical sites often have distinct cellular origins, pathogenic mechanisms, and clinical manifestations, significantly influencing their diagnosis and treatment. Therefore, it is necessary to provide a comprehensive summary of the pathogenesis, diagnosis, and treatment progress of extranodal lymphoma overall and specifically for different anatomical sites. This review summarizes the current progress in the common key signaling pathways in the development of extranodal lymphomas and intervention therapy. Furthermore, it provides insights into the pathogenesis, diagnosis, and treatment strategies of common extranodal lymphomas, including gastric mucosa-associated lymphoid tissue (MALT) lymphoma, mycosis fungoides (MF), natural killer/T-cell lymphoma (nasal type, NKTCL-NT), and primary central nervous system lymphoma (PCNSL). Additionally, as PCNSL is one of the extranodal lymphomas with the worst prognosis, this review specifically summarizes prognostic indicators and discusses the challenges and opportunities related to its clinical applications. The aim of this review is to assist clinical physicians and researchers in understanding the current status of extranodal lymphomas, enabling them to make informed clinical decisions that contribute to improving patient prognosis.

Pulmonary lymphomas are rare. With the current less invasive approaches used to obtain material for diagnosis, the diagnosis of pulmonary lymphoma is now frequently established in a small biopsy rather than in a resection. Therefore, the diagnosis has become more challenging and requires correlation with the clinico-radiologic presentation and with ancillary studies (immunohistochemistry, flow cytometry, cytogenetics, and/or molecular analysis). Due to the rarity of pulmonary lymphomas, clinical suspicion of a lymphomatous process is low at initial presentation, and material may be only submitted for histopathology. For this reason, herein, we provide recommendations to arrive at the correct diagnosis of the most common lung B-cell lymphomas (marginal zone lymphoma of mucosa-associated lymphoid tissue, diffuse large B-cell lymphoma, intravascular large B-cell lymphoma, lymphomatoid granulomatosis) in the setting of small biopsies, utilizing only immunohistochemistry. The differential diagnosis varies according to the lymphoma subtype and includes reactive conditions, solid tumors, and other hematolymphoid malignancies. Although morphology and immunohistochemistry may be sufficient to establish a diagnosis, in some cases, the best recommendation is to obtain additional tissue via a VATS biopsy/wedge resection with material submitted for flow cytometry, cytogenetics, and/or molecular studies to be able to properly classify a pulmonary lymphoid process. [ABSTRACT FROM AUTHOR]

Non-Hodgkin lymphomas are a heterogeneous group of lymphoproliferative disorders with various behaviors and responses to treatment. As a primary extranodal NHL, the disease must be confined to one location, and bone marrow should not be involved. Primary uterine cervix lymphoma is a rare malignancy as well as a rare site of extranodal lymphoma. Because of the rarity of the disease, there is no standard treatment guideline for women with primary uterine cervix lymphoma. Patients mostly present with abnormal uterine bleeding, vaginal discharge or pelvic pain. Typically, a pap smear may not show the malignant cells in the specimen. When the diagnosis is made, management may be debated due to its rarity and lack of standard treatment. Surgery, chemotherapy, chemo-immunotherapy and radiotherapy, either alone or in combination, are the treatment options. Most patients respond well to chemotherapy and radiation therapy. The prognosis is usually favorable. Here, we report a case of primary cervical lymphoma in a 50-year-old woman who presented with abnormal vaginal bleeding. She was treated with radiotherapy and chemoimmunotherapy and remained disease-free after nine months of treatment. [ABSTRACT FROM AUTHOR]

Approximately 30% of lymphomas occur outside the lymph nodes, spleen, or bone marrow, and the incidence of extranodal lymphoma has been rising in the past decade. While traditional chemotherapy and radiation therapy can improve survival outcomes for certain patients, the prognosis for extranodal lymphoma patients remains unsatisfactory. Extranodal lymphomas in different anatomical sites often have distinct cellular origins, pathogenic mechanisms, and clinical manifestations, significantly influencing their diagnosis and treatment. Therefore, it is necessary to provide a comprehensive summary of the pathogenesis, diagnosis, and treatment progress of extranodal lymphoma overall and specifically for different anatomical sites. This review summarizes the current progress in the common key signaling pathways in the development of extranodal lymphomas and intervention therapy. Furthermore, it provides insights into the pathogenesis, diagnosis, and treatment strategies of common extranodal lymphomas, including gastric mucosa-associated lymphoid tissue (MALT) lymphoma, mycosis fungoides (MF), natural killer/T-cell lymphoma (nasal type, NKTCL-NT), and primary central nervous system lymphoma (PCNSL). Additionally, as PCNSL is one of the extranodal lymphomas with the worst prognosis, this review specifically summarizes prognostic indicators and discusses the challenges and opportunities related to its clinical applications. The aim of this review is to assist clinical physicians and researchers in understanding the current status of extranodal lymphomas, enabling them to make informed clinical decisions that contribute to improving patient prognosis. [ABSTRACT FROM AUTHOR]

Background: Lymphoma of the head and neck poses a diagnostic challenge which may lead to a delay in the treatment. Histological workup of patients with head and neck lymphoma would depend on the primary site. Image guided core biopsy is gaining acceptance as it provides excellent diagnostic yields from cervical lymph nodes. Design: 10-year retrospective study at the Department of Otorhinolaryngology Head & Neck Surgery, Hospital Pakar Sultanah Fatimah (Department of ORL HNS, HPSF), Muar, Johore. Results: Forty-eight patients were diagnosed with head and neck lymphoma during the study period with 85.4% non-Hodgkin lymphoma and 14.6% Hodgkin lymphoma. There were 47.9% males and 52.1% females with median age of 55.44 years. Neck swelling was the commonest presentation in 45.8% patients. Other ENT presentations included oral mass 22.9%, nasal mass 10.4%, epistaxis 10.4%, nasal blockage 8.3% and hoarseness 2.1%. Punch biopsy was the commonest mode of tissue biopsy in this study with 47.9% whereby surgical excision remained the standard diagnostic approach for cervical lymphadenopathy at 29.2%. Non-surgical approaches such as core biopsy is gaining acceptance because it is less invasive and not requiring anaesthesia consisted 8.3% in this study. Conclusion: Non-Hodgkin's lymphoma is more prevalent in the head and neck compared to Hodgkin's lymphoma. Cervical lymphadenopathy is the commonest presentation which can be diagnosed conventionally by open biopsy, but core biopsy may be considered as an alternative. [ABSTRACT FROM AUTHOR]

Extranodal marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue (MALT lymphoma) is among the more common types of lymphoma accounting for up to 8% of newly diagnosed lymphoma cases. As opposed to other B-cell lymphomas, however, no predominant genetic hallmark has been defined in MALT lymphoma, but different localizations appear to be affected by different, sometimes distinct changes. Nonetheless, a high proportion of these genetic changes reported in MALT lymphomas dysregulate the pathways leading to NF-kB activation. t(11;18)(q21;q21)/BIRC3::MALT1 appears to be MALT lymphoma specific and is found in 24% of gastric and 40% of pulmonary MALT lymphomas. The translocation is associated with more disseminated disease in gastric MALT lymphoma and is found in a large percentage of patients whose lymphoma is unresponsive to antibiotic eradication of Helicobacter pylori. In addition to t(11;18)(q21;q21), nuclear expression of BCL10 or NF-kB appears to be highly associated with lymphoma cell survival independence of H. pylori- mediated stimulations. Antibiotic eradication, however, is the recommended therapy of choice irrespective of genetic findings, and molecular analysis is not required before initiation of therapy. The influence of genetic translocations including t(11;18)(q21;q21) on systemic therapies, however, is less clearly defined. While small series have shown no influence on the outcome for treatment with the anti-CD20 antibody rituximab (R) or treatment with cladribine (2-CdA), conflicting data have been reported for alkylating agents, especially chlorambucil and the combination of R + chlorambucil. None of other genetic changes seen in MALT lymphoma to date has discernible value in routine clinical applications, but recent data suggest that changes in TNFAIP3 (A20), KMTD2 and CARD11 might be associated with response to Bruton kinase inhibitors. [ABSTRACT FROM AUTHOR]

Intravascular large B-cell Lymphoma (IVLBCL) is a rare subtype of extranodal non-Hodgkin’s lymphoma that is challenging to diagnose and has a poor prognosis. Here, we describe two patients newly diagnosed with IVLBCL at our institution: an African man with hemophagocytic-syndrome-associated IVLBCL and an Italian woman with a cutaneous variant of IVLBCL. They presented with very different clinical manifestations. Both cases were diagnosed in a timely manner, successfully treated, and achieved long-lasting remissions.

Abstract Objective To evaluate the overall survival (OS), disease-specific survival (DSS), and recurrence-free survival (RFS) for primary cervical lymphoma (PCL), an extremely rare disease without treatment consensus. Methods We conducted a retrospective study included 177 patients, including 169 cases identified from literature review. The Kaplan-Meier methods and Cox regression were used to determine the OS, DSS, RFS, and relevant risk factors. Results The 5-year OS and 5-year DSS rates were 85.8 and 87.2%, respectively, while the 5-year RFS rate was 85.5%. Diffuse large B-cell lymphoma (DLBCL) was the predominant subtype that comprised 63.8% (113 cases) in this cohort. Multivariate analysis in the DLBCL subgroup revealed that age ≥ 60 years (Odds ratio [OR]: 26.324, 95% Confidence Interval [CI]: 5.090–136.144, P

Central nervous system lymphoma (CNSL) represents one of the most aggressive forms of extranodal lymphoma. The gold standard for CNSL diagnosis remains the stereotactic biopsy, with a limited role for cytoreductive surgery that has not been supported by historical data. Our study aims to provide a comprehensive overview of neurosurgery's role in the diagnosis of systemic relapsed and primary CNSL, with an emphasis on the impact on management and survival. This is a single center retrospective cohort study with data collected between August 2012 and August 2020, including patients referred with a potential diagnosis of CNSL to the local Neuro-oncology Multidisciplinary Team (MDT). The concordance between the MDT outcome and histopathological confirmation was assessed using diagnostic statistics. A Cox regression is used for overall survival (OS) risk factor analysis, and Kaplan–Meier statistics are performed for three prognostic models. The diagnosis of lymphoma is confirmed in all cases of relapsed CNSL, and in all but two patients who underwent neurosurgery. For the relapsed CNSL group, the highest positive predictive value (PPV) is found for an MDT outcome when lymphoma had been considered as single or topmost probable diagnosis. Neuro-oncology MDT has an important role in establishing the diagnosis in CNSL, not only to plan tissue diagnosis but also to stratify the surgical candidates. The MDT outcome based on history and imaging has good predictive value for cases where lymphoma is considered the most probable diagnosis, with the best prediction for cases of relapsed CNSL, questioning the need for invasive tissue diagnosis in the latter group. [ABSTRACT FROM AUTHOR]

Introduction: Primary testicular lymphoma (PTL) is a rare disease, accounting for <5% of all testicular malignancies and 1–2% of non-Hodgkin lymphoma cases. Diffuse large B-cell lymphoma (DLBCL) is the most common histological diagnosis. The literature data concerning PTL is scarce and based mainly on small series or retrospective studies. Methods and results: In this paper, we present six patients with DLBCL-PTL who were treated in the Department of Hematology and Bone Marrow Transplantation at Poznan University of Medical Science, Poland between 2006 and 2022. All the patients obtained complete remission (CR) after six cycles of R-CHOP-21 (cyclophosphamide, doxorubicin, vincristine and rituximab on day 1, and prednisolone on days 1–5, administered every 21 days for a total of eight cycles) as immunochemotherapy. Five of them additionally received prophylaxis of central nervous system involvement with intrathecal methotrexate/arabinoside cytosine. One patient received scrotal radiotherapy, and in another one radio- therapy is planned. Relapse was confirmed in one patient after 72 months in the contralateral testis, and the patient was successfully retreated. After a median follow-up of 146 (range 5–196) months, all patients remain alive and in CR. Conclusion: Despite all interpretative limitations, the current standard DLBCL-PTL therapy seems to be six courses of CHOP-R-21 combined with intrathecal metothrexate and scrotal irradiation. [ABSTRACT FROM AUTHOR]

Intravascular large B-cell Lymphoma (IVLBCL) is a rare subtype of extranodal non-Hodgkin's lymphoma that is challenging to diagnose and has a poor prognosis. Here, we describe two patients newly diagnosed with IVLBCL at our institution: an African man with hemophagocytic-syndrome-associated IVLBCL and an Italian woman with a cutaneous variant of IVLBCL. They presented with very different clinical manifestations. Both cases were diagnosed in a timely manner, successfully treated, and achieved long-lasting remissions. [ABSTRACT FROM AUTHOR]

Among diffuse large b-cell lymphoma (DLBCL) subtypes, primary testicular lymphoma (PTL) has one of the highest risks of central nervous system (CNS) relapse. The converse, primary CNS lymphoma (PCNSL) relapse outside the CNS is rare. Molecular analysis has illustrated a genetic similarity between PTL and PCNSL. Here we present a case of a 64-year-old man with testicular relapse of PCNSL 20 months after a complete response to high dose methotrexate-based chemotherapy. His tumor demonstrated a molecular profile similar to both PCNSL and PTL on next generation sequencing, and molecular analysis confirmed common clonal origin of his CNS and testicular lesions. We review prior cases of testicular relapse of PCNSL, which lacked molecular investigations, and discuss the implications of the genomic findings in our patient, including future treatment options. [ABSTRACT FROM AUTHOR]

Extranodal dissemination is an important feature of aggressive B-cell lymphoma. Owing to the lack of available animal models, the study on extranodal dissemination of lymphoma is greatly limited. Here, we identified a novel cell line, named MA-K, which originated from the Eμ-Myc;Cdkn2a−/− cell line, named MA-LN in this study. Compared to MA-LN, MA-K tended to disseminate in the kidney rather than the lymph nodes in the lymphoma transplantation model, resembling human primary renal lymphoma. The transcriptome analysis revealed that MA-K had undergone transcriptional evolution during the culture. The specialized transcriptional pattern analysis we proposed in this study identified that the FOXO1-BTG1-MYD88 pattern was formed in MA-K. Further analysis found that the translation pathway was the most enriched pathway in specially expressed genes (SEGs) in MA-K. Among the SEGs, three upregulated genes, RPLP2, RPS16, and MRPS16, and five downregulated genes, SSPN, CD52, ANKRD37, CCDC82, and VPREB3, in MA-K were identified as promising biomarkers to predict the clinical outcomes of human DLBCL. Moreover, the joint expression of the five-gene signature could effectively predict clinical outcomes of human DLBCL in three groups. These findings suggested that the MA-K cell line had strong clinical relevance with human aggressive B-cell lymphoma. Moreover, the MA-K primary renal lymphoma model, as a novel syngenetic mouse model, will be greatly useful for both basic research on lymphoma dissemination and preclinical efficacy evaluation of chemotherapy and immunotherapy.

Purpose: Surgery of primary thyroid lymphoma (PTL) has been mostly limited to diagnostic work-up. This study aimed to further study its potential role. Methods: This was a retrospective study from a multi-institutional registry of PTL patients. Clinical, diagnostic work-up (fine needle aspiration, FNA; core needle biopsy, CoreNB), contribution of surgery (open surgical biopsy, OpenSB; thyroidectomy), histology subtype, and outcome data were evaluated. Results: Some 54 patients were studied. Diagnostic work-up included FNA in 47 patients, CoreNB in 11, and OpenSB in 21. CoreNB yielded the best sensitivity (90.9%). Thyroidectomy was performed in 14 patients with other diagnosis (incidental PTL), in 4 for diagnosis and in 4 for elective treatment of PTL. Incidental PTL was associated with not performed FNA nor CoreNB (OR 52.5; P = 0.008), mucosa-associated lymphoid tissue (MALT) subtype (OR 24.3; P = 0.012), and Hashimoto’s thyroiditis (OR 11.1; P = 0.032). Lymphoma-related death (10 cases) mostly occurred within the first year after diagnosis and was associated with diffuse large B-cell (DLBC) subtype (OR 10.3; P = 0.018) and older patients (OR 1.08 for every 1-year increase; P = 0.010). There was a trend towards lower mortality rate in patients receiving thyroidectomy (2/22 versus 8/32, P = 0.172). Conclusion: Incidental PTL accounts for most of thyroid surgery cases and are associated with incomplete diagnostic work-up, Hashimoto’s thyroiditis and MALT subtype. CoreNB appears to be the best tool for diagnosis. Most of PTL deaths occurred during the first year after diagnosis and mostly related to systemic treatment. Age and DLBC subtype are poor prognostic factors. [ABSTRACT FROM AUTHOR]

Objective: To evaluate the overall survival (OS), disease-specific survival (DSS), and recurrence-free survival (RFS) for primary cervical lymphoma (PCL), an extremely rare disease without treatment consensus. Methods: We conducted a retrospective study included 177 patients, including 169 cases identified from literature review. The Kaplan-Meier methods and Cox regression were used to determine the OS, DSS, RFS, and relevant risk factors. Results: The 5-year OS and 5-year DSS rates were 85.8 and 87.2%, respectively, while the 5-year RFS rate was 85.5%. Diffuse large B-cell lymphoma (DLBCL) was the predominant subtype that comprised 63.8% (113 cases) in this cohort. Multivariate analysis in the DLBCL subgroup revealed that age ≥ 60 years (Odds ratio [OR]: 26.324, 95% Confidence Interval [CI]: 5.090–136.144, P < 0.001) or stage IIIE-IVE (advanced stage) (OR: 4.219, 95%CI: 1.314–13.551, P = 0.016) were the risk factors for OS, while patients with age ≥ 60 years (OR:23.015, 95%CI: 3.857–137.324, P = 0.001), and stage IIIE-IVE (OR: 4.056, 95% CI: 1.137–14.469, P = 0.031) suffered a poor DSS. Chemotherapy and/or radiotherapy improved the OS (P = 0.008), DSS (P = 0.049), and RFS (P = 0.003). However, cancer-directed surgery did not improve the OS, DSS, and RFS. The risk factor was unavailable in other subtypes of PCL due to limited cases. Conclusion: The survival outcomes in patients with PCL at early stage were satisfactory, while the advanced disease stage and age ≥ 60 years were the two major factors predicting poor prognosis in DLBCL subtype. [ABSTRACT FROM AUTHOR]

Background: Intravascular large B-cell lymphoma (IVLBCL) is a rare subtype of extranodal B-cell lymphoma, which has traditionally been associated with poor outcomes. Despite increasing recognition, IVLBCL requires a high degree of clinical suspicion on the part of the clinician for its diagnosis. Case Series: We present four patient cases: A 69-year-old female with constitutional symptoms and cognitive decline; a 78-year-old female with kidney injury and constitutional symptoms whose disease rapidly progressed to multiorgan failure and death; a 70-year-old asymptomatic female with an incidentally found, enlarged thyroid; and a 63-year-old male with cytopenia and constitutional symptoms. Retrospective chart analysis was performed on these four patients diagnosed with IVLBCL at our Institute to identify the pathognomonic features of the disease and compare these to the published evidence. IVLBCL has a heterogeneous presentation, as seen in our four patients. The disease is characterized by the exclusive presence of malignant cells inside the blood vessels and lack of organ infiltration. Traditional preliminary diagnostic modalities such as imaging are usually inconclusive, given the paucity of lymphomatous aggregates. A bone marrow biopsy, random skin biopsies, or a focal organ biopsy in appropriate cases is required for diagnosis. Immunosuppression might play a role in the pathogenesis. Timely initiation of aggressive cancer-directed therapy was associated with improved outcomes. Monitoring for disease response and relapse continues to be a challenge. Conclusion: Our mini-series highlights the significance of timely diagnosis and intervention in IVLBCL and emphasizes the importance of further research to determine its association with immunosuppression. [ABSTRACT FROM AUTHOR]

Primary colorectal diffuse large B-cell lymphoma (DLBCL) is very rare colon malignancy. It is important to know the main demographic and clinical characteristics of these patients. We conducted a retrospective analysis of 18 patients diagnosed with primary colorectal DLBCL during a 17-year period at the National Cancer Institute of Brazil (INCA) between 2000 and 2018. Demographic characteristics, tumor localization, HIV status, lactate dehydrogenase (LDH) levels, treatment modality and follow-up status were obtained from medical records. Survival was estimated from the date of diagnosis until death. There were 11 male and seven female patients in our cohort, the median age at diagnosis was 59.5 years and four patients were HIV positive. Tumor was mainly localized in the right colon. Patients were treated with chemotherapy (CT) and/or surgical resection. Eleven patients died during a median follow-up of 59 months and the median survival time was 10 months. Six or more cycles of CT (HR=0.19; CI 95% 0.054–0.660, p = 0.009), LDH levels below 350 U/L (HR=0.229; CI 95% 0.060–0.876, p = 0.031) and surgical resection (HR=0.23; CI 95% 0.065–0.828, p = 0.030) were associated with reduced risk of death in univariate analysis. Patient's age and DLBCL right colon localization should be considered at diagnosis to distinguish between DLBCL and other diseases for differential diagnosis. Six cycles of CT, LDH levels below 350 U/L and surgical resection were associated with better survival. Our results are consistent with previous publications and address the importance of correct colorectal DLBCL diagnosis and treatment.

Abstract Background Primary female genital system lymphoma (PFGSL) is an infrequent entity. All genital organs may be affected, and most PFGSLs are localized to the cervix, uterine body, and ovaries. The clinical manifestations are nonspecific, which complicates a timely diagnosis. We report an unexpected case of PFGSL and discuss the disease characteristics by reviewing the literature. Case presentation A 48-year-old G3/P2 woman presented to the Department of Gynecology with a physical examination. Ultrasound examination and CT revealed pelvic masses. The woman underwent surgical treatment because of the pelvic masses and underwent a hysterectomy for a recurrent mucinous borderline ovarian tumor. However, the results of the postoperative pathological examination showed diffuse large B-cell lymphoma of the endometrium. After four courses of chemotherapy, the woman was in good condition. The clinical manifestations were nonspecific, which made a timely diagnosis complex. Conclusion This case highlights the importance of the difficulty in detecting early PFGSL early and how easily nonspecific manifestations can be ignored. It may lead to missing the best time for early treatment.

Compared to Asian and Latin American populations, sinonasal NK- or T-cell lymphoma is rare in Europe. All patients with sinonasal NK- or T-cell lymphoma in Denmark from 1980 to 2017 were validated histologically, and the disease behavior and demographics were extracted from medical records and national registries. Prognostic factors associated with mortality were determined using survival statistics. We included 56 patients: 40 extranodal NK/T-cell lymphoma (nasal type) (ENKTCL) and 16 peripheral T-cell lymphoma (not otherwise specified) (PTCL). The median age was 66, and most patients were male (72%). The ENKTCL and PTCL 5-year overall survival was 48% and 50%, respectively; progression-free survival was 38% for both. With ENKTCL, stage and performance status increased mortality significantly (HR = 8.6; p < 0.001 and HR = 4.23; p = 0.04). In conclusion, disseminated disease had a dismal outcome and the onset of ENKTCL in this ethnically homogeneous European cohort was about a decade later than reported in Asian populations. [ABSTRACT FROM AUTHOR]

Primary thyroid lymphoma (PTL) is an uncommon malignancy. The majority of PTLs are non-Hodgkin's lymphoma. Fluorodeoxyglucose positron emission tomography-computed tomography (FDG PET/CT) is the state-of-the-art imaging in lymphoma and plays a pivotal role in staging, follow-up, and treatment decisions. There is good evidence that it performs well in a wide variety of nodal and extranodal lymphomas (ENL). However, the data are scarce on its usefulness in rare ENL. Herein, we describe four cases of PTLs and a brief review of the literature. FDG PET/CT was performed at baseline to assess the disease status and response to treatment. This case series highlights the varied appearance of PTL and response to treatment. It establishes the importance of FDG PET/CT in the personalized management of PTL. [ABSTRACT FROM AUTHOR]

Marginal zone lymphoma of the mucosa-associated lymphoid tissue (MALT lymphoma) represents 8% of all B-cell lymphomas and it is the most common small B-cell lymphoma arising at extranodal sites. The gold-standard test to establish a diagnosis of MALT lymphoma remains histopathologic analysis with the aid of immunohistochemistry (IHC) and/or flow cytometry immunophenotypic analysis. MALT lymphoma represents a progression from a persistent chronic inflammatory process, and therefore distinguishing MALT lymphoma from chronic inflammation by histopathology may be challenging in some cases. Despite recent trends to consider IGH rearrangement/clonality as a confirmatory diagnostic test of MALT lymphoma, this method is far from ideal for this purpose since a positive or a negative result does not necessarily confirm or exclude that a process is lymphoma or reactive. This test must be correlated with the morphologic findings. Moreover, MALT lymphoma may arise in association with underlying autoimmune conditions where clonal lymphoid populations are not uncommonly detected. Therefore, we believe that an integrated approach including detailed morphologic review in combination with IHC and/or flow cytometry is best to establish a diagnosis of MALT lymphoma in most cases. We present helpful morphologic tips to avoid potential diagnostic pitfalls at some of the most common extranodal sites, including the stomach, ocular adnexa/conjunctiva, salivary gland, lung, thymus, breast, thyroid, small and large intestine and the dura. The differential diagnosis of MALT lymphoma with IgG4-related disease is also discussed., (Copyright © 2024. Published by Elsevier Inc.)

Intravascular large B-cell lymphoma (IVLBCL) is a distinct subtype of extranodal diffuse large B-cell lymphoma (DLBCL) and mainly affects elderly population characterized by selective infiltration of neoplastic cells within the lumina of small vessels. Prostate primary IVLBCL is extremely rare. Herein we present a case of a 76-year-old male patient who was admitted with symptoms of severe lower urinary tract obstruction. IVLBCL was diagnosed based on histopathological and immunohistochemical examination of the precious tissue specimens though transurethral prostate resection. Awareness and accurate diagnosis are very important to guide the clinicals in formulation of diagnosis and treatment plan.

The gastrointestinal tract is the primary localization site of lymphoproliferative lesions ranging from reactive lymphoid hyperplasia to lymphoma. Diagnosis of these diseases is difficult, especially in small endoscopic biopsies. And integrated approach based on clinical, morphological, immunohistochemical and molecular data is needed for accurate diagnosis. In this review, the basic histological features of non-Hodgkin lymphomas in the gastrointestinal tract and the entities included in the differential diagnosis are discussed. [ABSTRACT FROM AUTHOR]

Primary soft-tissue lymphoma (PSTL) is a rare extranodal non-Hodgkin lymphoma, characterized by a mass growing within soft-tissue, which is connective tissue, adipose tissue, and skeletal muscle. Here, we describe a case of biphenotypic lymphoblastic lymphoma arising from soft tissue of the popliteal fossa in an 11-year-old boy. A pediatric review about PSTL revealed that anaplastic large cell lymphoma is the most common histological type and a biphenotypic lymphoblastic lymphoma has not yet been reported in childhood. Lymphoma should always be considered in patients presenting with a soft-tissue mass, and a comprehensive immunohistochemical evaluation, including B-cell, T-cell, and myeloid markers, is needed to make a correct diagnosis and establish the most suitable treatment. [ABSTRACT FROM AUTHOR]

Primary lymphoma of the central nervous system (PLCNS) is a rare form of extranodal lymphoma characterized by an aggressive course. In recent decades, the treatment of this disease has undergone significant changes. Modern treatment of PLCNS consists of two steps – induction and consolidation of remission. Improved overall survival of PLCNS patients was achieved through the use of combined immunochemotherapy regimens with high doses of methotrexate and/or cytarabine, which are currently the standard of induction therapy. High-dose chemotherapy followed by autologous hematopoietic stem cell transplantation at the consolidation stage provides the most complete elimination of the residual tumor clone. The most effective modes of conditioning are those with the inclusion of tiotepa. In elderly and comorbid patients who are not candidates for autologous hematopoietic stem cell transplantation, promising results were obtained while using maintenance therapy with procarbazine or temozolomide, and further researches will allow us to study the effectiveness of monocolonal anti-CD20 antibodies – rituximab and obinutuzumab.Treatment options for patients with relapses and / or refractory forms of PLCNS are limited. Certain successes were obtained with the use of new drugs – ibrutinib, nivolumab, lenalidomide, obinutuzumab.

B-lymphoblastic lymphoma is a neoplasm of immature B cells and is characterized by aggressive behavior and disease progression. Common sites of involvement are skin, lymph nodes, bone, soft tissues, breast, and the mediastinum. Gastrointestinal lesions are rarely encountered and therefore not fully described. We herein report the case of a 28-year-old male, who presented with abdominal pain and CT scan showed a tumor involving the small bowel and its mesentery. He underwent emergency laparotomy and enterectomy. Histopathology report revealed B-lymphoblastic lymphoma affecting the small bowel and the adjacent mesentery. This is the first documented case of a small bowel tumor diagnosed as B-lymphoblastic lymphoma in published literature. [ABSTRACT FROM AUTHOR]

Extranodal T/NK lymphoma (LTNKEN) is a rare and aggressive form of non-Hodgkin lymphoma of high-grade malignancy. A distinction is made between the nasal forms, characterized by primary involvement of the nasal cavity, and the extranasal forms. Primary skin involvement is highly rare. Diagnosis is based on immunohistochemical studies. Treatment includes chemotherapy and radiotherapy. This type of lymphoma has a poor prognosis even with treatment. Herein, we report a Moroccan case of cutaneous T/NK lymphoma in a young patient with a primary cutaneous localization of fatal evolution. [ABSTRACT FROM AUTHOR]

Diffuse large B-cell lymphoma (DLBCL) exhibits clinical, genetic, and immunohistochemical heterogeneity. However, the differences between primary extranodal or nodal DLBCL and double-expressor lymphoma (DEL), which is characterized by high MYC and BCL2 expression, remain unclear. This study aimed to elucidate the clinicopathological features, response to therapy, and clinical outcomes of primary extranodal (n=61) and nodal (n=128) DLBCL. Patients with primary nodal DLBCL had higher BCL2 expression than those with extranodal DLBCL (p =0.048), with high MYC expression and DEL as poor prognostic factors. Conversely, in patients with primary extranodal DLBCL, high BCL2 expression, low BCL6 expression, non-germinal center B-cell-like type, and DEL indicated poor prognosis. DEL was significantly associated with progression free survival and overall survival in patients with primary extranodal DLBCL (p =0.014 and p =0.021, respectively) but not in patients with primary nodal DLBCL (p =0.37 and p =0.084, respectively). Our findings highlight primary extranodal DEL as a strong adverse prognostic factor in DLBCL. • Clinicopathological features of primary extranodal and nodal DLBCL were analyzed. • High BCL2 expression is more frequent in primary nodal DLBCL. • Primary extranodal DEL is a strong, poor prognostic factor for DLBCL. [ABSTRACT FROM AUTHOR]

Background: Ultra-low dose radiotherapy (ULDRT) (2 × 2 Gy) has been used for symptomatic control of low-grade lymphomas with surprising local control rates, suggesting that these entities could respond to lower doses. These are particularly desirable for the treatment of orbital sites and some publications refer to high rates of complete responses. In this paper, we present our experience with the use of ULDRT for indolent orbital lymphomas. Materials and methods: Electronic files and treatment plans of patients treated with ULDRT for low-grade orbital lymphoma were retrospectively reviewed. Oncological outcomes and toxicities were collected and described for each patient. Results: Seven patients (median age of 75 years) with 8 lesions (3 follicular, 2 MALT, 1 marginal and 1 low-grade non-Hodgkin lymphoma) were considered for analysis. The majority had stage IE disease and one patient had bilateral disease. Six tumors were detected on imaging (median size of 20 mm). Involved orbital sites were periocular, conjunctival and palpebral; there was one case of intraocular (choroid) and one case of lacrimal gland involvement. One patient received consolidative rituximab after RT. The median follow-up time was 22 months. Two patients had partial response, one of them with persistent minimal choroidal disease and the other with partial response on CT. Five (71%) patients had clinical (n = 2) or radiologic (n = 3) complete response on treated sites. Reported late toxicities were minimal and included dry eye and pruritus. Conclusion: In our experience, ULDRT achieved a local control rate of 100% and complete response rate of 71% with minimal toxicity. [ABSTRACT FROM AUTHOR]

Lymphomas are common solid malignancies. They are associated with substantial morbidity and mortality. Hodgkin's lymphoma (HL) and Non-HL (NHL) are subtypes of lymphoma. Lymph nodes are the most common site of involvement, though practically any organ may be involved. NHL has preponderance for extranodal involvement. Primary uterine and ovarian NHL is scarce. However, in advanced systemic disease, secondary utero-ovarian involvement may be seen. 18F-fluorodeoxyglucose positron emission tomography/computed tomography (FDG PET/CT) is a pivotal imaging modality in lymphomas. It abets in pretreatment staging, posttherapy restaging, and surveillance. We present three stage-IV NHL cases with secondary utero-ovarian involvement. FDG PET/CT as a baseline imaging modality established the disease burden and organ involvement. [ABSTRACT FROM AUTHOR]