Primary esophageal follicular lymphoma: a rare entity / Linfoma folicular esofágico primario: una entidad rara

A 63-year-old woman presented with dyspepsia de novo. An esophagogastroduodenoscopy revealed a 30 mm flat yellowish esophageal lesion, 28 cm from the incisors (Fig. 1a), with the stomach and duodenum without any lesion. Helicobacter pylori infection was excluded. Histological examination was suggestive of a lymphoproliferative process (Fig. 1b). Immunohistochemistry showed diffuse positivity for CD20 (Fig. 1c) and BCL-2 (Fig. 1d), dim CD10 and BCL-6 staining, a Ki-67 of 20-25%, no CD21 or cyclin D1 expression, all these features compatible with low-grade follicular lymphoma. Physical examination was unremarkable. Computed tomography of the neck, chest, and abdomen revealed no lymph node enlargement, hepatosplenomegaly or metastasis. Blood routine tests and tumor markers were at normal levels. Bone marrow biopsy showed no involvement by lymphoma. Therefore, a diagnosis of primary follicular lymphoma of the esophagus was made. The patient opted for a watch-and-wait strategy and there is no evidence of disease progression after four years of follow-up.(AU)

Linfoma difuso de célulasB grandes primario de cérvix. A propósito de un caso / Primary diffuse large Bcell lymphoma of the cervix: Clinical case

Introducción: La afectación primaria del tracto genital femenino por linfoma no Hodgkin es muy poco frecuente, por lo que no existe un consenso sobre el tratamiento, y por ello presentamos este caso clínico y el tratamiento realizado, así como el pronóstico de nuestra paciente. Hallazgos clínicos: La paciente presentada es una mujer de 72años que consulta por hemorragia vaginal. Diagnóstico Se diagnostica de linfoma no Hodgkin extranodal primario de cérvix de inmunofenotipoB de alto grado citológico y elevado índice proliferativo. Intervenciones terapéuticas y resultados: El tratamiento de elección fue únicamente quimioterápico. Esta paciente ha presentado una supervivencia libre de enfermedad de 5años. Actualmente se encuentra en seguimiento mediante la realización de controles analíticos anuales. Conclusión: Los síntomas de este tipo de tumor son altamente inespecíficos y la citología es frecuentemente negativa, por lo que es necesario recurrir a la biopsia. La inmunohistoquímica resulta fundamental tanto para el diagnóstico como para el pronóstico. Existen múltiples técnicas de imagen empleadas tanto para estudio de extensión como para seguimiento, destacando el papel del FDG-PET. Actualmente parece que el tratamiento más recomendable es la pauta quimioterápica R-CHOP seguida de radioterapia. El pronóstico en general es bueno, con hasta un 80% de supervivencia a los 5años.(AU)

Introduction: Primary involvement of the female genital tract by non-Hodgkin lymphoma is very rare, so there is no consensus on treatment, and for this reason we present this clinical case and the treatment performed, as well as the prognosis of our patient. Clinical findings: The patient presented is a 72-year-old woman who consulted for vaginal bleeding. Diagnosis: Primary extranodal non-Hodgkin lymphoma of the cervix with immunophenotypeB of high cytological grade and high proliferative index was diagnosed. Therapeutic interventions and results: The treatment of choice was chemotherapy only. This patient has presented a disease-free survival of 5years. It is currently being monitored by carrying out annual analytical controls. Conclusion: The symptoms of this type of tumor are highly non-specific, as well as cytology is frequently negative, which is why it is necessary to resort to biopsy. Immunohistochemistry is essential for both diagnosis and prognosis. There are multiple imaging techniques used for both extension study and follow-up, highlighting the role of FDG-PET. It currently seems that the most recommendable treatment is the R-CHOP chemotherapy regimen followed by radiotherapy.The prognosis is generally good, with up to 80% survival at 5years.(AU)

Recent advances in CAR T-cell therapy for lymphoma in China

Lymphoma is a hematologic malignancy which mainly consists of Hodgkin lymphoma (HL) and non-Hodgkin lymphoma (NHL). Although systemic chemotherapy, radiotherapy, and other advanced therapeutics, including rituximab or immune checkpoint inhibitors, have improved the prognosis in recent decades, there are still a number of patients with relapsed or refractory (R/R) lymphoma with a poor prognosis. Chimeric antigen receptor (CAR) T-cell therapy has provided a curative option for patients with relapsed or refractory lymphoma. Numerous clinical trials have been conducted worldwide and presented inspiring results that give insight into this breakthrough therapy. The development of cancer cell therapy in China has been rapid in the past years and dominates the field with the USA. This review aims to summarize the published results of CAR T-cell therapy alone or in combination with other therapies in mainland China, both in R/R NHL and R/R HL (AU)

Diagnostic role of CXCL13 biomarker in primary Sjogren's syndrome patients with parotid non-Hodgkin's lymphoma complication / Papel diagnóstico del biomarcador CXCL13 en pacientes con síndrome de Sjogren primario con complicación de linfoma no Hodgkin parotídeo

Introduction Serum biomarkers are important predictive factors for development of parotid non-Hodgkin's lymphoma (NHL) complication in primary Sjogren's syndrome (pSS) patients. The aim was to evaluate the diagnostic accuracy of serum CXCL13 chemokine in pSS patients with parotid NHL complication. Material and methods Serum CXCL13 chemokine was assessed in 33 patients with pSS [7 with parotid NHL complication (pSS+NHL subgroup) and 26 without NHL (pSS-NHL subgroup)] and 30 healthy subjects. Results The serum CXCL13 levels in pSS+NHL subgroup [175.2 (107.9–220.4) pg/ml] were significantly higher comparing to the healthy subjects group (p=0.018) and the pSS-NHL subgroup (p=0.048). A cut-off value of 123.45pg/ml (Se=71.4%, Sp=80.8%, AUROC=0.747) was established for parotid lymphoma diagnosis. Conclusion The serum CXCL13 biomarker could be considered a valuable tool for the diagnosis of parotid NHL complication in pSS patients (AU)

Introducción Los biomarcadores séricos son factores predictivos importantes para el desarrollo de complicaciones del linfoma no Hodgkin (LNH) parotídeo en pacientes con síndrome de Sjogren primario (SSp). El objetivo fue evaluar la precisión diagnóstica de la quimiocina sérica CXCL13 en pacientes con SSp con complicación del LNH parotídeo. Material y métodos Se evaluó la quimiocina sérica CXCL13 en 33 pacientes con SSp [7 con complicación de LNH parotídeo (subgrupo SSp+LNH) y 26 sin LNH (subgrupo SSp-LNH)] y 30 sujetos sanos. Resultados Los niveles séricos de CXCL13 en el subgrupo pSS+NHL [175,2 (107,9-220,4) pg/ml] fueron significativamente más altos en comparación con el grupo de sujetos sanos (p=0,018) y el subgrupo pSS-NHL (p=0,048). Se estableció un valor de corte de 123,45 pg/ml (Se=71,4%, Sp=80,8%, AUROC=0,747) para el diagnóstico de linfoma de parótida. Conclusión El biomarcador sérico CXCL13 podría considerarse una herramienta valiosa para el diagnóstico de la complicación del LNH parotídeo en pacientes con SSp (AU)

Linfocele persistente tras tratamiento de isquemia aguda en paciente con bloqueo linfático tras radioterapia / Persistent lymphocele after treatment of acute ischemia in a patient with lymphatic blockade after radiation therapy

Introducción: los linfoceles son una complicación poco frecuente en cirugía vascular, pero pueden representarun gran riesgo en pacientes con material protésico. Caso clínico: presentamos el caso de un varón de 82 años sometido hace 43 a radioterapia por un linfoma noHodgkin que presenta un gran linfocele en contacto con material protésico tras una intervención de revasculari-zación urgente. Se realiza tratamiento escleroterápico con espuma de polidocanol al 3% sin conseguir resultadosatisfactorio, por lo que se decide la sustitución del material protésico por autólogo y mioplastia de sartorio. Discusión: a pesar del fracaso con la escleroterapia de la cavidad, en gran medida debido al gran bloqueo linfáti-co crónico, consideramos esta opción la de elección en casos de linfocele por su baja complejidad, invasividad yexcelente relación riesgo/beneficio.(AU)

Background: lymphoceles are a rare complication in vascular surgery but can represent a great risk in patientswith prosthetic material. Case report: we present the case of an 82-year-old man who underwent radiotherapy 43 years ago for non-Hodg-kin lymphoma who presented a large lymphocele in contact with prosthetic material after an urgent revasculari-zation. Sclerotherapy treatment with 3% polidocanol foam was performed without achieving satisfactory resultsfor what we chose replace the prosthetic with autologous material and sartorius myoplasty. Discussion: despite the failure with sclerotherapy of the cavity, largely due to the great chronic lymphatic blockage,we consider this option the choice in cases of lymphocele due to its low complexity, invasiveness and excellentrisk/benefit ratio..(AU)

Risk and prognosis of secondary breast cancer after radiation therapy for non-Hodgkin lymphoma: a massive population-based analysis

Purpose One of the best ways to control non-Hodgkin lymphoma (NHL) locally is radiation therapy (RT), which is a crucial component of care for many patients. There has not been any research on the risk and prognosis of secondary breast cancer (SBC) in females with NHL receiving RT. Methods In our study, females with NHL as their initial cancer diagnosis were included from 1975 to 2018 in the Surveillance, Epidemiology and End Results (SEER) database. Using Fine and Gray's competing risk regression assess the cumulative incidence of SBC. The standardized incidence ratios (SIR) and radiation-attributed risk (RR) for SBC were assessed using Poisson regression analysis. We evaluated the overall survival (OS) of SBC patients using the Kaplan–Meier technique. Results Of the 41,983 females with NHL, 10,070 received RT and 320 (3.18%) developed SBC. 31,913 females did not receive RT and 805 (2.52%) developed SBC. RT was significantly related with a greater chance of acquiring SBC in the Fine-Gray competing risk regression (adjusted hazard ratios (HR) = 1.14; 95% confidence intervals (CI), 1.09–1.30; P = 0.011). When an NHL diagnosis was made at an older age, the dynamic SIR and RR for SBC also declined over time. Regarding general survivability, there was not statistically significant (P = 0.970) after propensity score matching (PSM). Conclusions RT is an independent risk factor for SBC in females with NHL. Special attention should be paid to the monitoring of breast cancer indicators in them, especially young (AU)

Linfoma primario de base de cráneo presentándose como oftalmoplejia aguda / Primary skull base lynphoma presenting as acute ophtalmoplegia

Primary Skull base lymphoma (PSBL) represents a rare manifestation of extranodal lymphoma. Aggressive non-Hodgkin´s lymphoma such as diffuse large B-cell lymphoma, constitute the most commonly encountered subtype of PBSL. We report the case of a 70-year-oldwoman with acute diplopia and ptosis of the right eye. Neurological examination showed palsy of right III, IV and VI cranial nerves. Brain imaging studies showed a lesion showed invading the floor of the sella turcica, both cavernous sinuses, the clivus and part of the sphenoid sinus. The patient underwent endonasal endoscopic resection of the lesion. The histological diagnosis was diffuse large B-cell lymphoma. No systemic disease was found on staging the patient. (AU)

El linfoma primario de base de cráneo (LPBC) representa una variante poco frecuente del linfoma extranodal. Los linfomas no-Hodgkin agresivos, tales como el linfoma difuso de células grandes B son el subtipo más frecuente que se presentan como LPBC. Describimos el caso de una paciente mujer de 70 años que acude a nuestro centro con una clínica aguda de diplopía y posterior ptosis del ojo derecho. En la exploración neurológica se constató parálisis de III, IV y VI pares derechos. Los estudios de imagen cerebral mostraron una lesión que invadía el suelo de la silla turca, ambos senos cavernosos, el clivus y parte del seno esfenoidal. Se realizó una exéresis endoscópica endonasal de la lesión. El examen histológico de la lesión fue compatible con un linfoma difuso de células grandes B. No se constató enfermedad sistémica en los estudios de extensión. (AU)

Primary central nervous system Hodgkin lymphoma: A case report and review of the literature / Linfoma de Hodgkin primario del sistema nervioso central. Presentación de un caso y revisión de la literatura

Central nervous system (CNS) involvement in the context of relapsed/refractory Hodgkin lymphoma (HL) is a quite rare, but well-known complication. Nevertheless, primary CNS–HL is an exceedingly rare condition, which diagnosis is based on well-defined morphological and immunohistochemical features, in addition to isolated involvement of the CNS. In spite of limited casuistry (just over twenty cases reported in the literature), available data agree that primary and isolated CNS–HL, when treated with a combination of surgery followed by some form of adjuvant therapy (radiotherapy±chemotherapy), carries a better prognosis than those cases with CNS involvement in the context of relapsed/refractory HL or those with CNS non-Hodgkin lymphoma. We herein report a case of a 55-year-old female patient who was diagnosed with primary CNS–HL. The patient was treated with complete surgical resection followed by intrathecal chemotherapy and whole brain radiotherapy (WBRT), showing fourteen months of disease-free survival at the time of this case report. A review of the available literature is also presented (AU)

La afectación del sistema nervioso central (SNC) en pacientes con diagnóstico de linfoma de Hodgkin (LH) sistémico es una complicación muy poco frecuente, aunque bien definida. Sin embargo, el LH primario del SNC es una entidad extremadamente rara, cuyo diagnóstico precisa la identificación de un patrón morfológico e inmunohistoquímico específico, así como la afectación aislada del SNC. Pese a contar con una casuística muy limitada (apenas una veintena de casos publicados en la literatura) la bibliografía disponible coincide en que el LH con afectación primaria y aislada del SNC, cuando es tratado con cirugía y tratamiento adyuvante (radioterapia±quimioterapia), parece tener un mejor pronóstico que aquellos casos en los que la afectación del SNC se produce en el contexto de un LH sistémico o en el contexto de un linfoma no Hodgkin. En este artículo se presenta el caso de una mujer de 55 años con diagnóstico histopatológico de LH primario del SNC. La paciente fue sometida a una exéresis completa de la lesión y a tratamiento adyuvante con quimioterapia intratecal y radioterapia holocraneal, con una supervivencia libre de enfermedad hasta la fecha de 14 meses. Se presenta asimismo, la revisión de la literatura disponible (AU)

Anaplastic large cell lymphoma of breast masquerading suppurative mastitis on cytology / Linfoma anaplásico de células grandes de mama enmascarando mastitis supurativa en citología

Breast is an uncommon site of extra nodal lymphoma accounting for 2.2% of all extra nodal lymphomas. B cell lymphomas are more common than T cell types. The most common subtype is Diffuse Large B Cell Lymphoma. Breast lymphoma is very challenging to diagnose as it has nonspecific clinical and radiological features. We report a rare case of primary T cell lymphoma in a 20-year-old female. It was misdiagnosed as abscess on ultrasound and as suppurative mastitis on fine needle aspiration cytology (FNAC). However, excisional biopsy was suggestive of lymphoma and immunohistochemistry confirmed it as ALK positive, anaplastic large cell lymphoma. Even though FNAC is a part of the diagnostic triad for breast lesions, potential pitfalls cannot be overlooked and must be correlated with biopsy. (AU)

La mama es un sitio poco frecuente de linfoma extraganglionar que representa el 2,2 % de todos los linfomas extraganglionares. Los linfomas de células B son más comunes que los tipos de células T. El subtipo más común es el linfoma difuso de células B grandes. El linfoma de mama es muy difícil de diagnosticar, ya que tiene características clínicas y radiológicas inespecíficas. Presentamos un caso raro de linfoma primario de células T en una mujer de 20 años. Fue mal diagnosticado como absceso en ultrasonido y como mastitis supurativa en citología por aspiración con aguja fina (FNAC). Sin embargo, la biopsia por escisión sugirió linfoma y la inmunohistoquímica lo confirmó como linfoma anaplásico de células grandes ALK positivo. Aunque la FNAC es parte de la tríada diagnóstica de las lesiones mamarias, no se pueden pasar por alto los peligros potenciales y se deben correlacionar con la biopsia. (AU)

HIV-related non-Hodgkin lymphomas affecting the oral cavity: a clinicopathologic study of 11 cases

Background: HIV-related non-Hodgkin lymphomas of the oral cavity are rare lesions with aggressive clinical behaviour. The aim of this study is to describe the clinicopathological features of a series of HIV-related oral non-Hodgkin lymphomas. Material and Methods: Eleven cases of oral lymphomas affecting HIV-positive patients were retrieved from 2012 to 2019. Clinicopathological features regarding age, sex, tumour location, clinical presentation, laboratory findings, disease stage and follow-up were obtained. Histologic, immunohistochemical and in situ hybridization for EBV detection were done for diagnosis confirmation. Overall survival was estimated by Kaplan–Meier curve. Results: Males predominated, with a mean age of 40.3 years-old. Maxilla and mandible were the mostly affected. Plasmablastic lymphoma and diffuse large B-cell lymphoma not otherwise specified (NOS) were the main histological types. Lesions presented as reddish ulcerated swellings, representing the first sign of AIDS in six cases. Stage IV were common (7 cases) and the mean HIV viral load was 10,557 copies/mL, with a mean of 266 CD4+ cells/mm3, 1,278 CD8+ cells/mm3 and a CD4+/CD8+ ratio of 0.26. Eight patients died of the disease (72.7%). Overall survival revealed that 78.2% of the patients died after 21 months of follow-up. Conclusions: HIV-related oral lymphomas present a poor prognosis usually diagnosed in advanced stages and in our series plasmablastic lymphoma was the most common subtype.(AU)

Stance of MRD in Non-Hodgkin’s Lymphoma and its upsurge in the novel era of cell-free DNA

Cancer genomics has evolved over the years from understanding the pathogenesis of cancer to screening the future possibilities of cancer occurrence. Understanding the genetic profile of tumors holds a prognostic as well as a predictive value in this era of therapeutic surveillance, molecular remission, and precision medicine. Identifying molecular markers in tumors is the current standard of approach, and requires an efficient combination of an accessible sample type and a profoundly sensitive technique. Liquid biopsy or cell-free DNA has evolved as a novel sample type with promising results in recent years. Although cell-free DNA has significant role in various cancer types, this review focuses on its application in Non-Hodgkin’s Lymphoma. Beginning with the current concept and clinical relevance of minimal residual disease in Non-Hodgkin’s lymphoma, we discuss the literature on circulating DNA and its evolving application in the realm of cutting-edge technology (AU)

Actualización en el diagnóstico, pronóstico y tratamiento del linfoma folicular / Follicular lymphoma: an update on diagnosis, prognosis, and management

El linfoma folicular, el linfoma indolente más frecuente, se origina en los linfocitos B del centro germinal del folículo linfoide y se caracteriza por la traslocación t(14;18). Clínicamente se manifiesta por la presencia de adenopatías, que pueden asociarse a síntomas constitucionales o citopenias. El diagnóstico se establece mediante la observación de una proliferación linfoide B de patrón nodular en la biopsia ganglionar, y la estadificación mediante una PET-TC y una biopsia de médula ósea. Una proporción significativa de pacientes no requiere tratamiento inmediato. Si lo requieren, suele estar basado en inmunoterapia anti-CD20 asociada o no a quimioterapia. A pesar de que la mayoría de los pacientes presentan una supervivencia global prolongada, las recaídas son muy frecuentes, y la recaída precoz y la transformación a un linfoma agresivo confieren un mucho peor pronóstico. Se encuentran en desarrollo nuevos tratamientos que cambiarán las perspectivas de estos pacientes en un futuro próximo. (AU)

Follicular lymphoma, the most common indolent lymphoma, originates from germinal centre B-cells of the lymphoid follicle, and is characterized by t(14;18). Clinical manifestations include the presence of lymphadenopathy, sometimes accompanied by constitutional symptoms or cytopenia. Diagnosis is established through the identification of a B-cell proliferation of nodular pattern in the lymph node biopsy. Upon staging with PET-CT and bone marrow biopsy, a significant proportion of patients do not need immediate treatment. When therapy is indicated, commonly used regimens include anti-CD20 immunotherapy with or without chemotherapy. Although overall survival for most patients is prolonged, relapses are very frequent, and early relapse and transformation to an aggressive lymphoma portend a much worse prognosis. New therapies are under development, which will most likely change outcomes for FL patients in the near future. (AU)

Characterization of a xenograft model for anti-CD19 CAR T cell studies

Purpose Chimeric antigen receptor (CAR) T cell development for B cell malignancies treatment has triggered a paradigm shift in oncology. The development of anti-CD19 CAR T cells relies primarily on a panel of cell line-derived xenograft models, including Raji cells; however, the behavior of this model is under debate. We attempted to characterize this lymphoma model and propose outcome measures for CAR T cell studies Methods Raji cell line was inoculated into NOG mice via intra-venous (IV), intra-peritoneal (IP), and subcutaneous (SC) routes with different inoculum sizes, and consequent clinical and histopathological outcomes were assessed. Results Inoculum sizes of 105–106 resulted in a complete take rate. The mice with IV and SC-inoculated Raji cells presented the shortest and longest survival among lymphoma-bearing mice, respectively (P < 0.01). The IP group had the highest number of both infiltrated organs (P < 0.05; compared to SC) and involvement of lymphatic sites (P < 0.05; compared to IV). The number of lymphoma lesions on the liver was higher in the IV compared to IP (P < 0.001) and SC (P < 0.05). Conclusion We demonstrate that the Raji cell line inoculation route could determine the xenograft model system behavior in terms of survival, tumor burden, and dissemination pattern and gives the model the specific features suitable for testing the specific hypothesis in CAR T cell therapy. We also conclude outcome measures for CAR T cell studies that do not require imaging techniques (AU)