Linfoma no Hodgkin primario de vagina o cuello uterino. Un diagnóstico en ocasiones incierto: a propósito de un caso / Primary non-Hodgkin´s lymphoma of vagina or cervix. A diagnosis sometimes uncertain: about a case

Resumen ANTECEDENTES: La afectación primaria del aparato genital femenino por un linfoma no Hodgkin es excepcional, se reporta en 0.2 a 1.1% de los casos. Los órganos afectados con más frecuencia son los ovarios, seguidos del cuello uterino; el endometrio y la vagina son los menos aquejados. Debido a su baja frecuencia aún no se llega a un consenso del tratamiento más adecuado de estos linfomas; hasta ahora, por lo general, se ha individualizado para cada caso en concreto. CASO CLÍNICO: Paciente de 29 años que consultó por sangrado genital anómalo e incapacidad para la inserción de tampones vaginales. En la exploración se encontró una masa pélvica, palpable a través de la vagina, que impresionaba al infiltrar toda la luz vaginal y la parte media e izquierda de la vulva. En los estudios de imagen se objetivó una gran masa pélvica de hasta 10 centímetros que parecía depender del cuello del útero y que se extendía e infiltraba el canal vaginal, la vulva y el tercio inferior de la vejiga. El reporte anatomopatológico de la biopsia fue: infiltración de pared vaginal por un linfoma no Hodgkin B difuso de células grandes. Se le indicaron seis ciclos de quimioterapia con ciclofosfamida, vincristina, adriamicina y prednisona con los que se consiguió la remisión metabólica completa. CONCLUSIÓN: El diagnóstico del linfoma genital primario puede resultar complejo por la posibilidad de simular una neoplasia ginecológica. En casos de enfermedad avanzada, la manifestación clínica más frecuente es el sangrado genital anómalo. El esquema de tratamiento más aceptado en la actualidad es con rituximab-ciclofosfamida, vincristina, adriamicina, prednisona seguido de radioterapia de consolidación.

Abstract BACKGROUND: Primary involvement of the female genital tract by non-Hodgkin's lymphoma is exceptional, reported in 0.2 to 1.1% of cases. The most frequently affected organs are the ovaries, followed by the cervix; the endometrium and vagina are the least affected. Due to their low frequency, there is still no consensus on the most appropriate treatment of these lymphomas; until now, it has generally been individualized for each specific case. CLINICAL CASE: A 29-year-old female patient consulted for abnormal genital bleeding and inability to insert vaginal tampons. On examination a pelvic mass was found, palpable through the vagina, which impressed by infiltrating the entire vaginal lumen and the middle and left side of the vulva. Imaging studies showed a large pelvic mass of up to 10 centimeters that appeared to be dependent on the cervix and that extended and infiltrated the vaginal canal, the vulva and the lower third of the bladder. The anatomopathological report of the biopsy was: infiltration of the vaginal wall by diffuse large cell non-Hodgkin's B lymphoma. She was prescribed six cycles of chemotherapy with cyclophosphamide, vincristine, adriamycin and prednisone with which complete metabolic remission was achieved. CONCLUSION: The diagnosis of primary genital lymphoma can be complex because of the possibility of simulating a gynecologic neoplasm. In cases of advanced disease, the most frequent clinical manifestation is abnormal genital bleeding. The most accepted treatment scheme at present is rituximab-cyclophosphamide, vincristine, adriamycin, prednisone followed by consolidation radiotherapy.

Linfomas extranodales de cabeza y cuello / Extranodal lymphomas of the head and neck

RESUMEN Antecedentes: los linfomas extranodales de cabeza y cuello (LECC) son neoplasias infrecuentes. El papel diagnóstico de la cirugía justifica considerar estas entidades entre los diagnósticos diferenciales de la patología quirúrgica de la región. Objetivo: describir las características clínico-patológicas, la localización y la asociación con enfermeda des sistémicas de una serie de pacientes operados con LECC. Material y métodos: se realizó una revisión retrospectiva de las historias clínicas de 5489 pacientes operados entre junio de 2009 y junio de 2019. Diecinueve de ellos (0,35%) presentaron LECC. Resultados: el promedio de edad fue de 56 años (rango 17- 84, DS 19,3), 16 fueron mujeres (84%). La localización de los LECC fue: glándula parótida en 12 casos (63%), glándula submaxilar en 3 casos (16%), glándula salival menor en 2 casos (11%), glándula lagrimal en 1 caso (5%), glándula tiroides en 1 caso (5%). Todos fueron linfomas no Hodgkin. Los tipos citológicos fueron: linfoma de tejido linfoideo asociado a mucosas (MALT) 9 (48%), linfoma folicular 4 (21%), linfoma de células B grandes 3 (16%), linfoma de células pequeñas 1 (5%), linfoma del manto 1 (5%), linfoma anaplásico 1 (5%). Cuatro pa cientes (21%) presentaron síndrome de Sjögren en el momento del diagnóstico, tres linfomas MALT y uno linfoma folicular. Diferentes procedimientos quirúrgicos permitieron llegar al diagnóstico. Conclusión: los LECC constituyen una forma singular de presentación de neoplasias oncohematológica. En la presente serie se manifestaron en un amplio rango de edad, con mayor frecuencia de mujeres y en la glándula parótida. Su diagnóstico pudo alcanzarse con la biopsia o resección de la zona afectada.

ABSTRACT Background: Extranodal lymphomas of the head and neck (ELHN) are rare neoplasms. The role of surgery for the diagnosis justifies thinking of these neoplasms among the differential diagnoses of the surgical disorders of the region. Objective: The aim of this study was to describe the clinical and pathological characteristics of ELHN, their location and association with systemic diseases in a series of patients undergoing surgery. Material and methods: The clinical records of 5489 patients undergoing surgery between June 2009 and June 2019 were retrospectively reviewed. Nineteen patients (0.35%) presented ELHN. Results: Mean age was 56 years (range: 17-84; SD 19.3) and 84% (n = 16) were women. The location of ELHN were the parotid gland (n =12, 63%), submaxillary gland (n = 3, 16%), salivary gland (n = 1, 5%), lacrimal gland (n = 1, 5%) and thyroid gland (n =1, 5%). All the tumors were non-Hodgkin lymphomas with the following subtypes: mucosa associated lymphoid tissue (MALT) lymphoma (n = 9, 48%), follicular lymphoma (n = 4, 21%), large B-cell lymphoma (n = 3, 16%), small B-cell lymphoma (n = 1, 5%), mantle cell lymphoma (n = 1, 5%), and anaplastic lymphoma (n =1, 5%). Four patients (21%) presented Sjögren syndrome at the moment of the diagnosis; three were MALT lymphomas and one patient had follicular lymphoma. The diagnosis was made with different surgical procedures. Conclusion: ELHN constitute a singular presentation of onco-hematology neoplasms. In our series, these tumors occurred within a wide age range and were more common in women. The parotid gland was more commonly affected. The diagnosis was made by surgical biopsy or resection of the area affected.

Oral Mucosal Diffuse Large B-cell Lymphoma Caused by Long-term Oral Methotrexate:Report of One Case / 中国医学科学院学报

A case of primary oral mucosal diffuse large B-cell lymphoma(DLBCL)due to long-term use of methotrexate(MTX)for the treatment of rheumatoid arthritis(RA)was admitted to the Department of Hematology,Fujian Medical University Union Hospital.We analyzed and discussed the clinical features,diagnosis and treatment,and prognosis of specific malignant lymphoma induced by MTX in this RA patient.Our purpose is to improve the awareness and knowledge of other iatrogenic immunodeficiency-associated lymphoproliferative disorders of clinicians and pathologists.This study provides a new reference for the clinical diagnosis and treatment of MTX-associated DLBCL.

Clinical characteristics of primary extranodal versus nodal diffuse large B-Cell Lymphoma: A retrospective cohort study in a cancer center

Background The outcome of patients with primary extranodal diffuse large B-cell lymphoma (PE-DLBCL) varies according to the primary site involved. Primary gastrointestinal, breast, bone, craniofacial, and testicular DLBCL are rare extranodal manifestations of DLBCL. Objective The objective of the study was to describe the clinical course of patients with PE-DLBCL disease in a referral cancer center. Results From 637 patients, 51 (8.77%) were considered as having PE-DLBCL (25 gastrointestinal, 12 craniofacial, 6 breast, 5 bone, and 3 with primary testicular DLBCL). Complete remission was higher in all PE-DLBCL sites (100% in testicular, 92.6% craniofacial, 83.3% breast, 80% bone, and 80% gastrointestinal) compared with 73.3% in nodal DLBCL. Although 2 cases with breast PE-DLBC relapsed, they achieved a complete response with chemotherapy. The overall survival at 5 years was 100%, 80%, 78%, 58%, 58%, and 62% for patients with primary breast, primary bone, gastrointestinal, primary craniofacial, primary testicular, and nodal DLBCL, respectively. Conclusions PE-DLBCLs constitute rare, primary sites of lymphoproliferative disorders in most cases, with localized disease and good prognosis. They require a combined chemoimmunotherapy with radiotherapy in most cases to improve local and systemic disease.

SMILE en linfoma de células NK/T extranodal en recaída / SMILE in relapsed extranodal NK / T cell lymphoma

Introducción: El linfoma extranodal de células NK/T, tipo nasal es una variedad agresiva que se presenta con mayor frecuencia en Asia Oriental y América Latina. El régimen de tratamiento SMILE, basado en dexametasona, metotrexato, ifosfamida, L-asparaginasa y etopósido, mostró tasas de respuestas objetivas adecuadas en estudios de fase 1/2. Objetivo: presentar el primer reporte de la aplicación del protocolo SMILE en Cuba. Presentación del caso: paciente de 47 años de edad, blanca que presentó recaída temprana de linfoma extranodal de células NK/T tipo nasal en partes blandas de la pared anterior izquierda del tórax. Recibió quimioterapia SMILE por 4 ciclos y en la evaluación al final del tratamiento se comprobó que hubo una respuesta completa de la enfermedad(AU)

Introduction: Extranodal lymphoma of NK / T cells, nasal type is an aggressive variety that occurs most frequently in East Asia and Latin America. The SMILE treatment regimen, based on dexamethasone, methotrexate, ifosfamide, L-asparaginase and etoposide, showed adequate objective response rates in phase 1/2 studies. Objective: to present the first report of the application of the SMILE protocol in Cuba. Case presentation : 47-year-old white patient who presented early relapse of extranodal lymphoma of nasal NK / T cells in soft parts of the left anterior wall of the thorax. He received SMILE chemotherapy for 4 cycles and the evaluation at the end of the treatment showed that there was a complete response of the disease(AU)

Prognosis analysis of primary breast diffuse large B cell lymphoma / 中华肿瘤杂志

Objective@#To investigate the clinical characteristics, therapy modality and prognosis of primary breast diffuse large B-cell lymphoma(PB-DLBCL).@*Methods@#A total of 68 patients with PB-DLBCL treated in Tianjin Medical University Cancer Institute and Hospital were enrolled between January 1, 2004 and January 31, 2017. Clinicopathological data were retrospectively analyzed. 67 patients were female and only one male. The median age was 56 years old. 46 patients had Ann Arbor clinical stageⅠ~Ⅱ disease, and the other 22 were stage Ⅲ~Ⅳ. The patients with and without B symptom were 11 and 57, respectively. Kaplan-Meier method was used for univariate analysis to calculate the 5-year overall survival (OS) rate and 5-year progress-free survival (PFS) rate, compared using the log rank test. Cox regression analysis was used for multivariate analysis.@*Results@#The 1, 3, 5-year OS rate were 84.0%, 78.0% and 73.0%, and 1, 3, 5-year PFS rate were 80.0%, 71.0% and 51.0%, respectively. Univariate analysis indicated that eastern cooperative oncology group (ECOG) score, Ann Arbor clinical stage, international prognostic index (IPI) score, risk stratification, B symptom, β2-microglobulin(β2-MG) level, size of the tumor and cycles of chemotherapy were prognostic factors for OS (all P<0.05), and Ann Arbor clinical stage, IPI score, risk stratification and B symptom were prognostic factors for PFS (all P<0.05). Multivariate analysis indicated that Ann Arbor clinical stage was independent prognostic factor for OS(P=0.029) and B symptom was independent prognostic factor for PFS(P=0.028).@*Conclusions@#Prognosis of PB-DLBCL was relatively good. Ann Arbor clinical stage and B symptom were independent prognostic factors for OS and PFS, respectively.

Concurrent nodal and extranodal lymphoma misdiagnosed as metastatic gingival carcinoma: A case report / 中南大学学报(医学版)

Early diagnosis of malignant lymphoma is often difficult since the clinical manifestation of the lymphoma occurred in the maxillofacial region is very similar to that of the squamous cell carcinoma.When the pathological diagnosis is not clear,the surgeon is easy to misdiagnose and lead to mistreatment.A patient visited the Affiliated Haikou Hospital of Xiangya School of Medicine,Central South Universily for gingival mass with an ipsilateral submaxillary enlargement.The clinical manifestation and preoperative MRI are very prone to squamous cell carcinoma with metastasis,so we did not take a preoperative pathological examination.The gingival mass was surgical removed firstly,but frozen pathological result showed that it was malignant small round cell tumor.Since the patient was diagnosed as high degree malignant of small round cell tumor and the submandibular region have been significantly metastasized,so we carried out the combined radical dissection of gingival,mandible and neck surgery.The postoperative pathological report was malignant lymphoma,suggesting that the patient was a case of misdiagnosis and mistreatment.This article draws lessons from misdiagnosis and provided experience for seeking improvement measures.

The value of imaging diagnosis in primary extranodal lymphoma / 实用放射学杂志

Objective To summarize the imaging features of the primary extranodal lymphoma (PENL)and evaluate the diagnos-tic value.Methods The clinical manifestations,imaging findings and pathological types of 50 patients with PENL were analyzed retrospectively.Results Fifty patients of PENL included 4 cases in orbit (8.0%),1 in middle ear and mastoid (2.0%),5 in thyroid (10.0%),2 in breast (4.0%),8 in lung (16.0%),5 in stomach (10.0%),15 in intestine (30.0%),1 in adrenal gland (2.0%),5 in testis (10.0%),2 in prostate (4.0%)and 2 in soft tissue of shoulder and hip (4.0%).In all patients,the nodular type was 40% (20/50),diffuse type was 34% (17/50)and massive type was 26% (13/50).Most lesions were regular (54%,27/50)and had clear margin (94%, 47/50).26 cases (52%)were non-Hodgkin's lymphoma with diffuse large B cell type.Conclusion The imaging findings of PENL are various and nonspecific,which may be easily misdiagnosed.Various imaging methods have different sensitivity for the diagnosis of lymphoma in different sites.

Application of 18F-FDG PET-CT in detection and accurate staging of extranodal non-Hodgkin lymphoma / 白血病·淋巴瘤

Objective To investigate the value of 18F-FDG PET-CT in detection and accurate staging of extranodal non-Hodgkin lymphoma (NHL).Methods The results of PET-CT of 94 patients with NHL were retrospectively analyzed.The consistency of checking out lesions and accurate staging by PET-CT were compared with those by other imaging examination in extranodal NHL.Results 432 lesions were checked out by PET-CT, including 319 (73.8 ％) lymphoid tissues and organs with the average SUVmax of 13.4 (3.4-33.4), and 113 (26.2 ％) extranodal lesions with the average SUVmax of 13.5 (3.1-55.0).The detection consistent rate between CT and PET-CT for lymphoid tissues and lymph organ lesions was 95 ％, while the consistent rate of the extranodal lesions was only 54.9 ％.The detection rates of PET-CT for soft tissue, bone and gastrointestinal lesions were higher than those of CT, but the detection rate for the bone marrow lesion was lower than that for the bone marrow cytology.According to the results of PET-CT, the stages of 29 patients (31.0 ％) were re-adjusted, including up-regulated for 75.9 ％ (22/29) because of high detection rates of PET-CT for soft tissue and skeletal lesions, and down-regulated for 24.1％ (7/29) mainly due to the strong resolution capability of PET-CT for detection of non-neoplastic lymph nodes and spleen increasing or effusion.Conclusion 18F-FDG PET-CT can improve the detection rate of NHL extranodal lesions, especially for diffuse non-mass lesions in bone and soft tissues, which facilitates the accurate lymphoma staging.

Linfoma no Hodgkin primario de vagina / Primary non Hodgkin's lymphoma of the vagina

La afectación primaria del tracto genital femenino de los linfomas no Hodgkin es poco frecuente (2 por ciento de los linfomas primarios extraganglionares). Los órganos más afectados son los ovarios seguidos del cérvix, siendo la localización endometrial y vaginal extremadamente rara. Presentamos el caso de una paciente de 44 años diagnosticada de linfoma primario de vagina, estadio IE A, con remisión completa tras tratamiento combinado quimioterápico y quirúrgico. Es importante tener presente estas raras aunque posibles localizaciones de linfomas para evitar que se puedan confundir con lesiones inflamatorias u otros tipos de tumores que nos lleven a un fracaso terapéutico.

Primary non-Hodgkin lymphomas rarely involve the female genital tract (2 percent of primary extranodal lym-phomas). The ovaries followed by the cervix are the most common affected sites while the involvement of the endometrium and vagina is extremely rare. We report the case of a 44 year old woman with a primary lymphoma of the vagina, stage IE A, with complete remission after combined chemotherapy and surgical treatment. These rare but possible locations must be kept in mind to avoid misdiagnosis with inflammatory lesions or other types of tumors which may lead us to a therapeutic failure.

Addition of rituximab to the CHOP regimen has no benefit in patients with primary extranodal diffuse large B-cell lymphoma

BACKGROUND: The addition of rituximab to cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) chemotherapy (R-CHOP) has significantly improved clinical outcomes for patients with diffuse large B-cell lymphoma (DLBCL). However, new predictors of patient response to R-CHOP have not been established. We aimed to evaluate the impact of R-CHOP compared with CHOP in patients with DLBCL and to establish clinical predictors of better outcomes in these patients. METHODS: We retrospectively identified 177 patients diagnosed with CD20-positive DLBCL and treated with CHOP (N=82) or R-CHOP (N=95). The response rate, event-free survival (EFS), and overall survival (OS) rates were compared between the 2 treatment groups. All patients were classified into primary extranodal lymphoma (PENL) or nodal lymphoma (NL) subgroups, and the clinical parameters of each subgroup were analyzed. RESULTS: The overall response rate was higher in R-CHOP group (95% vs. 84%, P=0.07). The 3-year EFS rate was significantly higher in R-CHOP group (71% vs. 52%, P=0.013), but the OS rate was comparable between the 2 groups (79% vs. 69%, P=0.23). A significant survival benefit was seen with R-CHOP compared to CHOP therapy in NL patients (P=0.002 for EFS and 0.04 for OS). Multivariate analyses confirmed that R-CHOP therapy is an independent prognostic factor for EFS (hazard ratio of 0.32 [0.17-0.62], P=0.001) and OS (hazard ratio of 0.4 [0.18-0.87], P=0.02) in NL patients. CONCLUSION: Patients in the PENL group did not benefit from R-CHOP chemotherapy.

Linfoma Extranodal de Células NK/T tipo Nasal / Extranodal Nasal type NK/T-Cell Lymphoma / Extranodal Nasal type NK/T-Cell Lymphoma

O Linfoma Extranodal de Células NK/T tipo Nasal tem uma distribuição geográfica peculiar, ocorrendo mais frequentemente em países orientais e na população nativa de alguns países da América Central e da América do Sul. Sua localização preferencial é na cavidade nasal e nos seios paranasais, mas pode acometer outras estruturas da chamada região médio-facial. Tem um padrão de disseminação com "homing" característico, incluindo pele, testículo, SNC e trato digestivo. Este linfoma, menos frequentemente, pode acometer primariamente estas regiões. A maioria destas neoplasias apresenta um fenótipo NK, mas alguns poucos casos podem ter sua origem em células T verdadeiras, por este motivo é designado "linfoma NK/T". O genoma do vírus Epstein-Barr é detectado na maioria dos casos, sugerindo uma relação etiológica. Embora este linfoma seja sensível à radioterapia, apresenta mais frequentemente resistência a agentes quimioterápicos que outros linfomas. Uma possível explicação para a resistência é a usual expressão de glicoproteína-p. O prognóstico destes linfomas é pobre, sendo necessária a investigação de novas modalidades terapêuticas.

Extranodal Nasal type NK/T-Cell Lymphoma has a peculiar geographic distribution, occurring more frequently in Eastern countries and in the native populations of some Central and South American countries. It is commonly found in the nasal cavity and paranasal sinuses, but may also compromise other structures in the mid-facial region. The disease has a characteristic homing dissemination pattern, including skin, testis, CNS and digestive tract. This lymphoma can, less frequently, primarily compromise these regions. The majority of these neoplastic diseases present an NK phenotype, but a few cases can be truly of T-cell origin, because of which it is designed "NK/T-cell lymphoma". The Epstein-Barr virus genome can be detected in most of the cases, suggesting an etiological relationship. Although this lymphoma is responsive to radiotherapy, it is more resistant to conventional chemotherapy than other lymphomas. A possible explanation for this is the frequent expression of the p-glycoprotein. The prognosis of these lymphomas is still poor, making further investigation of new therapies imperativ.

Clinical Features of 110 Cases of Primary Extranodal Non-Hodgkin's Lymphoma / 中国肿瘤临床

Objective: To evaluate the incidence, clinical features, diagnosis and treatment of primary ex-tranodal non-Hodgkin' s lymphoma (PE-NHL). Methods: The data of 110 patients diagnosed as PE-NHL be-tween January 2001 and May 2008 were reviewed. Results: These PE-NHL patients counted 60.11% of the 183 malignant lymphoma patients at the same period. The primary sites affected were the gastrointestinal tract 21.82% (24/110), Waldeyer ring 10.91% (12/110), nasal cavity 9.10% (10/110), soft tissue 9.10% (10/110), mediastinum 7.27% (8/110) and other unusual sites 41.82% (46/110). Symptoms and signs of PE-NHL were not specific, and 77.27% of these cases had a swelling organ or lump of the primary organ affected. Ac-cording to the International Prognosis Index (IPI), the percentage of patients in low, intermediate, and high group was 41.11%, 44.44% and 14.44%, respectively. Immunophenotype was assayed in 93 cases. The per-centage of B-cell lymphoma was 69.90% while that of T-cell lymphoma was 30.10%. For those 95 cases treat-ed, the effective rate including complete remission (61.05%) and part remission (16.84%) was 77.89%, the median survival was 30 months, and the 5-year overall survival (OS) was 27%. While, for patients with prima-ry gastrointestinal non-Hodgkin' s lymphomas (PGIL), the complete remission rate, part remission rate and the effective rate was 65.21%, 17.39% and 82.80%, respectively. The median survival was 24 months, and the 5-year overall survival (OS) was 30%. Conclusion: PE-NHL is more common than nodal lymphoma. The symptoms and signs of PE-NHL of different sites are quite different. To improve the curative strategies of PE-NHL, it is important to make an allround understanding of PE-NHL and follow reasonable mode of diagno-sis and therapy.

Experiencia de 10 años en linfomas oculares y de anexos oculares / Ten year experience with ocular lymphomas and ocular adnexae lymphomas

Antecedentes. Los linfomas oculares (LO) y de los anexos oculares (LAO) tienen una incidencia de 7- 8% de todos los tumores extraganglionares. Objetivo. Describir las características clínicas, morfológicas e inmunofenotípicas de los LO y LAO atendidos en el Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán. Material y métodos. Se revisaron los expedientes de los pacientes con diagnóstico de LO y LAO de julio de 1994 a julio del 2005. Se analizaron los siguientes datos: presentación clínica, tratamiento, respuesta al tratamiento, supervivencia global, y supervivencia libre de enfermedad (SVLE). Resultados. Se analizaron 10 pacientes, 8 mujeres y 2 hombres. La media de edad fue de 50 años. La remisión completa (RC) se presentó en 8 de 10 pacientes y en los 6 pacientes con linfoma de tejido linfoide asociado a mucosas (MALT). Se encontraron dos con enfermedad refractaria, los cuales estaban en estadio IV. Conclusiones. En este estudio los LO y LAO correspondieron a 0.02% de todos los linfomas no Hodgkin (LNH) estudiados (498 casos). Los linfomas tipo MALT se presentan a edad más avanzada, se encontraron en estadios más tempranos y en todos hubo RC, con una SVLE promedio de 868 días y una media de supervivencia de 442 días.

BACKGROUND: Ocular lymphomas and ocular adnexae lymphomas (OL and OAL) constitute 7-8% of all extranodal lymphomas. OBJECTIVE: Describe the clinical, morphologic and immunophenotypic characteristics of OAL seen in our hospital. MATERIAL AND METHODS: Retrospective analysis of patient records with OL and OAL between July 1994 and July 2005. The following data was analyzed: Clinical presentation, therapy, treatment response, overall survival and disease free survival. RESULTS: Ten patients with OL and OAL were identified. Of these, 8 were women and 2 men. Median age was 50. Eight of 10 patients achieved complete remission, 6 of the 6 presenting MALT Lymphoma. Two patients with stage IV had refractory disease. CONCLUSIONS: In our series 0.02% of lymphomas were OL and OAL of a total 498 LNH. MALT lymphomas appear at a more advanced age, sixty percent of the cases were MALT lymphomas and were diagnosed during their early stages. Patients were followed during 21 months, global survival was 100%, free illness survival had a mean of 868 days and a survival median of 442 days.

Linfoma no Hodgkin de células B con extensión secundaria a glándula mamaria: Evaluación por imagen / Secondary non-Hodgkin's B-cell lymphoma involving the breast: radiologic imaging

Los linfomas secundarios con afectación de la glándula mamaria, aunque raros, representan el grupo más grande de tumores metastásicos de la mama. El linfoma no Hodgkin primario de la mama es también raro, representando del 1.7%-2.2% de los casos de linfoma no Hodgkin extranodal y del 0.38-0.7% de todos los linfomas no Hodgkin. Aproximadamente 300 casos han sido reportados en la literatura. El aspecto mamográfico ha sido descrito como áreas redondeadas u ovales con densidad aumentada. Las masas mamarias pueden aparecer homogéneas o heterogéneas. Las lesiones pueden ser muy bien definidas y ser confundidas con procesos benignos, esto último más probable en pacientes menores de 35 años de edad. Los hallazgos ultrasonográficos son descritos como masas de márgenes bien definidos con ecos de intensidad baja a moderada. Las paredes posteriores de la masa suelen estar bien definidas, probablemente con ligero reforzamiento acústico pero sin sombra posterior asociada. En resumen, el aspecto mamográfico del linfoma mamario no es específico, pero el diagnóstico puede ser excluido si hay presencia de calcificaciones o reacción desmoplásica. El tejido linfoide prominente en un paciente con masas mamarias debería despertar la sospecha de linfoma mamario. El aspecto más crítico en el estudio de una masa de la mama es la biopsia del tejido, puesto que una lesión linfomatosa tiene radiográficamente patrones indeterminados. Presentamos el caso de una mujer de 79 años con dolor abdominal y una masa palpable en la mama derecha; realizamos un breve resumen de las características clínicas y principales hallazgos por imagen del linfoma no Hodgkin con afección a la glándula mamaria (radiografía de tórax, mastografía, ultrasonido mamario, y tomografía de tórax).

Secondary lymphomas involving the breast, although uncommon, represent the largest group of metastasic tumors to the breast. Primary non-Hodgkin lymphomas (NHLs) of the breast are also rare, accounting for 1.7% to 2.2% of extranodal NHL cases and 0.38 to 0.7% of all NHLs. Approximately 300 cases have been reported in the Medical literature. Mammographic appearances are described as round or oval areas of opacity. The breast mass may appear homogeneous or inhomogeneous. The lesion may be very well defined and may be mistaken for a benign process, most notable in patients younger than 35 years of age. Ultrasonographic appearance is described as a sharply defined mass with low or medium echoes. The posterior aspect of the mass is well defined (possibly with slight acoustic enhancement but with no associated posterior shadowing). In summary, the mammographic appearance of the breast lymphoma is nonspecific, but the diagnosis can possibly be excluded if calcifications or a desmoplastic reaction are present. Prominent lymph vessels in a patient with a breast mass should raise the suspicion of breast lymphoma. The most critical aspect in the workup of a breast mass is the tissue biopsy, since radiographically lymphomatous lesions are indeterminate. We present the case of a 79-year-old woman with abdominal pain and a palpable breast mass; we also make a brief summary of the clinical features and main imaging findings of NHL (plain radiograph, mammography, breast ultrasound and thorax tomography).

Primary Malignant Lymphoma of the Breast: Clinicopathological study of 9 cases / 대한혈액학회지

BACKGROUND: Primary lymphoma of the breast is rare disease, accounting for 1.7% to 2.2% of extranodal lymphoma and 0.38% to 0.7% of non-Hodgkin's lymphoma. The aim of this study was to evaluate the clinicopathological features and treatment outcome of patients with primary breast lymphoma (PBL). METHODS: We conducted retrospective review of cases of non-Hodgkin's lymphoma diagnosed at Korea Cancer Center Hospital between 1989 and 2002. Nine of the 1050 cases fulfilled the criteria for PBL. RESULTS: All patients were women (median age, 45 years) and they usually had breast masses that had recently become enlarged. Six cases involved the breast alone (stage IE), whereas three cases also involved the ipsilateral lymph nodes (stage IIE). Histopathologic studies revealed diffuse large B cell in 7 cases, marginal zone B cell lymphoma in 1 case, small lymphocytic lymphoma in 1 case. Immunohistochemical analysis revealed B-cell phenotype in all cases. Modified radical mastectomy and chemotherapy was done in 4 cases, modified radical mastectomy and chemoradiotherapy was done in 1 case, chemoradiotherapy was done in 1 case, modified radical mastectomy alone was done in 1 case, chemotherapy alone was done in 1 case, and radiotherapy alone was done in 1 case. All cases achieved a complete remission, but median overall survival was 12 months. CONCLUSION: PBL represented 0.9% of all non-Hodgkin's lymphomas in our institute. The most frequent pathologic type was diffuse large B-cell lymphoma. There was no uniform approach to the treatment of PBL. The patients showed very poor prognosis irrespective of the type of treatment modality.

Primary Lymphoma of Bone Combined with Pancytopenia / 대한혈액학회지

Primary lymphoma of bone, first described by Parker and Jackson in 1939, is derived from bone marrow reticuloendothelial systems. It is uncommon neoplasm representing only 5% of all extranodal non-Hodgkin's lymphoma. Primary bone non-Hodgkin's lymphoma is characterized by initial symptoms of bone pain with associated single or multiple radiological bone lesions, with or without local soft tissue swelling, pathologic fractures,or regional/distant metastasis. Isolated bone marrow non-Hodgkin's lymphoma is rare, and determining the diagnosis difficult. The common features are fever, abnormal liver function, pancytopenia, polyserositis, neurologic symptoms, opportunistic infections, and a short, fatal course. Treatment includes surgery, radiotherapy, and chemotherapy depending on multiple factors, including histopathologic type of tumor, stage of disease, location of the lesion, and extent of osseous involvement. Five year survival rate is above 80%, when treated by combined radiation & chemotherapy. We experienced a patient who admitted to our department of Internal Medicine. She had mutiple bone pain, general weakness and diagnosed as primary lymphoma of the bone with severe pancytopenia. Her presentation, treatment, and clinical progress were discussed.

Nesal T-cell Lymphoma associated with Hemophagocytic Syndrome: A case report

Peripheral T-cell lymphoma is the generic group given to a family of tumors composed of neoplastic lymphocytes with phenotypic features of peripheral T-cells. Certain peripheral T-cell lymphomas develop a hemophagocytic syndrome that mimics malignant histiocytosis, both clinically and pathologically. We experienced a case of nasal T-cell lymphoma, histologically mimicking malignant histiocytosis in a 40-year-old male. The chief complaints were nasal obstruction and intermittent mild fever. Mild anemia, elevated SGOT and SGPT, polyclonal gammophthy, and moderate hepatomegaly were present. Two weeks later was present an enlarged cervical lymph node. The biopsied nasal mass showed angiocentric and angiodestructive peripheral T-cell lymphoma withextensive necrosis and marked erythrophagocytosis by non-neoplastic histiocytes. Subsequently, cervical lymph node was biopsied, which showed peripheral T-cell ltmphoma with extensive necrosis and erythrophagocytosis as well. The atypical lymphoid cells revealed pan-T(+), but CD4(-) and CD8(-), whereas the reactive histiocytes showed lysozyme(+), immunohistochemistry.

Malignant Histiocytic Lymphoma of Scalp

This paper reports a case of malignant histiocytic lymphoma mainly in the frontal region with intracranial extradural extension. Operation was performed to remove the mass but the tumor began to recur rapidly. And so under the condition that the operative wound was not fully healed, the patient received radiotheray and chemotherapy postoperatively, with remarkable regession of the tumor.