Linfoma de células B primario de mama en una paciente con sida. Presentación de un caso y revisión de la literatura / Primary B-cell lymphoma of the breast in an AIDS patient. Case report and literature review

Los LNH constituyen la segunda neoplasia más frecuente en pacientes con VIH. Estas neoplasias están ligadas a la inmunodeficiencia, suelen ser de período de latencia prolongado y más frecuentes en hombres. Más del 95% de estas neoplasias son de fenotipo B, de alto grado de malignidad, extranodales y representan la causa de muerte en un 12% al 16% de los casos. El linfoma no Hodgkin primitivo de mama (LPM) es una entidad infrecuente, que representa el 2,2% de todos los linfomas extranodales y el 0,5% de todas las neoplasias malignas de la mama. Se presenta una mujer con sida y linfoma primario de mama. (AU)

NHL is the second most common neoplasm in patients with HIV. It is linked to immunodeficiency, tends to have a long latency period and is more common in men. More than 95% of these neoplasms are of phenotype B, high-grade, extranodal and are the cause of death in 12% to 16% of cases. Primitive non-Hodgkin lymphoma of the breast is a rare entity, accounting for 2.2% of all extranodal lymphomas and 0.5% of all breast malignancies. A woman with AIDS and primary breast lymphoma is presented. (AU)

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.

Linfoma primario de mama. Reporte de un caso / Primary breast lymphoma Report of a case

RESUMEN El linfoma primitivo de la mama es una forma rara de linfoma extraganglionar localizado, con una incidencia de 1,7 a 2,2 % de los linfomas extraganglionares y de un 0,04 a un 0,50 % de las lesiones malignas de la glándula mamaria de todos los linfomas no Hodgkin. El linfoma no Hodgkin primario de la mama crece con rapidez, con diseminación a la médula ósea y sistema nervioso central, generalmente de corta sobrevida. Se presentó un caso de una paciente femenina de 65 años de edad, blanca, que refirió haberse notado una tumoración en la mama derecha, de 3 cm de diámetro. Al examen físico se constató una tumoración, dolorosa a la palpación, localizada en el cuadrante superior externo. No existían adenopatías axilares a la palpación. La paciente no reportó síntomas específicos. Se realizó un ultrasonido diagnóstico que arrojó una imagen hipoecoica de 2,5 cm, de bordes no bien definidos. En un estudio mamográfico realizado se apreció una masa de alta densidad, irregular, que fue clasificado como BIRADS 4C. El diagnóstico de linfoma maligno no fue sospechado clínicamente. Fue operada, realizando biopsia por congelación que resultó positiva. Se le ejecutó una mastectomía total ampliada cuyo informe histopatológico fue: linfoma linfocítico pobremente diferenciado sin metástasis ganglionares axilares (0/29). Como tratamiento adyuvante se utilizó la poliquimioterapia asociada a la radioterapia. La paciente en la actualidad no ha presentado recaída y se mantiene viva tras 13 años de seguimiento.

ABSTRACT Primary lymphoma of the breast is an unusual kind of located extranodal lymphoma, with an incidence from 1.7 % to 2.2% of extranodal lymphomas, and from 0.04 % to 0.50 % of the mammary glands malignant lesions of all non-Hodgkin lymphoma. The primary non-Hodgkin's lymphoma of the breast grows rapidly, with dissemination to the bone marrow and central nervous system, generally of short over-life. The case presented is the one of a female, white patient, aged 65 years who told she perceived a tumor in her right breast of 3 cm diameter. At the physical examination a tumor was noted, painful at palpation, located in the higher external quadrant. Axillary adenopathy were not found at palpation. The patient did not reported specific symptoms. An ultrasound diagnosis was carried out showing a hypo-echoic image of 2.5 cm, with non well-defined margins. An irregular, high density mass was found in a mammographic study; it was classified as BIRADS 4C. The diagnosis of malignant lymphoma was not clinically suspected. The patient underwent a surgery, and a frozen section biopsy was positive. A total wide mastectomy was carried out and its pathological inform was: poorly-differentiated lymphocytic lymphoma without axillary nodal metastasis (0/29). Polychemotherapy associated to radiotherapy was used as adjuvant treatment. Currently, after a 13-years follow-up, the patient did not relapsed and is still alive.

Manejo diagnóstico-terapéutico de las lesiones mamarias atípicas / Diagnostic-therapeutic Management of Atypical Breast Lesions

Las lesiones mamarias se dividen histológicamente en dos grandes grupos, malignas y benignas. Las lesiones malignas pueden ser de origen ductal o lobulillar, siendo el carcinoma ductal infiltrante la neoplasia invasiva más frecuente. Las lesiones benignas se clasifican en no proliferativas, proliferativas sin atipias y proliferativas con atipias. Dentro de los dos últimos grupos se encuentran entidades que conllevan un alto riesgo de desarrollar carcinoma de mama, como pueden ser la hiperplasia ductal atípica, la cicatriz radial o la neoplasia lobular. Revisamos en qué consisten dichas entidades y cuáles son sus características principales en imagen, fundamentalmente en mamografía y ecografía. Si tras realizar una biopsia se obtiene uno de esos diagnósticos histológicos, es importante analizar las características imagenológicas y el tipo de procedimiento realizado (número de cilindros obtenidos, calibre de aguja...), para realizar un adecuado manejo posterior. En algunos casos la actitud a seguir será la extirpación quirúrgica completa de la lesión, mientras que en otros se podrá realizar una extirpación percutánea (mediante biopsia con aguja de vacio), o incluso seguimiento estricto por imagen. Mediante diferentes casos mostraremos nuestra experiencia y analizaremos la literatura vigente para recordar esas entidades y llegar a un consenso adecuado sobre el manejo de las mismas.

Breast lesions are divided histologically into two large groups, malignant and benign. Malignant lesions may be of ductal or lobular origin, with infiltrating ductal carcinoma being the most frequent invasive neoplasm. Benign breast lesions are classified as proliferative, proliferative without atypia and proliferative with atypia. Within the last two classifications are entities that carry a high risk of developing breast carcinoma, such as atypical ductal hyperplasia, radial scar or lobular neoplasia. We review what these entities consist of and what are their fundamental characteristics in image, fundamentally in mammography and ultrasound. When we perform one of these histological diagnoses after a biopsy, it is important to analyze the radiological characteristics and the type of procedure performed (number of cylinders, needle gauge ...) to perform an appropriate posterior management. In some cases the attitude to be followed will be the complete surgical removal of the lesion, while in others a percutaneous excision (through vacuum needle biopsy) or even strict image follow-up may be performed. Through different cases we will show our experience and analyze current literature to remember these entities and reach an adequate management consensus.

Metástasis mamaria de linfoma de células del manto / Mammary metastasis of mantle cell lymphoma

Resumen ANTECEDENTES: Los linfomas mamarios primarios constituyen menos de 1% de todos los linfomas no-Hodgkin, 1.7-2.2% de todos los linfomas no-Hodgkin nodales adicionales y 0.04-0.5% de todas las neoplasias malignas de la mama. El linfoma de células del manto representa 4% de los linfomas en Estados Unidos, y entre 7-9% en Europa y se diagnostica en pacientes con una mediana de edad de 60 años. Aparece en mujeres posmenopáusicas en forma de masa mamaria indolora, unilateral. El linfoma de mama secundario es sistémico, con afectación simultánea o posterior en otro sitio. CASO CLÍNICO: Paciente con una tumoración mamaria, inicialmente postraumática, que en el contexto de plaquetopenia se catalogó clínica y radiológicamente como hematoma y que, más tarde, el diagnóstico fue: linfoma de células del manto secundario a linfoma previo nodal inguinal en segunda línea de tratamiento con quimioterapia. CONCLUSIONES: La persistencia o el rápido crecimiento de un hematoma mamario o de una lesión con apariencia radiológica benigna, sobre todo con antecedente de linfoma previo, puede resultar maligna. La biopsia mediante aguja gruesa es decisiva en el diagnóstico porque permite el estudio histológico e inmunohistoquímico para la confirmación molecular. El tratamiento se basa, sobre todo, en quimio y radioterapia, opciones que mejoran la supervivencia y disminuyen la recurrencia.

Abstract BACKGROUND: Primary mammary lymphomas represent less than 1% of all Non Hodgking Lymphomas (NHL). Mantle cell lymphoma (MCL) represents 4% of lymphomas in the United States, and 7-9% in Europe and is diagnosed in patients with a median age of 60 years. Unilateral painless mammary mass in postmenopausal women use to be the clinical presentation. Secondary breast lymphoma is defined as the presence of systemic lymphoma with breast involvement, as the patient below. CLINICAL CASE: 57-year-old female patient with post-traumatic mammary tumor, with history of thrombocytopenia, first-time diagnosed of hematoma, with breast enlargement and later diagnosed as lymphoma of mantle cells with pathology test and classified as secondary to previous inguinal nodal lymphoma. CONCLUSIONS: It is important to keep in mind this diagnosis in view of the persistence or rapid growth of a mammary hematoma or other lesion with benign radiological appearance. The biopsy is the gold standard for diagnosis, since it allows the histological and immunohistochemical study, for molecular confirmation. The treatment is mainly based on chemo / radiotherapy, which improves survival and decreases recurrence.

Primary breast lymphoma:a prognostic analysis and literature review / 中华放射肿瘤学杂志

Objective To summarize the clinical characteristics and evaluate the feasible treatments of primary breast lymphoma (PBL).Methods The clinical data of 34 PBL patients (age 24-79 years) who were treated in our hospital between April 2006 and December 2013 were reviewed.Of these 34 patients, 18 had stage ⅠE PBL and 16 had stage ⅡE PBL.Pathological types included diffuse large B cell lymphoma (29 patients), anaplastic large cell lymphoma (2 patients), marginal zone lymphoma (2 patients), and mantle cell lymphoma (1 patient).Two patients underwent surgery, four patients received chemotherapy alone, five patients received chemoradiotherapy, fourteen patients underwent surgery plus chemotherapy, and nine patients underwent surgery plus chemoradiotherapy.The 5-year overall survival (OS) and progression-free survival (PFS) rates were determined by the Kaplan-Meier estimator.Results During follow-up, 26 patients were alive without lymphoma and 8 patients had died by the end of follow-up (7 died from lymphoma and 1 died from chemotherapy-related hepatic failure).Among the 6 patients who relapsed, 5(83.3%) had recurrence within the first 2 years of treatment.In particular, 1 patient who had bilateral breast involvement developed left breast relapse after bilateral mastectomy and chemotherapy, 2 patients had bone marrow metastasis, 1 patient had lung and mediastinal lymph node metastases, and 2 had skin relapse.The 5-year OS and PFS rates of all patients were 75% and 75%, respectively.Conclusions Since PBL is a rare malignancy, its overall prognosis is fair and the incidence of local relapse is low with chemotherapy alone or in combination with other treatments.However, further studies on the development of more effective treatments will be required for patients who have failed the existing treatments.

Research progress of diagnosis and treatment in primary breast diffuse large B-cell lymphoma / 白血病·淋巴瘤

Primary breast lymphoma is a rare form of extranodal lymphoma with distinct characteristics.It comprises diverse histologic subtypes with diffuse large B-cell lymphoma (DLBCL) as the most common one.In this review,the primary breast DLBCL was focused and the definition,staging,the role of systemic chemotherapy,local adjuvant radiotherapy and rituximab in treatment of primary breast DLBCL were described.

Linfoma primário da mama: relato de caso e revisão da literatura / Primary breast lymphoma: Case report and literature review

Introdução: O linfoma primário da mama (LPM) é um tumor raro, sendo responsável por 0,04% a 0,5% de todos os tipos de câncer de mama. O tipo mais comum de LPM é o linfoma não Hodgkin de grandes células B (LNHDGCB), correspondendo a 40% a 70% dos casos. Relato do caso: Paciente de 59 anos de idade apresentava uma massa mamária de cerca de 13 cm de extensão, aumento progressivo e dor local. O diagnóstico foi realizado através de biópsia incisional da mama. A análise imuno-histoquímica confirmou o diagnóstico de linfoma não Hodgkin, com expressão dos marcadores CD20, MUM-1, Bcl2. O tratamento foi realizado com quatro ciclos de quimioterapia com esquema R-CHOP (rituximab, ciclofosfamida, adriamicina, vincristina e prednisona) e complementação com radioterapia, tendo apresentado resposta clínica completa. Conclusão: O LPM é um subtipo raro de neoplasia mamária. O tratamento do LPM é similar ao de linfomas de mesmo estádio e histologia de outras localizações.

Isolated Post-Transplantation Lymphoproliferative Disease Involving the Breast and Axilla as Peripheral T-cell Lymphoma

Post-transplantation lymphoproliferative disorders (PTLDs) are a heterogeneous group of diseases that represent serious complications following immunosuppressive therapy for solid organ or hematopoietic-cell recipients. In contrast to B-cell PTLD, T-cell PTLD is less frequent and is not usually associated with Epstein Barr Virus infection. Moreover, to our knowledge, isolated T-cell PTLD involving the breast is extremely rare and this condition has never been reported previously in the literature. Herein, we report a rare case of isolated T-cell PTLD of the breast that occurred after a patient had been treated for allogeneic peripheral blood stem cell transplantation due to acute myeloblastic leukemia.

US Findings of Secondary Breast Lymphoma Detected by PET/CT

Breast lymphomas, either manifesting as a primary extranodal disease or secondary involvement, are a rare form of malignancy. Breast lymphomas can be visualized as hypermetabolic lesions in 18F-fluorodeoxyglucose-positron emission tomography/computed tomography (18F-FDG PET/CT) scans. Here, we describe a case of a secondary breast lymphoma, detected with 18F-FDG PET/CT.

Clinical analysis of 11 patients with bilateral primary breast lymphoma / 肿瘤

Objective: To investigate the clinicopathological features, treatment and prognosis of patients with bilateral primary breast lymphoma (BPBL). Methods: The clinical records of 11 patients with BPBL were collected, and the clinicopathological features, treatment and prognosis were retrospectively analyzed. Results: The medium age of 11 patients was 34 years old (ranged from 13 to 63 years). The breast tumors grew quickly, and the largest diameter of tumor was up to 20 cm. The occurrence of bilateral breast tumors was more often in synchronism than in heterochronism. The incidence rates of B symptoms and high level of serum LDH were both low in BPBL patients, and the axillary lymph node metastasis and distant metastasis were frequent. The BPBL was originated mainly from B cells, and the most common pathological grade of BPBL was grade high/intermediate. The median survival of BPBL was 33 months. Furthermore, chemotherapy played an important role in the treatment of BPBL. Conclusion: In consideration of BPBL which has a high rate of diagnostic errors, the clinicopathological features and medical imagings should be applied in the diagnosis of BPBL. The major modality used in the treatment of BPBL is chemotherapy. Copyright© 2011 by TUMOR.

Linfoma não Hodgkin primário de mama: relato de caso / Primary breast lymphoma: case report

Linfomas primários da mama são infrequentes, com incidência variando entre 0,04% e 0,53% de todas as doenças malignas da mama, e compreendem 2,2% de todos os linfomas extranodais. Para ser considerado linfoma primário da mama, deve preencher alguns requisitos já estabelecidos. As publicações de casos estão aumentando provavelmente pelo uso mais frequente da imunoistoquímica no esclarecimento diagnóstico, e anticorpo monoclonal já está sendo incluso no arsenal terapêutico, melhorando o prognóstico. Relatou-se um caso de linfoma não Hodgkin primário da mama que preencheu os requisitos necessários, discutindo os aspectos clínicos, histopatológicos, imunoistoquímicos, exames de imagem e tratamento.

Primary breast lymphomas are not frequent having an incidence between 0,04% and 0,53 % of all malignant breast diseases and englobe 2,2% of all extra nodal lymphomas. To be considered a primary breast lymphoma, it must fulfill some requisites previously established. The publications of cases are increasing in number, probably due to the more frequent use of immunohistochemistry in the diagnostic enlightenment and monoclonal antibody, being already included in the therapeutical arsenal to improve prognostics. We report here a case of a primary non-Hodgkin breast lymphoma that fulfilled all the necessary requisits, discussing the clinical, histopathologic, immunohistochemical aspects, image exams and treatment.

A Case of Primary Breast Lymphoma with Brain Metastasis Detected during Pregnancy

Primary breast lymphoma (PBL) is a rare clinical presentation of localized non-Hodgkin's lymphoma (NHL), and it makes up 0.04~1.1% of all breast tumors and it is 0.38~0.7% of all NHLs. The prognosis and patterns of relapse of PBL are still not clearly defined. The clinical features of PBL are different from those of breast carcinoma and the usual form of lymphoma. These features are a rapidly enlarging breast mass, multiple lesions, the absence of nipple discharge and retraction, and softer axillary lymph nodes as compared to the metastatic lymph nodes from breast carcinoma. B symptoms are unusual in PBL. A 30-year-old pregnant woman was admitted due to dysarthria and right side weakness that she had experienced for 7 days. She had several medical problems: intrauterine pregnancy at 34 weeks, some neurologic deficits and enlargement of both breasts. A biopsy from the breast and a brain magnetic resonance image (MRI) revealed diffuse large B cell lymphoma and multiple brain metastases, respectively. After delivery of a healthy, premature infant by Cesarean section, whole brain radiation therapy and combination chemotherapy (rituximab, cyclophosphamide, adriamycin, vincristine and prednisone) were started. She showed good response to therapy. We report here on this unusual case and we review the related literature.

Primary Malignant Lymphoma of the Breast: Case report

The authors report a case of primary breast lymphoma in a 29-year-old female patient. The patient had a growing, nontender, palpable mass in the left breast. Film-screen mammogram showed a sharply marginated mass with a thin perimeter of radiolucency, halo and slight lobulation.Sonography revealed a well marginated hypoechoic mass with posterior acoustic enhancement. The patient had undergone wide excision of mass and ipsilateral axillary dissection. Histologic examination and immuno-histochemistry confirmed a diagnosis of diffuse B-cell type non-Hodgkin's lymphoma. Abdominal ultrasonography, thoracic computed tomography and bone scan, checked postoperatively, showed non-specific findings. We concluded a final diagnosis of primary breast lymphoma, and report the case with a brief review of the literatures.

Primary breast lymphoma

We report the case of a 20-year-old female with lymphoma of the breast. Mammography showed an asymmetric pattern of confluent densities without any discrete mass. Sonography revealed diffuse heterogenous echoic mass intermingled with low-and medium level echoes. We present the clinical, radiographic and histologic features of primary breast lymphoma with a brief review of the literatures.