RESUMO
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.
Assuntos
Antineoplásicos/administração & dosagem , Imunoterapia/métodos , Linfonodos/patologia , Linfoma Difuso de Grandes Células B/patologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Estudos de Coortes , Terapia Combinada , Feminino , Humanos , Linfoma Difuso de Grandes Células B/terapia , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Taxa de Sobrevida , Resultado do TratamentoRESUMO
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.
Assuntos
Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Idoso , Idoso de 80 Anos ou mais , Linfoma Difuso de Grandes Células B/patologia , Imunoterapia/métodos , Linfonodos/patologia , Antineoplásicos/administração & dosagem , Prognóstico , Taxa de Sobrevida , Estudos Retrospectivos , Estudos de Coortes , Linfoma Difuso de Grandes Células B/terapia , Resultado do Tratamento , Terapia CombinadaRESUMO
Não há evidências relatadas na literatura de associação entre linfoma ósseo primário e osteocondroma ou da coexistência deles em uma mesma região óssea. Este relato de caso descreve um caso raro de linfoma ósseo primário ocorrendo juntamente com um osteocondroma no terço proximal de tíbia. Os sinais de imagem na ressonância magnética neste caso simulam uma degeneração sarcomatosa do osteocondroma.
In the literature, there is no evidence of relationship between primary bone lymphoma and osteochondroma or of coexistence of both of them in a single bone. The present report describes an uncommon case of primary bone lymphoma occurring simultaneously with osteochondroma in the proximal third of the tibia. In the present case, magnetic resonance imaging signs simulated the presence of sarcomatous degeneration.
Assuntos
Idoso , Osso e Ossos , Neoplasias Ósseas , Linfoma não Hodgkin , Osteocondroma , Tíbia/patologia , Diagnóstico por Imagem , Radiografia , Tíbia , Tomografia Computadorizada por Raios XRESUMO
El linfoma primario de hueso es una enfermedad infrecuente, que tiene una presentación y evolución diferente a los linfomas de otras localizaciones. Se presenta un caso de linfoma primario de hueso de localización craneana y esternal de rápido crecimiento. En su evolución, realizada la exéresis de la lesión primaria de calota, presentó aparición de nuevas lesiones de rápido crecimiento a nivel craneano y fémur y progresión de lesión preesternal que, con anatomía patológica de linfoma no Hodgkin difuso de células grandes B, inició R-CHOPP (ciclofosfamida, doxorrubicina, vincristina, prednisona y rituximab) con rápida disminución de todas las lesiones sin evidencia de progresión al cabo de los seis ciclos.(AU)
Primary bone lymphoma is a rare disease, which usually has a different presentation and evolution than lymphomas of other locations. We are presenting a case of primary bone lymphoma of rapid growth, in cranial and sternal locations. In its evolution, once the excision of the primary lesion of the skull was performed, the patient presented new lesions of rapid growth at the skull and femur level, and progression of pre-sternal lesion. With large B-cell diffuse non-Hodgkin lymphoma pathology, the patient initiated R-CHOPP (cyclophosphamide, doxorubicin, vincristine, prednisone and rituximab) with rapid reduction of all lesions without evidence of progression after the six cycles.(AU)
Assuntos
Feminino , Humanos , Pessoa de Meia-Idade , Neoplasias Ósseas/patologia , Linfoma Difuso de Grandes Células B/patologia , Neoplasias Primárias Múltiplas/patologia , Neoplasias Cranianas/patologia , Esterno/patologia , Anticorpos Monoclonais Murinos/uso terapêutico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias Ósseas/tratamento farmacológico , Ciclofosfamida/uso terapêutico , Doxorrubicina/uso terapêutico , Neoplasias Femorais/tratamento farmacológico , Neoplasias Femorais/patologia , Linfoma Difuso de Grandes Células B/tratamento farmacológico , Neoplasias Primárias Múltiplas/tratamento farmacológico , Prednisona/uso terapêutico , Doenças Raras/tratamento farmacológico , Doenças Raras/patologia , Neoplasias Cranianas/tratamento farmacológico , Vincristina/uso terapêuticoRESUMO
El linfoma primario de hueso es una enfermedad infrecuente, que tiene una presentación y evolución diferente a los linfomas de otras localizaciones. Se presenta un caso de linfoma primario de hueso de localización craneana y esternal de rápido crecimiento. En su evolución, realizada la exéresis de la lesión primaria de calota, presentó aparición de nuevas lesiones de rápido crecimiento a nivel craneano y fémur y progresión de lesión preesternal que, con anatomía patológica de linfoma no Hodgkin difuso de células grandes B, inició R-CHOPP (ciclofosfamida, doxorrubicina, vincristina, prednisona y rituximab) con rápida disminución de todas las lesiones sin evidencia de progresión al cabo de los seis ciclos.
Primary bone lymphoma is a rare disease, which usually has a different presentation and evolution than lymphomas of other locations. We are presenting a case of primary bone lymphoma of rapid growth, in cranial and sternal locations. In its evolution, once the excision of the primary lesion of the skull was performed, the patient presented new lesions of rapid growth at the skull and femur level, and progression of pre-sternal lesion. With large B-cell diffuse non-Hodgkin lymphoma pathology, the patient initiated R-CHOPP (cyclophosphamide, doxorubicin, vincristine, prednisone and rituximab) with rapid reduction of all lesions without evidence of progression after the six cycles.