Asunto(s)
Factores de Transcripción de Tipo Kruppel/genética , Linfoma de Células B de la Zona Marginal/genética , Receptor Notch2/genética , Neoplasias del Bazo/genética , Humanos , Linfoma de Células B de la Zona Marginal/diagnóstico , Mutación , Pronóstico , Neoplasias del Bazo/diagnóstico , Resultado del TratamientoRESUMEN
INTRODUCTION: Primary breast lymphoma is a rare form of localized extranodal lymphoma, which affects the mammary glands unilaterally or bilaterally, and can also affect the regional lymph nodes. MATERIALS AND METHODS: We reviewed 55 patients, with disease stages IE and IIE, diagnosed in 16 Spanish institutions between 1989 and 2016. A serial of clinical variables and treatment were collected, and overall survival (OS) and progression-free survival (PFS) were calculated. RESULTS: Of the 55 patients, 96.4% were women with an average age of 69 years. A total of 53 patients corresponded to non-Hodgkin lymphoma (NHL), of whom 36.3% had lymph node involvement upon diagnosis. Of the patients, 58.2% were stage IE, and 41.8% were stage IIE. Treatments received included radiotherapy (36.3%), chemotherapy (85.5%), and rituximab (in 38 of the 45 patients with NHL treated with chemotherapy). In all, 82.2% of complete responses were achieved. OS and progression-free survival at 5 years in NHL patients was 76% and 73%, respectively. CONCLUSION: Current treatments (chemotherapy, immunotherapy, and radiotherapy) achieve good control of the disease, with an OS of 5 years in 80% of the patients, although there is no consensus in treatment, given the scarce incidence of these lymphomas.
Asunto(s)
Antineoplásicos/uso terapéutico , Neoplasias de la Mama Masculina/tratamiento farmacológico , Neoplasias de la Mama/tratamiento farmacológico , Linfoma/tratamiento farmacológico , Adulto , Anciano , Anciano de 80 o más Años , Neoplasias de la Mama/mortalidad , Neoplasias de la Mama/patología , Neoplasias de la Mama Masculina/mortalidad , Neoplasias de la Mama Masculina/patología , Supervivencia sin Enfermedad , Femenino , Humanos , Ganglios Linfáticos/patología , Linfoma/mortalidad , Linfoma/patología , Linfoma no Hodgkin/tratamiento farmacológico , Linfoma no Hodgkin/mortalidad , Masculino , Persona de Mediana Edad , Pronóstico , Resultado del TratamientoRESUMEN
Association of Hodgkin lymphoma and non-Hodgkin lymphoma is rare and, specifically, the combination of Hodgkin lymphoma and mantle cell lymphoma has not been previously described. Here we describe composite mantle cell lymphoma and Hodgkin lymphoma affecting the spleen in one case and the eyelid and cervical lymph nodes in a second. In both, nodules of classical Hodgkin lymphoma were intermixed with diffuse or nodular areas of typical mantle cell lymphoma. Immunohistochemical and molecular analyses confirmed cyclin D1 overexpression secondary to the translocation t(11;14) in the small mantle cell lymphoma component; with CD30, CD15, and EBV expression in the Hodgkin and Reed-Sternberg cells. Finally, clonal analysis of rearranged immunoglobulin genes performed on microdissected Hodgkin and Reed-Sternberg and mantle cell lymphoma cells provided definite evidence of separate clonal origins of the two tumors in the patients. These EBV-positive, clonally unrelated tumors seem to represent true composite neoplasms, in contrast to cases showing merely clonal progression.