Asunto(s)
Mastocitosis Sistémica , Mieloma Múltiple , Anticuerpos Monoclonales Humanizados , Familia , Humanos , Lenalidomida , Activación de Linfocitos , Mastocitosis Sistémica/diagnóstico , Mastocitosis Sistémica/tratamiento farmacológico , Mastocitosis Sistémica/genética , Familia de Moléculas Señalizadoras de la Activación LinfocitariaAsunto(s)
Antineoplásicos/farmacología , Biomarcadores de Tumor/metabolismo , Leucemia Linfocítica Granular Grande/patología , Linfoma de Células T/patología , Familia de Moléculas Señalizadoras de la Activación Linfocitaria/metabolismo , Adulto , Anciano , Anticuerpos Monoclonales Humanizados/farmacología , Anticuerpos Monoclonales Humanizados/uso terapéutico , Antineoplásicos/uso terapéutico , Biopsia , Femenino , Humanos , Leucemia Linfocítica Granular Grande/sangre , Leucemia Linfocítica Granular Grande/diagnóstico , Leucemia Linfocítica Granular Grande/tratamiento farmacológico , Ganglios Linfáticos/patología , Linfoma de Células T/diagnóstico , Linfoma de Células T/tratamiento farmacológico , Masculino , Persona de Mediana Edad , Células T Asesinas Naturales/patología , Familia de Moléculas Señalizadoras de la Activación Linfocitaria/antagonistas & inhibidoresRESUMEN
BACKGROUND: Cardiac tumours are of rare incidence and usually occur in the form of secondary tumours. Most metastatic tumours are melanomas, sarcomas, lung, and haematological malignancies. Neuroendocrine carcinomas (NECs) of the heart are extremely unusual. This case report demonstrates a solitary high-grade NEC of the heart with an individual therapy strategy and follow-up. CASE SUMMARY: A 50-year-old gentleman presented with a 2 days history of recurrent episodes of chest pain. Echocardiography, computed tomography, and magnetic resonance imaging revealed tumorous lesions of the ventricles and aortic valve with large circular pericardial effusion. Histopathology results of the biopsy revealed a poorly differentiated small cell tumour of the neuroendocrine type. Despite further investigations with multiple imaging modalities and laboratory, no primary was found. Chemotherapy was initiated but size progression of the tumour was detected. As no other tumorous lesions were detected and resection was not possible because of the tumour complexity, decision on heart transplantation was made. However, due to the necessary immunosuppression after the heart transplantation, multiple metastasis where discovered in the course of treatment. DISCUSSION: The presence of a NEC in the heart without evidence of any other metastasis or evidence of primary tumour in other organs is clinically unique. For this individual case, heart transplantation was the therapy of choice due to tumour progression under chemotherapy and lacking possibility of resection, as no other suspect lesion was found other than the ones found in the heart. However, the risk of exacerbation of undiscovered micrometastases under necessary immunosuppression following the heart transplantation should be considered.
RESUMEN
Study Design Case report and review of the literature. Objective To report a unique case of an intraspinal chondrosarcoma that was diagnosed 18 years after radiotherapy for a cervical carcinoma and its remarkably unusual clinical presentation. Methods A retrospective case description of an intraspinal mass lesion that occurred 6 weeks after previous spinal surgery. Results Within â¼9 weeks, the tumor had infiltrated the peritoneal cavity and reached the lumbar subcutaneous tissue. Conclusion Radiation-induced sarcomas are rare, are highly aggressive, and may be difficult to diagnose. Furthermore, the only means of achieving long-term survival is through early and extensive surgery.
RESUMEN
This study was undertaken to analyze the differentiation profiles assessed by immunophenotyping in AIDS-related B-cell lymphoma (ARL) and their relation to the clinical course. Paraffin-embedded sections of 89 ARL cases during 1989 to 2004 were stained immunohistochemically with antibodies to CD3, CD10, CD20, CD38, CD138/Syndecan-1 (Syn-1), multiple myeloma-1/interferon regulatory factor-4 (MUM1/IRF4), B-cell lymphoma protein-2 (BCL-2), BCL-6, latent membrane protein-1 (LMP-1), and Ki-67. Expression of CD10 and CD20 were associated with better overall survival (OS; P = .009 and P = .04, respectively). Expression of CD20 was associated with longer disease-free survival (DFS; P = .03), whereas expression of CD138/Syn-1 was associated with shorter DFS (P = .03). OS and DFS were worse in patients with immunophenotypic profiles related to post-germinal center (GC) differentiation (BCL-6 and CD10 negative, MUM1/IRF4 and/or CD138/Syn-1 positive) when compared with GC differentiation (P = .01). When controlled for age-adjusted International Prognostic Index (IPI), prior AIDS-defining illness (ADI), and year of ARL diagnosis, a post-GC differentiation remained significantly associated with poor OS and DFS. Expression of CD10 was associated with a preserved immunocompetence, whereas CD20 was less frequent in patients developing ARL while on highly active antiretroviral therapy (P = .04). In summary, lack of CD20 or CD10 expression and a post-germinal center signature are associated with a worse prognosis in ARL.