Asunto(s)
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Biomarcadores de Tumor/metabolismo , Linfoma de Células T Periférico/genética , Linfoma de Células T Periférico/metabolismo , Mutación/genética , Fosfolipasa C gamma/genética , Biomarcadores de Tumor/genética , Femenino , Estudios de Seguimiento , Humanos , Técnicas para Inmunoenzimas , Linfoma de Células T Periférico/mortalidad , Linfoma de Células T Periférico/patología , Masculino , Persona de Mediana Edad , Estadificación de Neoplasias , Fosfolipasa C gamma/metabolismo , Pronóstico , Tasa de Supervivencia , Análisis de Matrices TisularesRESUMEN
A 70-year-old male presented with orbital masses affecting the muscular cone. His past medical history was notable for diabetes mellitus, ischemic cardiopathy, sleep-apnea syndrome, and multiple serous effusions. The first biopsy specimen of affected orbital tissue revealed fibrohistiocytic infiltration resembling xanthogranuloma or Erdheim-Chester disease (ECD). An ulterior biopsy of affected orbital tissue showed lymphocyte emperipolesis with immunopositivity for CD68 and S100 but negative staining for CD1a marker, strongly suggesting Rosai-Dorfman disease (RDD). Afterward, pericardium and peritoneal effusions resulted in constrictive pericarditis and retroperitoneal fibrosis, respectively. The absence of distinctive clinical features made the diagnosis especially challenging. Attempts to control the disease using corticosteroids, radiation, orbital surgery, and interferon were unsuccessful. Aggressive treatments such as chemotherapy were not considered appropriate due to the general deterioration of our patient. Although the possibility of two concurrent diseases (e.g., systemic ECD and orbital RDD) cannot be discarded, we interpreted the orbital findings as likely due to RDD, and the entire condition of our patient as an extranodal RDD with atypical clinicopathological findings and outcome.
RESUMEN
We present the case of a 76-year-old woman who presented with dyspnea on exertion. Echocardiography revealed a mass located in the left atrium in proximity to the posterior leaflet of the mitral valve. The mass was successfully excised under cardiopulmonary bypass. Histopathologic analysis showed a capillary hemangioma. Twenty-five months after surgery, the patient remained asymptomatic, and her echocardiogram was normal. Herein, we discuss the histopathology and the clinical, diagnostic, and therapeutic aspects of hemangiomas. We include a selected overview of the literature on surgically treated patients.