RESUMEN
Paraneoplastic Pemphigus (PNP), a rare autoimmune blistering disease, can be accompanied by both benign and malignant neoplasms. The most frequently reported associated malignancies include lymphomatoid and hematologic malignancies, Castleman's disease, carcinoma, thymoma. In a patient suspected of PNP, with no known history of malignancy, an extensive workup is suggested to look for underlying malignancy, which has to be treated to induce PNP remission. In this clinical case report, cross sectional imaging of a young female diagnosed with PNP, unveiled a pericardial mass lesion extending into transverse pericardial sinus. Excisional biopsy was performed. Histopathology revealed pericardial ectopic thymoma.
Asunto(s)
Neoplasias Cardíacas/complicaciones , Neoplasias Cardíacas/diagnóstico por imagen , Síndromes Paraneoplásicos/etiología , Síndromes Paraneoplásicos/patología , Pénfigo/etiología , Pénfigo/patología , Pericardio/diagnóstico por imagen , Timoma/complicaciones , Timoma/diagnóstico por imagen , Adulto , Imagen de Difusión por Resonancia Magnética , Femenino , Humanos , Tomografía Computarizada por Tomografía de Emisión de Positrones , Tomografía de Emisión de PositronesRESUMEN
We present a rare case of hemodynamically significant recurrent primary chylopericardium due to a rare lymphatic malformation, mediastinal lymphangiomyoma, treated by complete surgical resection with total pericardiectomy. Recurrent chylopericardium or chylothorax, a common complication following the primary intervention, was successfully treated by diaphragmatic fenestration technique which is as good as thoracic duct ligation.