RESUMO
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.
Assuntos
Neoplasias Cardíacas/complicações , Neoplasias Cardíacas/diagnóstico por imagem , Síndromes Paraneoplásicas/etiologia , Síndromes Paraneoplásicas/patologia , Pênfigo/etiologia , Pênfigo/patologia , Pericárdio/diagnóstico por imagem , Timoma/complicações , Timoma/diagnóstico por imagem , Adulto , Imagem de Difusão por Ressonância Magnética , Feminino , Humanos , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada , Tomografia por Emissão de PósitronsRESUMO
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.