RESUMEN
INTRODUCTION: Behçet's disease (BD) is a multi-system, chronic and relapsing disorder classified as "vasculitic syndrome". It typically affects young adult females between 20 and 40 years of age. There are some typical clinical manifestations associated with this disease, however, at times; rare sign and symptoms pose a challenge to the treating physician and making a definitive diagnosis. Presentations with cardiac symptoms are one of the extremely rare manifestations of the Behçet's disease. METHODS: The authors present clinical, laboratory and imaging findings of a patient who presented with a cardiac mass which was the first presenting feature or manifestation of Behçet's disease. RESULTS: A 19-year-old boy was admitted to our hospital for the investigation of "fever of unknown origin", weight loss, shortness of breath and a scrotal ulcer of recent on-set. X-ray chest and electrocardiograms were inconclusive. Transthoracic echocardiography revealed a right ventricular (RV) mass attached to the interventricular septum measuring 1.5 × 1.5 cms (Panel A). Cardiac MRI identified it as a RV Myxoma. In addition, on CT scan of the chest pulmonary embolism was noted. The patient underwent excision biopsy of the tumor under cardiopulmonary bypass via right atriotomy (Panel B). Histopathology of the mass described it as "an organizing thrombus with a few groups of interrupted myocardial fibers and some infiltration of lymphocytes and plasma cells". Moreover his HLA typing was found positive for HLA-B51 (5). In view of the above findings and associated lesions, the patient was diagnosed as a case of Behçet's disease. The medical management included immunosuppressant and anticoagulation. CONCLUSION: Behçet's disease, even in the absence of the typical clinical features, should be considered in the differential diagnosis of right ventricular mass, especially when dealing with young adults from the Mediterranean basin and the Middle-East.
RESUMEN
Cardiac inflammatory myofibroblastic tumor (IMT) is a rare entity and is associated with distinct clinical, pathological and molecular features. The clinical behavior, natural history, biological potential, management and prognosis of such tumors are unclear. We present herewith an adolescent girl who presented with similar entity involving the junction of the right atrium and the inferior vena cava (IVC) in association with thrombocytosis and IVC thrombosis leading to obstruction of blood flow. Diagnostic tools included imaging and immuno-histopathology studies. Surgical management included resection of the tumor and thrombo-embolectomy of the IVC under cardiopulmonary bypass. This case is unique due to association of complete obstruction of IVC caused by the strategic location of the tumor, thrombosis of vena cava and association of thrombocytosis. These features have not been reported yet in relation to the cardiac IMT. This report will help in better understanding and management of similar cases in terms of planning cannulation of femoral veins or application of total hypothermic circulatory arrest during cardiopulmonary bypass and prompt us to look for recurrence or metastasis during follow up using echocardiography and laboratory investigations. The possibility of IMT should be kept in the differential diagnosis of cardiac tumors especially in children and adolescents.
