Inflammatory myofibroblastic tumor of the right atrium.
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.
Jha
Neerod K
NK
Division of Adult Cardiac Surgery, Sheikh Khalifa Medical City (Managed by Cleveland Clinic), P.O. Box 51900, Abu Dhabi, UAE.
Trudel
Michel
M
Eising
Gregory P
GP
Lange
Peter
P
Al Sousi
Awatif
A
Al Mahmeed
Wael
W
Khan
Javed A
JA
Saleh
Moataz A
MA
Von Canal
Friederike
F
Misra
Virendra K
VK
Augustin
Norbert
N