A rare case of cardiac lymphangioma

Abstract

IntroductionPrimary cardiac tumors are rare, their incidence ranging from 0.0017% to .33% at autopsy.1 Cystic lymphangioma, usually confined to head and neck, is a well-recognized benign tumor that occurs during childhood.2 However, a cardiac lymphangioma is exceptionally uncommon and a particularly rare form of cardiac disease. Case reportA 22-year-old girl admitted with accidental trauma and dyspnea on exertion since childhood. Chest X-ray showed right hydropneumothorax and mild cardiomegaly. MDCT chest showed right hydropneumothorax and a cystic mass which was present on the left side anterior to the heart. Then Echo was done which showed normal left ventricular function, normal great vessels, chambers and valves. A 12cm×8.5cm mass was seen in the pericardium over right ventricle (RV) compressing it with no features of obstruction. Cardiac MRI revealed a large hyperintense lesion of 14cm×8cm×12cm in anterior mediastinum with multiple septations which was present anteriorly to right atrium (RA) and right ventricle, compressing RA and RV. MDCT coronary angiogram RCA running through the mass. During surgery, after opening the pericardium a cystic mass of 20cm×15cm×15cm was found adherent to RA and RV moving with the heart. About 700ml of fluid was aspirated and mass was opened up which showed multiple strands and trabeculations. Mass was completely excised except the wall adherent to RV. RCA was transected and end to end anastomosed with saphenous venous graft. Histopathology of biopsied mass confirmed it as cardiac lymphangioma containing lymphatic spaces lined by single layer of endothelium. DiscussionCardiac lymphangiomas are very rare. Cardiac lymphangioma are most often diagnosed incidentally by chest X-ray presenting as asymptomatic masses. However, they may cause congestive heart failure, syncopal or embolic pathology, arrhythmias, palpitations, or cardiac tamponade. Majority of symptoms are accredited to their size and location. These tumors most commonly occur in the pericardial space, but other unusual primary sites include the myocardium, the posterior wall of the left atrium, and AV node regions.3–5 At MRI, these tumors may have a high signal intensity on T1 weighted images, perhaps due to the presence the proteinaceous material in the lymph within the stroma and cystic spaces.6 Cystic lymphangioma is typically a multiloculated lesion with cystic cavities divided by the septa of variable thickness. Cardiac lymphangiomas have a possible risk of recurrence, especially if there has been incomplete resection. Therefore, extensive resection is recommended.7 ConclusionImaging with MDCT and MRI aid in the proper diagnosis of pericardial lymphangioma. A meticulous planning is required for surgical excision of this lesion. We report this case because of its rarity and to highlight the role of imaging and the surgical challenge in view of its location and proximity to coronary vessels.