Successful Resection of Rapidly Developing Right Atrial Myxoma in a Patient with Familial Carney Complex

Background: Carney syndrome is a rare autosomal dominant inherited neoplastic condition characterised by atrial myxomas, buccal and cutaneous lentigines and non-cardiac neoplasia. The condition arises secondary to a mutation in the PRKAR1A gene. Successful surgical resection has been reported in several cases.

Objective: This case highlights successful surgical resection of a rapidly growing tumour including the atrial septum, where the tumour was anchored.

Methods: We report a case of a rapidly growing atrial myxoma in a patient with Carney Complex and review contemporary literature.

Results: A 14 year old young man with Carney syndrome initially presented to the clinic for screening for atrial myxoma. . Three generations of family members were affected including the patients’ mother and grandmother who previously underwent surgical resection of a left atrial myoma.

Annual screening failed to detect any intracardiac lesion until the patient presented at 17 years of age with a massive right atrial myxoma(Image 1). The tumour measured 30 x 50mm. Surgical resection was undertaken via a median sternotomy(Image 2). Despite the rapid growth of the tumour the patient recovered well and is now asymptomatic.

Conclusion: In conclusion patients with Carney complex have the potential for rapid growth of an atrial myxoma, coexistent tumours, and a high risk of recurrence. Close serial surveillance of patients with this condition is warranted.