Recurrent cardiac myxoma in a 25 year old male

We present a 25 year old Caucasian male patient with multiple recurrences of cardiac myxomas after surgical removal of the original tumor. His mother was operated on for right ventricular myxoma. The genetic analyses disclosed an aneuploid DNA content by flow cytometry analysis. The familial form of the cardiac myxomas must be distinguished from Carney complex syndrome. A long- term echocardiographic follow up is recommended to patients and their first degree relatives with cardiac myxomas. Background Primary tumors of the heart are extremely rare with a prevalence rate of around 0.0017% to 0.003% in collec- tive autopsy series. The majority of them are benign (1,2). Cardiac myxomas are the commonest primary be- nign tumor in adults. The natural history of sporadic cardiac myxomas is characterized by a predilection for the left atrium of the heart, and occurs in 75% of cases, mainly in middle-aged women and arising usually from the interatrial septum at the border of the fossa ovalis. They have an incidence of relapse in up to 3%, by con- trast with familial and complex types of disorder which have recurrence rates 12% and 22% respectively (3). The familial form of cardiac myxomas is characterized by occurring in a younger patient age group and pre- dominately in males, and with unusual location of the tumor with a tendency to be multifocal in one or more chambers of the heart (2,4). The association of cardiac myxomas with primary pigmented nodular adrenocortical disease, pituitary-related symptoms and multiple neoplasia syndromes of the cutaneous, thyroid, testicular, breast and neural systems are referred as complex myxomas or Carney syndrome. The size, mobility and location determine the clinical manifestations of the tumor. The typical triad includes intracardiac obstruction, embolic events and constitu- tional symptoms. The echocardiography examination is a useful diagnostic tool to determine the tumor size and type, anatomical localization and valvular abnormalities (2,5). The MRI and CT scan can also often be helpful in clarifying the diagnosis of cardiac myxomas. In the presence of clinical and /or imaging evidence of a cardiac mass, surgical excision must be performed in order to avoid complications of intracardiac obstruction and embolization of tumor fragments or thrombi. The high incidence of recurrence of the myxoma in our case highlights the difficult management of the tumor and un- derlines the necessity of the genetic profile analysis in the younger age group patients and their first degree relatives.