myxomas are the most common type of cardiac tumors , and approximately 75% of these tumors originate in the left atrium and 15% to 20% in the right atrium , with only less than 10% arising from the right and left ventricles . however , the incidence of myxomas in the great vein is extremely rare , and a thorough review of the literature demonstrates only one case in the inferior vena cava ( ivc ) , one case in the femoral vein , and one case in the superior vena cava ( svc ) .
most patients present clinically with one or more of a triad of emboli , intracardiac obstruction , and constitutional manifestation ; furthermore , a small myxoma has no symptoms .
a 24-year - old man presented with moderate shortness of breath of two months duration , which was intermittent and unrelated to exercise or rest . physical examination showed normal vital signs , blood pressure of 110/80 mm hg , and pulse rate of 75 beats per minutes and regular .
there were no abnormal physical findings such as jugular venous distention , rales , and edema .
there was a holosystolic murmur ( grade 2/6 ) over the right parasternal area with radiation to the left .
laboratory findings of note were a hematocrit value of 43% and an erythrocyte sedimentation rate of 12 .
chest x - ray revealed no abnormalities , and the electrocardiogram demonstrated a normal sinus rhythm with normal voltage waves .
the patient underwent two - dimensional echocardiography , which demonstrated a 4  5  5 cm atrial mass attached by a long stalk to the svc . during atrial systole , the mass moved into the tricuspid annulus , resulting in an apparent obstruction .
based on these findings , a right atrium tumor was diagnosed and the patient underwent surgery .
the venous return was accomplished by svc - ivc cannulation , and the aorta was cross - clamped with cardioplegic arrest .
core cooling to 32 c , aortic occlusion , and cold cardioplegic arrest provided exposure to the tumor through an extended right atriotomy to the svc . the right atrium was opened , and a cluster of grape - shaped , glistening , white masses of leathery consistency ( as reported by devig ) was noted within it .
the mass was not attached to the right atrial wall and had a long stalk , which was emerging from the svc orifice ( figures 1 - 3 ) .
cavotomy was performed at the site where the stalk was palpable ; and under tension and stretching of the stalk , the point of origin was determined to be in the proximal part of the svc wall .
decision was made to extirpate the stalk base under caudal traction . with the aid of this maneuver
, the tumor base was excised from the svc wall and the small defect in the svc was repaired .
the patient was weaned off cardiopulmonary bypass uneventfully , and his postoperative course was unremarkable .
pathologic examination of the tumor revealed a 57 cm firm , cluster tumor , and histological examination revealed a benign myxoma .
myxomas were reported for the first time in 1845 by king and then by goldberg in 1952 .
diagnostic methods for myxomas include chest radiography ( pulmonary hypertension , atrial enlargement ) , two - dimensional transthoracic echocardiography ( tte ) , transesophageal echocardiography ( tee ) , and angiography , as well as some other noninvasive modalities such as computed tomography and magnetic resonance imaging .
tee is helpful for the precise localization of a tumor and its point of attachment as well as obstruction of cardiac valves .
the indication for selective angiography in patients with myxomas remains for the detection of coronary artery disease if the patients are more than 40 years old .
rath and demorise utilized this imaging method for the detection of coronary artery embolus and myocardial infarction in patients with myxomas .
cardiac tumors are exceedingly rare , with the incidence ranging between 0.001% and 0.03% of the autopsied specimens .
approximately , 75% of primary cardiac tumors are benign , and myxomas are the most frequent type of benign intracardiac tumors with an incidence ranging from 83% to 92% .
a literature search revealed only 5 previously reported cases of myxomas arising from the femoral vein , ivc ( two cases ) , eustachian valve , and svc .
another instance of a lesion arising from the svc was reported by teixido et al .
bontolotti and associates reported a patient with unusual findings of a right atrial myxoma arising from the supra hepatic segment of the ivc ; the tumor was excised under core cooling and cardioplegic arrest with a short period of low - flow cardiopulmonary bypass .
along the same lines , devig and colleagues described a young man who underwent an excision of a right atrial myxoma located at the junction of the right atrial wall and the anterosuperior aspect of the ivc .
chiming in with the previous findings , cujec and associates reported a 21-year - old man with a right atrial myxoma arising from the eustachian valve that prolapsed to the ivc , causing the budd - chiari syndrome , and elmusa et al . reported the excision of a right atrial myxoma from the supra hepatic segment of the ivc .
were the first to report the case of a young man diagnosed with a testicular germ cell tumor with pulmonary metastasis : there was a large vegetative mass arising from the svc and extending through the right atrium .
right atrial myxomas may present with embolic obstructive or constitutional signs and symptoms like syncopal attack , exertional dyspnea , and chest discomfort due to a transient occlusion of the tricuspid by a thrombus or a fragment of the tumor tissue .
the most characteristic finding is an ejection - type systolic murmur in the pulmonary area that often varies from day to day or with positional change .
be that as it may , the absence of symptoms , with the exception of dyspnea , in our patient was remarkable insofar as other patients with right atrial myxomas are reported to complain of vague constitutional symptoms such as malaise , low - grade fever , weakness , and unexplained weight loss .
laboratory examinations typically reveal microcytic anemia in addition to an elevation in the erythrocyte sedimentation rate .
once the diagnosis of a cardiac myxoma is established , a prompt excision is essential to prevent complications . in our patient
, the myxoma was attached to the medial wall of the svc and the stalk of the tumor was clearly visualized by the tee .
patients with complex and familial myxomas are characterized by younger age , atypical location , multiple tumor sites , and high incidence of recurrent tumors .
painstaking surgical management and postoperative follow - up are , therefore , of vital importance in this group of patients .
as this case report clearly shows , rare and bizarre variations in the origin of myxomas may occur and they can not be discovered preoperatively unless an index of suspicion is maintained .
 myxomas are the most common benign cardiac tumors . 
 myxomas are more common in the left heart chamber than the right side chamber . an extracardiac origin presenting as 
 a right atrial mass is very rare . 
 we present a case of myxoma originating in the superior vena cava ( svc ) in a 24-year - old man , who underwent surgical resection . 
 preoperative two - dimensional echocardiography demonstrated a mass in the right atrium . 
 intraoperatively , the tumor was found to have originated from the svc orifice . 
 the tumor was excised from the svc by opening the one - third proximal portion of the svc . 
 pathological examination revealed a myxoma , and one - year follow - up showed no evidence of the recurrence of any tumors in the svc . 
Introduction
Case Report
Discussion
Conclusion