ECHO OF THE MONTH
Primary TUMOR OF THE HEART
Manish Bansal*, Rahul Mehrotra*, Vinayak Agrawal*, Ravi R Kasliwal**, Naresh Trehan#

* Consultant Cardiology, ** Senior Consultant Cardiology and # Senior Consultant Cardiothoracic Surgery,
Indraprastha Apollo Hospital, New Delhi.

A 32-year old gentleman presented with history of swelling of feet for two weeks. There was no pain, redness involving the lower limbs. The patient did not have any breathlessness, cough or fever. There was no other symptom to suggest involvement of any other organ system at all. There was no predisposing factor for venous thrombosis. Clinical examination did not reveal any significant finding except for bilateral pedal edema. Jugular venous pulse was not elevated.
On echocardiography, a lobulated mass attached to right atrial wall was seen which was extending in to the inferior vena cava and causing dilatation of inferior vena cava (figures 1 and 2). Rest of the echocardiographic examination was normal. There was no evidence of deep vein thrombosis on lower limb venous duplex scanning. On extensive investigations, no evidence was found to show involvement of any other organ system. Endomyocardial biopsy was taken from this mass which revealed it to be a neoplasm.

What are the likely possibilities?

1. Myxoma
2. Angiosarcoma
3. Rhabdomyoma
4. Lymphoma
5. Carcinoid
6. Papillary fibroelastoma

Answer:
The two most likely diagnoses in such a situation would be- angiosarcoma and lymphoma. Both these tumors are more common in males than in females and have special predilection for right side of the heart, particularly right atrium. Right heart failure is the most common presenting symptom of both of them. Of these two, angiosarcoma is much more common whereas primary lymphoma of the heart is rare. Angioscarcoma is the second most common primary cardiac tumor after myxoma and is the commonest tumor of right atrium.
Myxomas usually originate from left atrium and are most often attached to interatrial septum. In majority of cases, myxomas are pedunculated tumors. Systemic symptoms are common in patients with cardiac myxomas.
Rhabdomyoma is a tumor of children and originates from the myocardium of either of the ventricles.

Papillary fibroelastomas are the most common tumors of heart valves and are usually seen in elderly.
Carcinoid disease almost exclusively affects right side of the heart. However, cardiac involvement is usually in an infiltrative form with inflow portion of right ventricular being affected the most often. Tricuspid valve dysfunction is the most common cardiac manifestation of carcinoid disease.

Figure 1: Large, lobulated mass (arrows) seen in right atrium along its free wall.

Figure 2: Extension of mass in to inferior vena cava- right atrial junction (arrows) with dilatation of inferior vena cava

As already mentioned, the patient described above underwent endomyocardial biopsy of the tumor which revealed angiosarcoma. Twelve cycles of chemotherapy were given which resulted in partial resolution of the tumor. The patient is now undergoing further treatment.

Correspondence: Ravi R Kasliwal,Senior Consultant Cardiology, # 4262, Department of Cardiology, Indraprastha Apollo Hospital,
Sarita Vihar, New Delhi-110076. INDIA
Email: rrkasliwal@hotmail.com, Ph: +91-11-26925858, Fax: +91-11-41402511