ww.sciencedirect.com

i n d i a n h e a r t j o u r n a l 6 4 ( 2 0 1 2 ) 4 1 6e4 1 9

Available online at w

journal homepage: www.elsevier .com/locate / ih j

Case report

Left atrial spindle cell sarcoma e Case report

Nihar Mehta a, Ajit Desai b,c,*, Bharat Shivdasani b, Satyajeet Suryawanshi a,A.B. Mehta d, A. Behranwala e, Boman Dhabhar f

aSenior Registrar, Department of Cardiology, Jaslok Hospital and Research Centre, 15 Dr. G. D. Deshmukh Marg,

Mumbai 400026, IndiabConsultant Cardiologist, Jaslok Hospital, 15 Dr. G. D. Deshmukh Marg, Mumbai 400026, IndiacConsultant Cardiologist, Nanavati Hospital, Indiad Interventional Cardiologist, Director of Cardiology, Jaslok Hospital and Research Centre, 15 Dr. G. D. Deshmukh Marg,

Mumbai 400026, IndiaeConsultant Cardiosurgeon, Jaslok Hospital and Research Centre, 15 Dr. G. D. Deshmukh Marg, Mumbai 400026, IndiafConsultant Oncologist, Jaslok Hospital and Research Centre, 15 Dr. G. D. Deshmukh Marg, Mumbai 400026, India

a r t i c l e i n f o

Article history:

Received 4 April 2012

Received in revised form

9 May 2012

Accepted 15 June 2012

Available online 22 June 2012

Keywords:

Spindle cell sarcoma

Left atrium

Recurrence

* Corresponding author. Department of Car2266573333, þ91 9821016642.

E-mail address: dr_ajitd@hotmail.com (A0019-4832/$ e see front matter Copyright ªhttp://dx.doi.org/10.1016/j.ihj.2012.06.008

a b s t r a c t

Primary spindle cell sarcoma of the left atrium is an extremely rare tumour. Surgical

excision is the mainstay of treatment since it responds poorly to chemotherapy or radio-

therapy. In spite of all the treatment, the prognosis remains poor due to inadvertent delay

in diagnosis, few therapeutic options and propensity to metastasize. We present a 47-year-

old male who underwent a surgical excision of a left atrial mass in February 2010. It was

proved to be a high-grade spindle cell sarcoma on histopathology. He presented again in

October 2010 with recurrence of the tumour for which he was re-operated. However, the

tumour recurred again within one month, to which the patient succumbed.

Copyright ª 2012, Cardiological Society of India. All rights reserved.

1. Introduction sarcomas have a poor prognosis in spite of surgical

The most common type of benign primary cardiac tumours

is myxomas. The most common malignant type of primary

cardiac tumours is sarcomas.1,2 Both myxomas and

sarcomas often have a similar presentation. Therefore

many sarcomas are misdiagnosed as myxomas. However

both these types of cardiac tumours behave quite

distinctly. Myxomas can be completely resectable and

therefore have an excellent prognosis. On the other hand,

diology, Jaslok Hospital,

. Desai).2012, Cardiological Socie

resection.1,2

There are very few case reports of primary spindle cell

sarcomas of the left atrium from India.3 We would like to

presentacaseofa leftatrial spindlecell sarcomawhopresented

with a recurrence within 6e7 months of his first surgery and

again 1 month after his second surgery. Based on initial work

up, he was diagnosed to have a left atrial myxoma but his

histopathology revealed spindle cell sarcoma. Thus accurate

diagnosis is extremely important in determining theprognosis.

15 Dr. G. D. Deshmukh Marg, Mumbai 400026, India. Tel.: þ91

ty of India. All rights reserved.

Fig. 1 e Pre operative echocardiography showing a left

atrial mass.

i n d i a n h e a r t j o u rn a l 6 4 ( 2 0 1 2 ) 4 1 6e4 1 9 417

2. Case report

A 47-year-old male, presented on 26th February 2010 with

symptoms of heaviness in the chest on exertion, dyspnoea on

exertion NYHA grade II and giddiness for 1 week. Therewas no

variation of symptoms related to posture. There were no asso-

ciated symptoms of syncope or cough. There were no consti-

tutional symptoms like fever, weight loss or appetite loss.

On examination, the patient had a heart rate of 94 beats per

minute, regular; blood pressure was 122/70 mmHg, supine.

Cardiovascular system revealed a normal first and second

heart sound with a grade II/III, soft, low pitched, mid diastolic

murmur without any thrill. His respiratory, abdominal and

neurological examinations were unremarkable.

The patient’s haematological and serological investiga-

tions were within normal limits. An electrocardiogram

showed sinus tachycardia. 2D Echocardiography showed

a large solitary mass arising from the lateral wall of the left

atrium, close to the inter-atrial septum. It was broad based,

occupying nearly the entire left atrium and was mobile. It

prolapsed into the left ventricle during diastole. The left

ventricular ejection fraction was 60%. There was mild mitral

regurgitation. There was no pericardial effusion.

On 27th February 2010, the patient underwent surgery for

excision of the left atrial mass via a median sternotomy.

Intraoperatively, the tumour was found to be adhearant to the

postero-lateral wall of the left atrium, extending into the

pulmonary veins. Its consistencywas firm to hard. A near total

excision was done without excising any part of the atrial wall.

There was no extension into the myocardium, pericardium or

other adjacent structures. Features which raised doubt about

the benign nature of this mass were that it was arising from to

the postero-lateral wall of the left atrium unlike myxomas

which usually arise from the inter-atrial septum, its consis-

tency was firm to hard, it extended into the pulmonary veins

and it was adherent to the atrial wall.

The histopathology revealed a 5 � 3 � 3 cm firm polypoid

smooth mass, which on microscopy showed an apparent

sarcomatous tumour composed of spindle shaped cells in

flowing fascicles supported by a myxomatous matrix. This

was diagnosed as a high-grade spindle cell sarcoma of the left

atrium.

Post operatively the patient’s symptoms improved and he

was subsequently discharged.

Follow up Echocardiography in May 2010 showed no

residual mass and a left ventricular ejection fraction of 55%.

However, an echocardiography done in September 2010

showed a 2.4� 1.2 cm sessile, broad basedmass occupying the

left atrium, arising from the lateral wall, not reaching up to the

mitral valve with trivial mitral regurgitation. There was no

pericardial effusion. The patient failed to take the recom-

mended treatment at that time.

On 16th October 2010, the patient was readmitted with

symptoms of sudden onset breathlessness NYHA grade II

since 3e4 days, which rapidly progressed to NYHA grade IV

within 4 days. There was no postural variation of the

symptoms.

On examination, the patient had a heart rate of 128 beats

per minute, regular; blood pressure was 110/78mmHg supine.

Cardiovascular system revealed a normal first and second

heart sound with a short mid diastolic murmur and no other

sounds.

A 2D Echocardiography showed a 5 � 3 cm mass arising

from the lateral wall of the left atrium, close to the inter-atrial

septum impinging on the mitral valve and prolapsing into the

left ventricle during diastole. It was sessile, broad based and

occupying nearly the entire left atrium. There was no peri-

cardial effusion (Fig. 1). A CT Scan of the chest and abdomen

showed a 6.2 � 3.3 � 5.0 cm soft tissue space occupying lesion

occupying almost the entire left atrial chamber, bilateral

moderate pleural effusion (left > right sided) & peri-hilar

pulmonary congestion.

The patient was re-operated on 18th October 2010 and the

mass in the left atrium was excised via a right anterolateral

thoracotomy. The tumour was hard in consistency. It arose

from the postero-lateral wall of the left atrium andwas tightly

adherent to the atrial wall which precluded total excision of

the tumour. There was no involvement of the myocardium or

pericardium. Hence debulking of the mass was carried out to

relieve the obstructive symptoms. The histopathology report

revealed a 7 � 5 � 5 cm nodular mass (Fig. 2), which on

microscopy showed a fairly well circumscribed cellular

tumour consisting of bundles of spindle shaped cells sepa-

rated by myxoid stroma (Fig. 3). The nuclei of the cells were

pleomorphic and exhibited 15e20 mitosis per ten high power

fields. The tumour cells stained positively with Vimentin. CD

34, SMA and Desmin were negative. The diagnosis was

a poorly differentiated spindle cell sarcoma of the left atrium.

The patient improved symptomatically. The post operative

Echocardiography showed no residual mass in the left atrium

(Fig. 4). The patient had an uneventful recovery and was dis-

charged on the 23rd of October 2010.

Within one month of discharge, the patient developed

recurrent symptoms of breathlessness on exertion and was

readmitted on 18th November 2010. An Echocardiography

showed a 2.8 � 3.0 cm mass in the left atrium causing left

ventricular inflow obstruction (Fig. 5). The patient was started

on a palliative chemotherapy consisting of Dacarbazine and

Fig. 2 e Gross pathology of cardiac spindle cell sarcoma.

Fig. 4 e Post operative echocardiography (four chamber

view).

i n d i a n h e a r t j o u r n a l 6 4 ( 2 0 1 2 ) 4 1 6e4 1 9418

Ifosfamide. The patient refused any further surgical inter-

vention and expired on 27th November 2010 due to Cardiac

failure and Nosocomial Pneumonia.

3. Discussion

Primary cardiac neoplasms are rare with an incidence varying

between 0.001 and 0.28%.1 Metastatic disease of the heart is

20e40 times more common than primary cardiac neoplasms.

Among primary cardiac tumours, approximately 75% are

benign of which cardiac myxomas account for majority. The

remaining 25% of cardiac neoplasms are malignant; sarcomas

being the most common, accounting for half to three quarters

of the cases.2 Benign cardiac tumours are more common on

the left side of the heart whereas malignant ones are more

common on the right side of the heart.

Malignant primary cardiac tumours include angiosarco-

mas, rhabdomyosarcomas, leiomyosarcomas, fibrosarcomas,

malignant fibrous histocytomas, undifferentiated sarcomas

and malignant lymphomas. Primary cardiac angiosarcoma is

the most common primary cardiac sarcoma in adults and

rhabdomyosarcoma is the most common primary cardiac

sarcoma in children. Cardiac spindle cell sarcomas are

Fig. 3 e Histopathology of cardiac spindle cell sarcoma.

classified as undifferentiated sarcomas. They lack a specific

histology, with negative immunohistochemistry for desmin,

factor VIII, and keratin. They typically involve the left atrium,

and may extend to the pericardium resulting in a hemoper-

icardium. This is in contrast to angiosarcomas, which have

a predilection for the right atrium.4,5

Cardiac sarcomas can have a varied presentation. They can

present with sudden onset, intermittent or positional symp-

toms of chest discomfort, dyspnoea, orthopnoea, cough or

syncope. They may present with heart failure, pericardial

effusion and tamponade, arrythmias, valvular abnormalities,

obstructive symptoms depending on the chamber of the heart

involved. Embolic episodes resulting in systemic emboli, brain

being the most common site or pulmonary emboli can be

a presenting feature.2 Non-specific constitutional symptoms

such as fever, fatigue and weight loss can occur in 10%

without cardiac symptoms.1 Frequently, sarcomas present

Fig. 5 e Recurrence of left atrial mass (parasternal long axis

view).

i n d i a n h e a r t j o u rn a l 6 4 ( 2 0 1 2 ) 4 1 6e4 1 9 419

with metastasis, common sites being lung, liver, bone, lymph

nodes, adrenal and brain. Rarer presentations include supe-

rior vena cava syndrome, myocardial infarction or cardiac

rupture. On examination, an audible tumour plop may be

heard due to protrusion of the tumour through the mitral

valve. There may be murmurs that may have positional vari-

ation or signs of left or right-sided cardiac failure. Due to their

rarity, they may bemisdiagnosed as mitral stenosis or cardiac

myxomas.

The diagnosis is usually established using non-invasive

modalities such as trans-thoracic or trans-esophageal echo-

cardiography. CT scan and cardiacMRI are useful in predicting

the malignant nature of the cardiac mass. Tumour location,

tissue composition and pericardial or pleural effusions are key

predictors of lesion type.6 Features suggestive of malignancy

are broad based lesions, lesions occupying the entire cardiac

chamber, infiltration of the myocardium, pericardium or

adjacent structures, hilar lymphadenopathy, necrosis and

metastases. Definitive diagnosis is made by tissue biopsy,

which may be obtained by transvenous or percutaneous

biopsy, endoscopy, mediastinoscopy or thoracotomy.

However, individual subtypes of cardiac sarcomas do not

affect the outcome. Prognosis depends on the grade of the

tumour, which is based on the mitotic activity, necrosis and

degree of cellular differentiation. High mitotic activity (>5

mitotic figures/10 high power fields), extensive tumour

necrosis and poor cellular differentiation have a worse

prognosis.2

Cardiac sarcomas have poor prognosis with a mean

survival of less than 1 year. In rare cases prolonged survival

has been documented.7 Surgical debulking is the mainstay of

the treatment. It serves as palliative treatment to relieve

symptoms. Bench surgery (explantation and auto-

transplantation of the heart) has been tried to achieve

complete resection of the tumour.8 The role of orthotropic

heart transplantation for malignant cardiac tumours is

controversial.9 Neo-adjuvant chemotherapy to reduce the

tumour mass and facilitate surgical excision can be tried.

Adjuvant chemotherapy (with regimens combining several

agents, usually including doxorubicin) or radiation therapy

may play a role in some cases. In spite of treatment, the

prognosis remains poor with the most common cause of

death being recurrence of the tumour.

Conflicts of interest

All authors have none to declare.

r e f e r e n c e s

1. Chahinian A Philippe, Gutstein David E, Fuster Valentin.Chapter 95 tumors of the heart and great vessels. In:Kufe Donald W, Pollock Raphael E, Weichselbaum Ralph R,et al., eds. Holland-Frei Cancer Medicine. 6th ed. 2003.

2. McManus Bruce, Lee Cheng-Han. Chapter 69 primary tumorsof the heart. In: Libby Peter, Bonnow Robert O, Mann Douglas L,et al., eds. Braunwald’s Heart Disease. 8th ed. 2008:1815e1828.

3. Bishnoi SK, Bardia A, Yadav V, et al. Primary cardiac sarcoma.Indian J Med Paediatr Oncol. 2007;28:40e43.

4. Jassal DS, Thakrar A, Neilan TG, et al. Cardioembolic stroke ina patient with spindle cell sarcoma of the left atrium. J Am SocEchocardiogr. 2007 Apr;20(4):438.e1e438.e4.

5. Shanmugan G. Primary cardiac sarcoma. Eur J CardiothoracSurg. 2006;29:925e932.

6. Hoffmann Udo, Globits Sebastian, Schima Wolfgang, et al.Usefulness of magnetic resonance imaging of cardiac and paracardiac masses. Am J Cardiol. Oct 2003;92(7):890e895.

7. Modi Amit, Lipnevicius Arturas, Moorjani Narain, et al.Prolonged survival with left atrial spindle cell sarcoma. InteractCardiovasc Thorac Surg. 2009;8:703e704.

8. Hoffmeier A, Deiters S, Schmidt C. Radical resection of cardiacsarcoma. Thorac Cardiovasc Surg. Apr 2004;52(2):77e81.

9. Jimenez Mazuecos JM, Fuentes Manso R, Segovia Cubero J,et al. Is heart transplantation for primary cardiac sarcomaa useful therapeutic option? Rev Esp Cardiol. Apr2003;56(4):408e411.