Angiosarcoma of - Heart · comaor haemangioendotheliosarcoma,3410 although...

Br Heart J 1984; 51: 94-7

Angiosarcoma of the heartUnusual presentation and survival after treatmentDAG S0RLIE, EIVIND S P MYHRE, HELGE STALSBERG

From the Cardiovascular Section, Department ofSurgery, Section of Cardiology, Department ofInternal Medicine,and Department of Pathology, University Clinic, Tromso, Nonvay, and The Norwegian Radium Hospital, Oslo,Norway

SUMMARY A 49 year old man presented with severe cyanosis and dyspnoea on exercise. Clinicalexamination together with echocardiography, cardiac catheterisation, and angiography showed aballoting tumour in the right atrium, intermittently occluding the tricuspid ostium, and an atrialright to left shunt. At operation a pedunculated vascular tumour was found with a broad base whichwas embedded in the atrial wall and continued into the interventricular septum. Histological exami-nation showed angiosarcomatous features and signs of a less than radical excision. The patient, whomade an uneventful recovery, was given postoperative radiotherapy. After 36 months there are nosigns of recurrence or metastasis.

Primary tumours of the heart are rare, and in reviewof 480 331 necropsies Straus and Merliss' found anincidence of only 0-17 per 1000. Angiosarcoma isamong the least common of such tumours,2 and thesigns and symptoms with which it presents are welldocumented.34 The most common symptoms aremalaise, chest pain, fever and haemoptysis, while thephysical signs include raised central venous pressureand a precordial friction rub. The tumour usuallyarises from the right atrium3 5 and only rarely fromother parts of the heart.67 The diagnosis in reportedcases has usually been made at necropsy or immedi-ately before death.3 When operation has been attemp-ted signs of inoperability have usually been encoun-tered, and only a few patients have survived for morethan six months.8

Case report

In June 1980 a 49 year old male truck driver wasadmitted to hospital. During the previous two years,he had become increasingly dyspnoeic during exerciseand he had noted bluish discoloration of the skin dur-ing physical activity. On examination he was strik-ingly cyanotic, and even on minor exercise thecyanosis increased and he became breathless. Thepatient was in sinus rhythm at a heart rate of 84beats/min and his blood pressure was 120/100mm Hg; slight clubbing of the fingers was present. Atall a wave in the jugular venous pulse was noted. Onauscultation a diastolic murmur with a distinct presys-

tolic component was audible in the fourth intercostalspace by the left parasternal border and an early ejec-tion systolic murmur was weakly audible in the samearea. Neither hepatomegaly nor dependent oedemawas found.The chest x ray film showed a normal heart

configuration and normal lung fields. Pulmonaryspirometry and pulmonary scintigram were normal.Blood haemoglobin concentration was 20-4 g/100 ml,the packed cell volume 67%, and the erythrocytesedimentation rate 1 mm in the first hour. Pao2 was41*3 mm Hg (5-5 kPa) and PaCo2 was 25.2 mm Hg(3.35 kPa). The electrocardiogram showed P waveswith amplitude exceeding 0*25 mV in limb leads IIand III and precordial lead Vi together with depres-sion of the PR segment. The graded bicycle ergometertest had to be discontinued after 3 minutes at 48 Wbecause the patient became breathless and increas-ingly cyanotic. Heart rate rose to 118 beats/min dur-ing exercise.The echocardiogram showed a space occupying

structure in the right atrium moving into the tricuspidorifice during diastole (Fig. 1). Cardiac catheterisa-tion showed tricuspid stenosis with a pressure differ-ence between the right atrium and right ventricle of8 mm Hg, increasing to 20 mm Hg during atrial sys-tole. A defect in the atrial septum and a pressure gra-dient of about 7 mm Hg from right to left atrium wasalso found. By the hydrogen method no left to rightshunt could be detected. The fraction of total venousreturn shunted from right to left atrium was 43% (cal-

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Fig. 1 M moe echocardiogram of the tricuspul valve (TV).Tumour echoes (T7) are seen behind the le~'ets. The echoproducing structure nearlyfills the ostium during diastole.

culated from oxygen saturations in blood samplesfrom the right atrium, the pulmonary vein, and thefemoral artery). Angiocardiography also showed aspace occupying structure in the right atrium movingpartially into the right ventricle (Fig. 2) during dias-tole and a right to left shunt at the atrial level.

During operation on total cardiopulmonary bypasswith cold cardiac arrest, a movable tumour fillingalmost the whole right atrium was found. The fora-men ovale was wide open and the tricuspid orifice waswidened. The tumour was round with a diameter ofabout 7 cm and was coloured blue grey with swollenveins. The oval base had a diameter of about 3 cm andthe tumour continued below the fibrous trigone intothe intraventricular septum, from which a roundedand rather well defined protuberance was enucleated.The cutting edges were apparently into normalmyocardial tissue, and no obvious infiltrative growthwas seen. Neither the mitral apparatus, the bundle ofHis, the coronary sinus, nor the circumflex branch ofthe left coronary artery was damaged. The foramenovale and the inflicted atrial septal defect were closed

Fig. 2 Angiocardiogram, right oblique view. Tumour (T), leftatrium (LA) and right atrium (RA), and the atrial right to leftshunt (arrow).

with running monofilamental nylon 4-0. The patientmade an uneventful recovery and experiencedimmediate subjective functional improvement. Theexcised tumour (Fig. 3) had a distinct external cap-sule and histological examination showed a well dif-ferentiated haemangiosarcoma, with vascular spaceslined with atypical endothelium and partly filled witherythrocytes (Fig. 4). A few mitoses and scattered

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Fig. 3 Photograph of resected tumour. Arrozws show theborderline between the intramyocardial and thefree pedunculatedparts.

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Sorlie, Myhre, Stalsberg

Fig. 4 Light micrograph ofparaffin embedded tumour tissue.Vascular cavities covered by spindleshaped ceUs with aypical nuclearpattem and pleomorphism are seen.Haematoxylin and eosin stain(original magn#ication x 350).

large nuclei were seen, although in general only mod-erate nuclear pleomorphism was present. Tumour tis-sue had infiltrated into fat tissue and heart muscle andextended to the resection border. Because the resec-tion was probably incomplete postoperativeradiotherapy was given (Dr 0 Solheim). The irradia-tion was planned on the basis of computed tomogra-phy and given with photon beams by a 16 MV linearaccelerator. Two oblique anterior fields with wedgefilters included most of the heart. The total dose in thecritical tissue was 50 Gy. During the radiation treat-ment electrocardiograms were normal until theaccumulated dose of 40 Gy was reached. At that timethe T waves became negative in the precordial leadsV2 and V3, but as the importance of these changeswas uncertain, the irradiation was continued.

Subsequent clinical examinations, together withechocardiography and computed tomography of theheart, have not shown any signs of tumour recurrenceor metastasis 36 months after the operation. Thepatient is in good health.

Discussion

Besides being a rare tumour, the angiosarcoma in thispatient had two rare features: it presented withunusual clinical symptoms and was successfully tre-ated by surgery and radiotherapy. The preoperativediagnosis was an intra-atrial balloting tumour whichintermittently obstructed the tricuspid ostium. Theraised right atrial pressure had effected an atrial rightto left shunt through the foramen ovale, explaining

the considerable cyanosis on effort. A case of atrialmyxoma with a similar clinical picture has beenreported,9 and this diagnosis was considered mostlikely. At operation, however, it was apparent that thetumour was not a myxoma but was probably malig-nant. A wide excision was not easily performed espe-cially along the extension into the interventricularseptum. The intracardiac defect was easily repaired,and apart from a temporary tricuspid insufficiency nopostoperative sequelae have been detected.The histological picture was typical of angiosar-

coma or haemangioendotheliosarcoma,3 410 althoughnot highly undifferentiated. Pleomorphism and mito-tic activity were moderate. Infiltrating growth alongthe line of resection indicated less than radical exci-sion, and postoperative radiotherapy was consideredappropriate. "1 12

In an extensive study on previously reported car-diac angiosarcoma (56 cases) Grontoft and Hellquist3state that no other type of sarcoma is so strictlylocated to the right atrium. In their study, the meanage of the patients at diagnosis was 41 years and thetumour was three times as common in men as inwomen. Only seven cases were diagnosed beforedeath, with an average survival of six months, and65% had distant metastases at necropsy. Peduncu-lated growth, without early infiltration and distantmetastases, as described in this report, is obviouslyrare.

This case shows that cardiac angiosarcomata maypresent clinically like myxomata and that treatmentcan be successful.

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References

1 Straus R, Merliss R. Primary tumor of the heart. ArchPathol 1945; 39: 74-8.

2 Rosman HS, Goodwin JF, Cleland WP, Bentall HH.Two decades of atrial tumour. Eur Heart J 1982; 3:100-6.

3 Grontoft 0, Hellquist H. Cardiac haemangioen-dotheliosarcoma. Acta Pathol Microbiol Scand [A] 1977;85A: 33-44.

4 Shackell M, Mitko A, Williams PL, Sutton GC.Angiosarcoma of the heart. Br Heart J 1979; 41: 498-503.

5 Morlino T, Carbognin S, Causarano D, Peranzoni PF,Vincenzi M. Angiosarcoma of the right atrium. Clinicaland pathological study of one case. G Ital Cardiol 1980;10: 229-32.

6 Datta BN, Khattri HN. Angiosarcoma of pulmonaryvein-left atrium junction [Letter]. Arch Pathol Lab Med1979; 103: 363.

7 Lin TK, Stech JM, Eckert WG, Lin JJ, Farha, SJ,Hagan CT. Pericardial angiosarcoma simulating pericar-

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dial effusion by echocardiography. Chest 1978; 73: 881-3.

8 Mavroudis C, Way LW, Lipton M, Gerz EW, Ellis RJ.Diagnosis and operative treatment of intracavitaryliposarcoma of the right ventricle. J Thorac CardiovascSurg 1981; 81: 137-40.

9 Talley RC, Baldwin BJ, Symbas PN, Nutter DO. Rightatrial myxoma. Unusual presentation with cyanosis andclubbing. Am J Med 1969; 48: 256-60.

10 Gough JC, Connolly CE, Kennedy JD. Primary sarcomaof the heart: a light and electron microscopic study of twocases. J Clin Pathol 1979; 32: 601-7.

11 Graham WJ, Bogardus CR Jr. Angiosarcoma treatedwith radiation therapy alone. Cancer 1981; 48: 912-4.

12 Bjerregaard P, Baandrup U. Haemangioendothelio-sarcoma of the heart. Diagnosis and treatment. Br HeartJ 1979; 42: 734-7.

Requests for reprints to Dr Dag S0rlie, Cardiovascu-lar Section, Department of Surgery, 9012 Region-sykehuset i Troms0, Norway.



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