Unusual intracardiac metastasis of a testicular embryonal carcinoma

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cases. Normal postoperative perfusion of the repaired lung can be documented without the risks and expense of cardiac catheterization. The doses of radiation incurred by the patient and staff performing radionuclide angiography are quite small relative to those of cineangiography. When preoperative radionuclide angiography is performed in conjunction with comprehensive cross-sectional endocardiography, the need for cineangiography prior to surgery for anomalous origin of a pulmonary artery from the ascending aorta may be lessened if not eliminated. As in the case reported here, repeat radionuclide angiograms postoperatively can document normal pulmonary perfusion after repair of the anomalous pulmonary artery.

References Keane JF, Maltz D, Bernhard WF. Corwin RD. Nadas AS. Anomalous origin of one pulmonary artery from the ascending aorta. Circulation 1974;50:588-594. Rice MJ, Seward JB, Hagler DJ, et al. Impact of 2-dimensional echocardiography on the management

of distressed newborns in whom cardiac disease is suspected. Am J Cardiol 1983;51:288-292. King DH, Huhta JC, Gutgesell HP, Ott DA. Two-dimensional echocardiographic diagnosis

of anomalous origin of the right pulmonary artery from the aorta: differentiation from aorto-pulmonary window. J Am Co11 Cardiol 1984;4:351-356. Smallhorn JF, Anderson RH, McCartney FJ. Two dimensional echocardiographic assessment of communications between ascending aorta and pulmonary trunk or individual pulmonary arteries. Br Heart J 1982;47:563-572. Rabinovitch M. Keane JF, Norwood WI, Castaneda AR, Reid L. Vascular structure in lung tissue obtained at biopsy correlated with pulmonary hemodynamic findings after repair of congenital heart defects. Circulation 1984;69:655-667. Thomason CB, Rao BR. Lung imaging - unilateral absence or near absence of pulmonary perfusion on lung scanning. Sem Nucl Med 1983;13:388-390. Long WA, Lawson EE, Perry JR, Harned HS Jr, Henry GW. Radionuclide diagnosis of infradiaphragmatic total anomalous pulmonary venous drainage. Pediat Cardiol. in press.

IJC 0291D

Unusual intracardiac metastasis of a testicular embryonal carcinoma Stefania Maione ‘, Anna Giunta ’ and Lucia Agozzino 2 ’ I Clinica Medica, Second School of Medicine, University of Naples, Naples, Italy; and ’ Istituto di Anatomia Patologica, First School of Medicine, University of Naples, Naples, Italy (Received

13 April 1984; revision accepted

29 January

1985)

In this report, we present a case of a metastasis of an emhryonal carcinoma, unusually in the right atrium, which was clinically revealed by a systemic paradox embolism through an atrial septal defect. The primary tumor was found in an undescended testis.

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Correspondence and reprint requests to: Prof. Lucia Agozzino, Medicina e Chirurgia, Via Armanni 5, 80138 Napoli, Italia. International Journal of Cardiology, 8 (1985) 496-500 0 Elsevier Science Publishers B.V. (Biomedical Division)

Istituto

di Anatomia

Patologica,

I FacoltB di

497

(Key words: intracardiac secondary cardiac tumor)

masses;

cross-sectional

echocardiography;

embryonal

carcinoma;

Introduction

Cardiac metastasis from a testicular tumor is very uncommon. In the Harvard Cancer Commission report of 4375 autopsies performed in patients dying of cancer [l]. 146 (3.4%) exhibited cardiac metastases. which were secondary to a testicular tumor in only 2 cases. The present case was unique for the unusual location of the metastasis [2] and for the uncommon macroscopic appearance of the tumor. Case Report

An apparently healthy 2%year-old man had a sudden stroke resulting in a right hemiparesis. The patient immediately underwent left carotid arteriography which revealed luminal occlusion without parietal anatomic alterations, suggesting an embolic source of the stroke. Physical examination revealed normal heart size, sinus tachycardia (120 beats/min), S, gallop and systolic and diastolic murmurs best heard across the sternum. There was no jugular venous distension, hepatomegaly or peripheral edema. The chest radiograph showed a normal cardiac silhouette and clear lung fields. The electrocardiogram showed a bilateral atria1 enlargement. M-mode echocardiography disclosed dense masses of echoes in the right ventricle which were posterior to the tricuspid valve during the latter part of diastole. The right ventricular cavity was slightly enlarged and paradoxical septal motion was present. Cross-sectional echocardiography showed a huge and mobile mass which. in diastole. crossed the tricuspid tunnel and nearly filled the right ventricular cavity. This was suggestive of a pedunculate right atria1 myxoma. Contrast echocardiography was consistent with presence of an atria1 septal defect (Fig. 1). The patient was sent to cardiac surgery for removal of the right atria1 mass.

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Fig. 1. M-mode echocardiograpbic feature of the tumor which is represented by band-like echoes filling the right ventricle during diastole. The area behind the tricuspid valve is relatively free of echoes during systole. After a peripheral venous injection of contrast solution, a cloud of echoes is visualized in the right ventricle in systole (upper arrow) and subsequently appears in the left ventricular cavity. entering through the mitral valve orifice (lower arrow). This flow pattern strongly suggests a right-to-left shunt at atria1 level.

498 Morphologic Findings At surgery, a milk-white tumor measuring cm 7 X 8 X 6 and weighing 102 g was found, with a papillary surface and a short broad-based stalk (Fig. 2). Histological examination revealed that the papillae consisted of a core of a loose, often mixoid tissue with fibrinous and thrombotic or hemorrhagic areas covered by unbroken endocardium. Some papillae contained sheets of large, pleomorphic cells arranged in solid or pseudoglandular pattern. The cytoplasm of these cells was often vacuolated without distinct cellular borders. The nuclei tended to be rounded or vesicular and showed a coarse irregular membrane, varying degrees of vacuolization and one or more prominent nucleoli. Small intra- and extracellular eosinophilic hyaline droplets were focally present which were faintly PAS positive. The cells were arranged in small or large papillary, tubular and acinar structures. Mitotic patterns and mononuclear or multinuclear giant cells were frequent. Some isolated or grouped syncytiotrophoblastic giant cells and embryoid-like disks were also seen (Fig. 2b). A positive reaction for alphafetoprotein (peroxidase antiperoxidase technique according to Taylor [3]) was found in some cells. It consisted of a granular, brown, intracytoplasmic deposit localized within mononuclear embryonal cells or small clusters. The distribution was focal, either in single cells or small clusters. Syncytiotrophoblastic giant cells and embryoid bodies did not react with this technique. Some syncytiotrophoblastic giant cells were faintly stained with the human chorionic gonadotropin, but the cells which were positive for alphafetoprotein failed to react for gonadotropin, indicating that the two protein antigens were localized in different cells. Hyaline droplets were negative for both antigens. Primary Tumor After the pathological study of the excised cardiac mass, a diagnostic work-up was performed to discover the primary tumor. Computerized tomography of the chest and abdomen together with bone scans failed to reveal any primary mass. Since the patient had an undescended testis, it was suspected as the tumor source and was surgically removed. A nodular mass, of the size of 2 x 3 x 5 mm was found. The cut surface showed a variegated appearance and little evidence of encapsulation. The epididymis and cords were not invaded. The histological examination revealed an embryonal carcinoma. No syncytiotrophoblastic cells were present. The patient died suddenly one year later and autopsy was not performed. Discussion Cardiac metastasis of embryonal carcinomas is rare [4]. The first uncommon finding in this case, therefore, is spread to the right atrium from the distant testicular tumor. Indeed, intracavitary detection of neoplastic thrombi is rare. The known metastasis to the right atrium from kidney and liver carcinomas is generally secondary to direct invasion via the inferior caval vein. The other interesting finding is the macroscopic pattern of the tumor which resembled a papillary myxoma or endocardiac fibroelastoma. Regarding pathogenesis, two different hypotheses can be proposed. The neoplastic cells may have reached the right atrium directly via the inferior caval vein with subsequent endocardiac implantation. Alternatively, they could have passed through the coronary circulation with subendocardial metastasis and secondary thrombosis. As with other endocardial papillary lesions, the thrombotic mass probably increased in size as a result of deposition of successive layers of thrombotic material. The papillary configuration would be acquired gradually as additional thrombotic material was added in some zones and lost in others [5].

Fig. 2. a. External appearance Microscopic view: embryoid

of the surgical specimen showing the papillomatous surface of the tumor. b. like disk. Giant cells at the left suggest a syncytiotrophoblast (HE. X 250)

500

On the other hand the mass could have increased by tumor proliferation, the papillae being subsequently invaded by the tumor cells. Since roughly 75% of the coronary flow returns to the right atrium (through the thebesian and coronary veins) embolic tumor cells have more opportunity to lodge on the right than on the left side). Finally, we must point to the presence of an atria1 septal defect as a likely source of the systemic paradox embolism which revealed the tumor and the usefulness of contrast echocardiography in its detection.

Acknowledgements We thank Drs. Massimo Chiariello and Vinceruo Santinelli, from the Department of Medicine, Second School, University of Naples, Drs. Luigi De Luca and Michelangelo Scardone, Department of Cardiac Surgery, First School of Medicine, University of Naples, for their help in clinical diagnosis and follow-up of the patient.

References 1 Prichard RW. Tumors of the heart. Review of the subjects and report of one hundred and fifty cases. Arch Path01 1951;51:98-128. 2 Gallenstein PE, Balluscio RL, Berger M, Onseng F, Jurado RA. Right to left intracardiac shunt: a unique presentation of metastatic cardiac disease. J Am Co11 Cardiol 1984;3:865-867. 3 Taylor CR, Bums J. The demonstration of plasma cells and other immunoglobulin containing cells in formalin fixed, paraffin embedded tissue using peroxidase-labelled antibodies. J Clin Pathol 1974;27:14-20. 4 Hanfling SM. Metastatic cancer to the heart. Review of the literature and report of 127 cases. Circulation 1960;22:474-483. 5 Sayler WR, Page DL, Hutchins G. The development of cardiac myxomas and papillary endocardial lesions from mural thrombus. Am Heart J 1975;89:4-17.