British Heart_Journal, I970, 32, 575-578.

Wilms' tumour propagated through theinferior vena cava into the right heart cavities

Guillermo Anselmi, Jose A. Suarez, Ivain Machado, Federico Moleiro, andPablo BlancoFrom the Sections of Pediatric Cardiology and Hemodynamics, Department of Cardiology,University Hospital, and the Institute of Pathology and Laboratory of ExperimentalCardiology, Ciudad Universitaria, Caracas, Venezuela

A report is made of a rare case of Wilms' tumour which simulated clinically a syndrome of'right heart failure', asfound in some cases of Ebstein's disease. The clinical, radiological, andelectrocardiographic study led to the suspicion of this type of malformation. The cine-angio-graphic study revealed two important facts: the impossibility of approaching the inferior vena

cava with opaque material injected into the azygos vein and a filling defect of the right atrium.The post-mortem study revealed the presence of a Wilms' tumour of the right kidney whichextended into the inferior vena cava and into the right atrium without producing metastases.This seems to be the first case of this peculiar course of a Wilm's tumour which has grown intothe right atrium, resulting in this bizarre clinical picture.

The present report refers to an unusual caseof Wilms' tumour which originated in theright kidney and through the renal vein, in-vaded the inferior vena cava, and ascendedinto the right atrium, without producingmetastases. This gave rise to a peculiar heartcondition, exhibiting clinical similarities withsome features of Ebstein's malformations ofthe tricuspid valve.

Case reportA 4-year-old boy was admitted to the Departmentof Pediatrics of the University Hospital becauseof enlargement of the abdomen, oedema of thelower extremities, oliguria, and vesical tenesmus.He was the product of a normal pregnancy andfull-term delivery. The past medical history wasunremarkable. He had ascites 4 months beforeadmission and oedema of the lower limbs 2months before.

Physical examination disclosed a slightly jaun-diced patient. His height was I02 cm.; he weighedI7-5 kg. He had ascites and moderate oedema ofthe legs. The jugular veins were distended; aprominent 'a' wave was a striking feature. A leftparasternal impulse lift was palpable. Auscultationof the heart disclosed a soft regurgitant systolicmurmur (2/6) over the tricuspid area which in-creased with inspiration. A 4th sound was audibleover the apex, and a fixed and closely split secondsound at the pulmonary area. The liver was I3 cm.below the costal margin.The electrocardiogram showed peaked P waves

measuring 5-5 mm. voltage in lead II, suggestingsystolic overloading of the right atrium.

The x-ray films (Fig. iA, B) revealed conspicu-ous right atrial enlargement.At cardiac catheterization a No. 7F Lehman

catheter was inserted into the right saphenousvein. Several attempts to advance the catheter intothe inferior vena cava were unsuccessful. Thecatheter was withdrawn into a lower positionwhere the radiopaque substance was injected. Acine-angiocardiogram (Fig. iC, D) showed ab-sence of the inferior vena cava, filling of theazygos and hemiazygos veins, with multiple anas-tomoses between both vessels; filling of thesuperior vena cava and the right atrium whichwas conspicuously enlarged. The tricuspid valvewas displaced upward and to the left. At first afilling defect in the right atrium was not recog-nized. The venous pressure was 20 mm. Hg andthe arterial 02 saturation 99 per cent.

Laboratory examinations revealed that urin-alysis, bilirubin, total and fractionate proteinswere normal.The patient improved with treatment for heart

failure and was discharged from hospital.One month later he was readmitted with vomit-

ing and dyspnoea; his liver again was very en-larged, the heart rate was i60/min.; 4 hours laterthe patient died in cardiac arrest after resuscitationprocedures had proved unsuccessful.

Necropsy The most important finding was atumour of the right kidney, which deformed theorgan; it measured 9 x 6 x 5-5 cm.; it had anirregular surface partly smooth and partly dented.It did not infiltrate the perirenal tissue. On sectionthere was a whitish tissue which replaced 8o percent of the right kidney, and invaded the right

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576 Anselmi, Suarez, Machado, Moleiro, and Blanco

FIG. I X-ray, top: frontal (A) and left oblique (B) positions showing important enlargementof the right atrial appendage. Bottom: Cine-angiocardiographic study. (C) The filling ofthe superior vena cava through the azygos vein and the anastomoses between the azygos andhemiazygos veins. (D) A filling defect in the right atrium and displacement of thetricuspid valve are apparent.

renal vein, distending and obliterating it. It ex-tended into the inferior vena cava (Fig. 2A) and itentered the right atrium which it filled. Further-more, it bulged into the right ventricle at the levelof the atrioventricular valve (Fig. 2C); no signs ofinvasion of the wall of the renal vein or inferiorvena cava were observed. There were no meta-stases.The heart showed moderate dilatation and hy-

pertrophy of the right ventricle and a great dilata-tion of the right atrium (Fig. 2B, C); the leftcavities were normal. The liver was enlarged andcongested; the remaining organs were unremark-able.

The microscopical study of the renal tumour(Fig. 2D), showed a structure formed by embry-onic connective tissue, clearly of tumoral aspect;in the centre of it there were tubular irregularformations, a small lumen, covered with columnarepithelium of limited cytoplasm and of irregularnucleus situated at different levels: some of thesetubules did not have lumina and appeared as red-dish spots similar to metanephric tubules of therenal blastema. The tumour was formed mostlyby embryonic connective tissue, areas of myxoidor adipose tissue and disseminated smooth musclefibres. The periphery showed a thin band of col-lagen tissue forming a capsule around the tumour.

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Wilms' tumour propagated through the inferior vena cava into the right heart cavities 577

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FIG. 2 Anatomical specimen and histology. (A) Posterior view of the thoracic and abdo-minal organs. Note the right kidney enlarged, with the tumour and a grossly distended inferiorvena cava. (B) Note the enlargement of the right atrium and atrial appendage. Congestivehepatomegaly. (C) Right atrium and ventricle opened: the encapsulated tumour protrudesthrough the atrium obstructing the tricuspid orifice. (D) Microscopically the tumour presentedundifferentiated tumoral tissue in the middle of which two metanephric tubular-like structurescan be seen.

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These features are consistent with the diagnosisof a nephroblastoma (Wilms' tumour). The en-capsulated growth which entered the renal vein,the inferior vena cava, and the right atrium, andthe absence of metastases were remarkable.

DiscussionAccording to Schaffer (I960), Wilms' tumouris the second most common of the abdominaltumours in children. It is an embryonic tu-mour which originates from the primitivemesenchyma from which the kidney is formed.At birth it may present a certain amount ofgrowth, and gives rise to symptoms almostalways before 5 years of age. When the tu-mours become palpable they are already ofconsiderable size, and have usually given riseto metastases. The prognosis of the tumourand the surgical results are poor. At the be-ginning the tumour is encapsulated; but itsoon metastasizes, particularly to the brain,the liver, and the lungs. It is rare for thenephroblastoma to make progress through therenal vein and inferior vena cava into the rightheart cavities, as shown by Macgregor (I960)and in our case. To date we have not found adescription of the clinical findings and thecardiovascular signs of similar cases. The en-capsulated nephroblastoma progressed upinto the right heart cavities via the inferiorvena cava, which it obstructed: an uncommonfact, since obstructions of the inferior venacava have been reported mainly in adults, andaccording to Deutsch et al. (I968) they arecaused by leiomyosarcomas and other primaryvenous tumours. When the obstruction of theinferior vena cava occurs in the middle thirdof the vessel (Lopez et al., I968; Missal,Robinson, and Tatum, I965), a nephroticsyndrome frequently develops, a complicationthat was not present in our patient, despitethe obstruction of the inferior vena cava fromthe level of the renal veins upwards. The ob-struction of the upper third of the inferiorvena cava produces the Budd-Chiari syn-drome characterized by hepatosplenomegaly,ascites, jaundice, and hepatic insufficiency.Our patient presented with hepatomegaly

and ascites but not jaundice, hepatic insuffici-ency, or splenomegaly. The clinical findingswere suggestive of 'right heart failure', asfound in the final phases of some cases of Eb-stein's malformation of the tricuspid valve,

when there is significant stenosis of the tri-cuspid orifice, with great dilatation of the rightatrium.

Partial absence of the inferior vena cava isrelatively frequent in association with certaincongenital malformations, and they are com-monly suspected when the catheter insertedin the femoral vein cannot be advanced. Aninjection of contrast medium in a low positionwill show the filling of the azygos vein empty-ing into the superior vena cava, or of thehemiazygos vein with its accessory vein, emp-tying into the persistent left superior venacava. In our patient, the venous angiographyshowed 'absence of the inferior vena cava',the contrast material ascended via the azygosvein into the superior vena cava, and fromthere it entered the right atrium; this led usto believe that there was partial absence ofthe inferior vena cava in association with Eb-stein's malformation of the tricuspid valve.Assuming that the interpretation of the fillingdefect of the right atrium in the cine-angio-cardiogram was suggestive ofan atrial tumour,it is still difficult to explain the obstruction be-neath the inferior vena cava and to relate itto the same cause. As far as we have been ableto see in the literature, we have not found acase of Wilms' tumour with this peculiar be-haviour. There are reported cases of primarytumours of the inferior vena cava which pro-gress into the right atrium, though to dateonly one, a case of leiomyosarcoma in anadult, has been diagnosed by venous angio-graphy (Deutsch et al., I968).

Deutsch, V., Fraenkel, 0., Frand, U., and Hulu, N.(I968). Leiomyosarcoma of the inferior vena cavapropagating into the right atrium. British HeartJournal, 30, 571.

L6pez, J. E., Martinez, F., Gubaira, J. A., and Arocha,R. (I968). Sindrome nefr6tico producido por ob-strucci6n de la vena cava inferior. Acta MedicaVenezolana, 15, 2I8.

Macgregor, A. R. (I960). Pathology of Infancy andChildhood. E. & S. Livingstone, Edinburgh andLondon.

Missal, M. E., Robinson, J. A., and Tatum, R. W.(I965). Inferior vena cava obstruction. Clinicalmanifestations, diagnostic methods, and relatedproblems. Annals of Internal Medicine, 62, 133.

Schaffer, A. J. (I960). Diseases of the Newborn. W. B.Saunders, Philadelphia and London.

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