Medical and Pediatric Oncology 6:313-316 (1979)
Wilms Tumor With Extension to the Right Atrium Svein Kolmannskog, MD, Peter J. Moe, MD, Emmanuel Besigye, MD, and Dag Sdrlie, MD Departments of Pediatrics (S.K., P.J.M.), Radiology (E.B.), and Surgery (D.S.), University of Tromsd,Norway
A seven-year-old boy had cardiac symptoms for five months before the diagnosis of Wilms tumor was made. The tumor extended into the inferior vena cava through the renal vein and protruded into the right atrium. All demonstrable tumor masses could be removed at a one-step surgical procedure without using cardiopulmonary bypass. Intensive postoperative chemotherapy has been given, and 15 months after surgery he was without signs of relapse. Key words: Wilms tumor, cardiac involvement, preoperative diagnosis
INTRODUCTION
Wilms tumor has been reported to invade the renal vein in 8-40% of patients [l-31. Further extension into the inferior vena cava is reported in 4.5-10% of these cases [ 2 ] .Involvement of the heart, however, is extremely rare. Thirteen cases of Wilms tumor have been reported propagating into the right heart cavities through the renal vein and the inferior vena cava [l-101 . Only eight of the 13 cases were diagnosed preoperatively [l-71, four were discovered at autopsy [8,9], and one was diagnosed during open-heart surgery for removal of a cardiac tumor [lo]. A preoperative diagnosis of cardiac involvement was known in only five cases [2-61. This is a report about a seven-year-old boy who presented with cardiac symptoms and signs. A preoperative diagnosis of Wilms tumor with cardiac extension was made. CASE REPORT
S.A.H., a male child born in June 1970, was admitted to a local hospital in May 1977 because of vomiting, fever, and diffuse abdominal pain. Clinical examination revealed a boy without cyanosis nor edema; n o cardiac murmur was heard. He had tachycardia, and the liver was transitorily enlarged. The sedimentation rate and the values of the transaminases were increased. An electrocardiogram showed low voltage; myocarditis was suspected. He improved without treatment, and the electrocardiogram was normal before discharge. Address reprint requests to Svein Kolmannskog, Department of Pediatrics, University of Tromsb, 9012 TromsQ,Norway.
0098-1532/79/0604-0313$01.10 0 1979 Alan R. Liss, Inc.
314
Kolmannskog et a1
Five months later he was admitted t o this hospital with a tentative diagnosis of endocarditis. His physical activity had decreased during the preceding two months, and his mother reported that he apparently had lost weight. Physical examination in November 1977 revealed a 7 4/12-year-old boy in rather poor condition. He was pale, his height was 125 cm (50th%), and he weighed only 20 kg (1 kg below 2.5th%). He was dyspneic during activity but had n o cyanosis and no edema. Blood pressure was normal. Auscultation of the heart disclosed a diastolic murmur in the left third intercostal space along the sternal border. The liver edge was palpated 7 cm below the right costal margin. The possibility of a myxoma in the right atrium, affecting the tricuspid valve, was discussed. Evaluation
Routine laboratory studies, including urinalysis, and a chest radiograph were within normal limits. An electrocardiogram showed low voltage. Cardiac catheterization was then attempted. A catheter was inserted into the right femoral vein, but stopped in the inferior vena cava at the level of the kidneys, and a contrast injection revealed an obstruction. Thereafter, intravenous pyelography, venacavography (Fig. l), and renal angiography (Fig. 2) showed a right-sided renal tumor and occlusion of the inferior vena cava.
Fig. 1 . Venacavography which shows the obstruction (arrow) of the inferior vena cava just above the right renal hilus.
Fig. 2. Selective renal angiography disclosed a hypovascular tumor (arrow), marginated by a superior capsular vein. The right renal vein is obstructed (transsected arrow).
Preoperative Chemotherapy
It was suspected that a Wilms tumor had propagated into the renal vein and inferior vena cava up t o the right heart cavities. Preoperatively the patient was given a single dose of vincristine (2 mg/m*), and dactinomycin (1 5 pg/kg) daily for four days.
Wilms Tumor Extending to the Heart
315
Surgery
A right thoracoretroperitoneal approach was used. The thoracic cavity was entered through the tenth intercostal space, whereafter the right diaphragm was completely separated from its attachment, including the medial crux. Displacement of the thoracic and abdominal contents forward and to the left, exposed the entire inferior vena cava. The tumor masses extended from the renal vein into the inferior vena cava and entered the right atrium. All four hepatic veins were obliterated. The inferior vena cava was ligated at the bifurcation, and the right renal vein was divided, after which the right kidney with its tumor was removed. The inferior vena cava was then incised at its dorsolateral aspect, between the hepatic veins, all the way up to the pericardial sac, which also was opened, and the tumor masses were removed. Some blood flow through the two superior hepatic veins was achieved. The thrombus entering the atrium was removed en bloc through open exposure without application of vascular clamps (CVP: 6 cm HzO, head down). The thrombus might have interfered with tricuspid function. Finally the inferior vena cava was reconstructed down below the hepatic veins. Histology showed a poorly differentiated nephroblastoma. Postoperative Course and Chemotherapy
On the first and eighth days of every month during the first half year the patient received vincristine (1.5 mg/m2), Adriamycin (30 mg/m2), dactinomycin (0.3 mg/m2) and cyclophosphamide (200 mg/m2). Since then he has received the same drug combination once a month. Three times this treatment has been postponed for two weeks because of bone marrow depression. No irradiation has been given. Clinical examination 15 months postoperatively revealed a boy in a generally good condition. His height was 132 cm (50th%) and he weighted 24.5 kg (2.5 kg below the loth%). He was not dyspneic during activity, had no cardiac murmur, and his liver was palpated 2 cm below the costal margin. The electrocardiogram was normal, and clinical examination and computed tomography of his abdomen did not show any local recurrence of the tumor. DISCUSS1ON
This is the fourteenth reported case of Wilms tumor extending into the right heart cavities. Seven children, including our patient, had cardiac murmur over the tricuspid area [ 1,4-7,9], four had no murmur [2,3,6,10], and it was unknown whether or not there was a tricuspid murmur in three cases [ 8 ] . A correct diagnosis is easily missed, probably because of the rarity of the condition and the dominating cardiac symptoms in these patients. This is, in fact, the sixth reported case of Wilms tumor propagating into the right atrium in which the diagnosis of the cardiac involvement was made preoperatively. The type of tumor was not obvious preoperatively, as it was a rather small kidney tumor, despite a five months’ history of cardiac symptoms. The possibility of the extremely rare carcinoma of the kidney in that age group could not be completely excluded. It was therefore decided to do an exploratory operation before the child was given intensive cytostatic and radiation therapy. One course of vincristine and dactinomycin was given preoperatively to minimize the risk of further metastases in connection with surgical intervention. A correct preoperative localization of the primary tumor and its extension was important both as an indication for surgery and the choice of surgical approach.
3 16
Kolmannskog et al
Only one of the 13 reported cases survived more than one year after surgery [l] . Our patient has been treated vigorously with a four-drug regimen. He was without signs of relapse 15 months after surgery, and therefore he has a reasonable chance of survival. Irradiation has not so far been given because of the extent and nature of the involved area. Recent promising results of chemotherapy show that aggressive surgery may be indicated, despite conveiitional criteria of inoperability. In spite of the rarity of Wilms tumor with extension to the right heart cavities, the possibility of a primary kidney tumor should always be considered when a cardiac tumor is diagnosed, particularly in a child with a cardiac murmur and obstructed inferior vena cava. Also, when a Wilms tumor is diagnosed, such noninvasive methods as echocardiography and computed tomography should be done in the routine evaluation to exclude cardiac involvement. REFERENCES 1. Murphy DA, Rabinovitch H, Chevalier L, Virmani S: Wilms’ tumor in right atrium. Am J Dis Child 126:210, 1973. 2. Schullinger JN, Santulli TV, Casarella WJ, MacMillan RW: The role of right heart angiography in the management of selected cases. Ann Surg 185:451, 1977. 3. Vaughan ED, Crosby IK, Tegtmeyer CJ: Nephroblastoma with right atrial extension: Preoperative diagnosis and management. J Urol 117:530, 1977. 4. Farooki ZQ, Green EW, Arciniegas E: Echocardiographic pattern of right atrial tumour motion. Br Heart J 38:580, 1976. 5. Farooki ZQ, Henry JG, Green EW: Echocardiographic diagnosis of right atrial extension of Wilms’ Tumour. Am J Cardiol 36:363, 1975. 6. Slovis TL, Cushing B, Reilly BJ, Farooki ZQ, Philippart A l , Berdon WE, Baker DH, Reed JO: Wilms’ tumor to the heart: Clinical and radiographic evaluation. Am J Roentgen01 131:263, 1978. 7. Anselmi G , Suarez JA, Machado I, Moleiro F, Blanco P: Wilms’ tumour propagated through the inferior vena cava into the right heart cavities. Br Heart J 32:575, 1970. 8. Nadas AS, Ellison RC: Cardiac tumors in infancy. Am J Cardiol 21:363, 1968. 9. Aytac A, Tuncali T, Tinaztepe K , Ikizler C, Saylam A: Metastatic Wilms’ tumor in the right atrium propagated through the inferior vena cava. Vasc Surg 10:268, 1976. 10. Utley JR, Mobin-Uddin K , Segnitz RH, Belin RP, Utley JF: Acute obstruction of tricuspid valve by Wilms’ tumor. J Thorac Cardiovasc Surg 66:626, 1973.
Wilms Tumor With Extension to the Right Atrium Svein Kolmannskog, MD, Peter J. Moe, MD, Emmanuel Besigye, MD, and Dag Sdrlie, MD Departments of Pediatrics (S.K., P.J.M.), Radiology (E.B.), and Surgery (D.S.), University of Tromsd,Norway
A seven-year-old boy had cardiac symptoms for five months before the diagnosis of Wilms tumor was made. The tumor extended into the inferior vena cava through the renal vein and protruded into the right atrium. All demonstrable tumor masses could be removed at a one-step surgical procedure without using cardiopulmonary bypass. Intensive postoperative chemotherapy has been given, and 15 months after surgery he was without signs of relapse. Key words: Wilms tumor, cardiac involvement, preoperative diagnosis
INTRODUCTION
Wilms tumor has been reported to invade the renal vein in 8-40% of patients [l-31. Further extension into the inferior vena cava is reported in 4.5-10% of these cases [ 2 ] .Involvement of the heart, however, is extremely rare. Thirteen cases of Wilms tumor have been reported propagating into the right heart cavities through the renal vein and the inferior vena cava [l-101 . Only eight of the 13 cases were diagnosed preoperatively [l-71, four were discovered at autopsy [8,9], and one was diagnosed during open-heart surgery for removal of a cardiac tumor [lo]. A preoperative diagnosis of cardiac involvement was known in only five cases [2-61. This is a report about a seven-year-old boy who presented with cardiac symptoms and signs. A preoperative diagnosis of Wilms tumor with cardiac extension was made. CASE REPORT
S.A.H., a male child born in June 1970, was admitted to a local hospital in May 1977 because of vomiting, fever, and diffuse abdominal pain. Clinical examination revealed a boy without cyanosis nor edema; n o cardiac murmur was heard. He had tachycardia, and the liver was transitorily enlarged. The sedimentation rate and the values of the transaminases were increased. An electrocardiogram showed low voltage; myocarditis was suspected. He improved without treatment, and the electrocardiogram was normal before discharge. Address reprint requests to Svein Kolmannskog, Department of Pediatrics, University of Tromsb, 9012 TromsQ,Norway.
0098-1532/79/0604-0313$01.10 0 1979 Alan R. Liss, Inc.
314
Kolmannskog et a1
Five months later he was admitted t o this hospital with a tentative diagnosis of endocarditis. His physical activity had decreased during the preceding two months, and his mother reported that he apparently had lost weight. Physical examination in November 1977 revealed a 7 4/12-year-old boy in rather poor condition. He was pale, his height was 125 cm (50th%), and he weighed only 20 kg (1 kg below 2.5th%). He was dyspneic during activity but had n o cyanosis and no edema. Blood pressure was normal. Auscultation of the heart disclosed a diastolic murmur in the left third intercostal space along the sternal border. The liver edge was palpated 7 cm below the right costal margin. The possibility of a myxoma in the right atrium, affecting the tricuspid valve, was discussed. Evaluation
Routine laboratory studies, including urinalysis, and a chest radiograph were within normal limits. An electrocardiogram showed low voltage. Cardiac catheterization was then attempted. A catheter was inserted into the right femoral vein, but stopped in the inferior vena cava at the level of the kidneys, and a contrast injection revealed an obstruction. Thereafter, intravenous pyelography, venacavography (Fig. l), and renal angiography (Fig. 2) showed a right-sided renal tumor and occlusion of the inferior vena cava.
Fig. 1 . Venacavography which shows the obstruction (arrow) of the inferior vena cava just above the right renal hilus.
Fig. 2. Selective renal angiography disclosed a hypovascular tumor (arrow), marginated by a superior capsular vein. The right renal vein is obstructed (transsected arrow).
Preoperative Chemotherapy
It was suspected that a Wilms tumor had propagated into the renal vein and inferior vena cava up t o the right heart cavities. Preoperatively the patient was given a single dose of vincristine (2 mg/m*), and dactinomycin (1 5 pg/kg) daily for four days.
Wilms Tumor Extending to the Heart
315
Surgery
A right thoracoretroperitoneal approach was used. The thoracic cavity was entered through the tenth intercostal space, whereafter the right diaphragm was completely separated from its attachment, including the medial crux. Displacement of the thoracic and abdominal contents forward and to the left, exposed the entire inferior vena cava. The tumor masses extended from the renal vein into the inferior vena cava and entered the right atrium. All four hepatic veins were obliterated. The inferior vena cava was ligated at the bifurcation, and the right renal vein was divided, after which the right kidney with its tumor was removed. The inferior vena cava was then incised at its dorsolateral aspect, between the hepatic veins, all the way up to the pericardial sac, which also was opened, and the tumor masses were removed. Some blood flow through the two superior hepatic veins was achieved. The thrombus entering the atrium was removed en bloc through open exposure without application of vascular clamps (CVP: 6 cm HzO, head down). The thrombus might have interfered with tricuspid function. Finally the inferior vena cava was reconstructed down below the hepatic veins. Histology showed a poorly differentiated nephroblastoma. Postoperative Course and Chemotherapy
On the first and eighth days of every month during the first half year the patient received vincristine (1.5 mg/m2), Adriamycin (30 mg/m2), dactinomycin (0.3 mg/m2) and cyclophosphamide (200 mg/m2). Since then he has received the same drug combination once a month. Three times this treatment has been postponed for two weeks because of bone marrow depression. No irradiation has been given. Clinical examination 15 months postoperatively revealed a boy in a generally good condition. His height was 132 cm (50th%) and he weighted 24.5 kg (2.5 kg below the loth%). He was not dyspneic during activity, had no cardiac murmur, and his liver was palpated 2 cm below the costal margin. The electrocardiogram was normal, and clinical examination and computed tomography of his abdomen did not show any local recurrence of the tumor. DISCUSS1ON
This is the fourteenth reported case of Wilms tumor extending into the right heart cavities. Seven children, including our patient, had cardiac murmur over the tricuspid area [ 1,4-7,9], four had no murmur [2,3,6,10], and it was unknown whether or not there was a tricuspid murmur in three cases [ 8 ] . A correct diagnosis is easily missed, probably because of the rarity of the condition and the dominating cardiac symptoms in these patients. This is, in fact, the sixth reported case of Wilms tumor propagating into the right atrium in which the diagnosis of the cardiac involvement was made preoperatively. The type of tumor was not obvious preoperatively, as it was a rather small kidney tumor, despite a five months’ history of cardiac symptoms. The possibility of the extremely rare carcinoma of the kidney in that age group could not be completely excluded. It was therefore decided to do an exploratory operation before the child was given intensive cytostatic and radiation therapy. One course of vincristine and dactinomycin was given preoperatively to minimize the risk of further metastases in connection with surgical intervention. A correct preoperative localization of the primary tumor and its extension was important both as an indication for surgery and the choice of surgical approach.
3 16
Kolmannskog et al
Only one of the 13 reported cases survived more than one year after surgery [l] . Our patient has been treated vigorously with a four-drug regimen. He was without signs of relapse 15 months after surgery, and therefore he has a reasonable chance of survival. Irradiation has not so far been given because of the extent and nature of the involved area. Recent promising results of chemotherapy show that aggressive surgery may be indicated, despite conveiitional criteria of inoperability. In spite of the rarity of Wilms tumor with extension to the right heart cavities, the possibility of a primary kidney tumor should always be considered when a cardiac tumor is diagnosed, particularly in a child with a cardiac murmur and obstructed inferior vena cava. Also, when a Wilms tumor is diagnosed, such noninvasive methods as echocardiography and computed tomography should be done in the routine evaluation to exclude cardiac involvement. REFERENCES 1. Murphy DA, Rabinovitch H, Chevalier L, Virmani S: Wilms’ tumor in right atrium. Am J Dis Child 126:210, 1973. 2. Schullinger JN, Santulli TV, Casarella WJ, MacMillan RW: The role of right heart angiography in the management of selected cases. Ann Surg 185:451, 1977. 3. Vaughan ED, Crosby IK, Tegtmeyer CJ: Nephroblastoma with right atrial extension: Preoperative diagnosis and management. J Urol 117:530, 1977. 4. Farooki ZQ, Green EW, Arciniegas E: Echocardiographic pattern of right atrial tumour motion. Br Heart J 38:580, 1976. 5. Farooki ZQ, Henry JG, Green EW: Echocardiographic diagnosis of right atrial extension of Wilms’ Tumour. Am J Cardiol 36:363, 1975. 6. Slovis TL, Cushing B, Reilly BJ, Farooki ZQ, Philippart A l , Berdon WE, Baker DH, Reed JO: Wilms’ tumor to the heart: Clinical and radiographic evaluation. Am J Roentgen01 131:263, 1978. 7. Anselmi G , Suarez JA, Machado I, Moleiro F, Blanco P: Wilms’ tumour propagated through the inferior vena cava into the right heart cavities. Br Heart J 32:575, 1970. 8. Nadas AS, Ellison RC: Cardiac tumors in infancy. Am J Cardiol 21:363, 1968. 9. Aytac A, Tuncali T, Tinaztepe K , Ikizler C, Saylam A: Metastatic Wilms’ tumor in the right atrium propagated through the inferior vena cava. Vasc Surg 10:268, 1976. 10. Utley JR, Mobin-Uddin K , Segnitz RH, Belin RP, Utley JF: Acute obstruction of tricuspid valve by Wilms’ tumor. J Thorac Cardiovasc Surg 66:626, 1973.
