Case report 56
Wilms' Tumor with Intracardiac Extension G. Martinez-Guerra,]. Ruano-Aguilar, R. Rivera-Luna, R. Cardenas-Cardos, E. Avila-Ramirez, G. BraunRoth, E. Altamirano-Alvarez, A. Moreno-Hidalgo, E. L. Flamand-Rodriguez Departments of Pedialric Onco!og.l'. Surgical Onco!og.l'. Palhology. :"Juclear Medicine and Cardiovascular Surgery. Inslilulo NacionaJ de Pediatria. Mexico
Abreviations
Intracardiac tumor extension from nephroblastoma is a rare eyent. \\'e report on t\\'o cases with this peculiar condition who presented with a different set of signs and symptom. Both were diagnosed in life but onJy one could be properly managed on time. Emphasis is made upon the most reJiable methodology for early detection and the surgical approach as the only plausible way to solve lhis particuIar complication.
ICTE = Inlracardiac tumor extension .W.T.S. = National WiIms' Tumor Study rvc = Inferior Vena Cava EKG = EJeclrocardiogram VCR = Vincristine Sulfale
Keywords tumor
.\Iots-eles Tumeur de Wilms - Nephroblastome - Metastase inlracardiaque
Abbreviations
WiIms' Intracardiac metastasis
ICTE: Extension lumorale inlracardiaque X W.T.S.: Elude nalionale sur les lumeurs de Wilms 1\'C: \'eine ca,'e inferieure EKG: Electrocardiogramme \'CH: Sulfale de ,incrisline
ephroblastoma
Resume L'extension tumorale intracardia que a partir d'un nephroblastome est rarement rencontree. Les auteurs rapportent deux cas qui presentaient la particularite de regrouper groupement differenl des signes et des symptomes differents. Les deux cas ont ere diagnostiques au cours de la vie mais un seul a etre traite directement atemps. L'accent est mis sur Ia methodologie la plus efficac~ pour une detection precoce et un traitement chirurgicaJ eule solution plausible pous resoudre cette complication particuliere.
Zusammenfassung Eine intrakardiale Ausbreitung von 1 ephroblastomen iSl außerordenUich selten. Häufiger ist die Ausbreilung im Bereiche der Vena cava. In der vorliegenden Arbeit ,vird über 2 Fälle berichtet, bei denen der Tumor sich intrakardial ausbreitete. Bei einem Patienten gelang es, den Tumor nach Exstirpation des Nephroblastoms zu entfernen, der andere Patient verstarb. Auf die lypischen Symplome einer intraventrikulären ephroblastommetastase wird hingewiesen. Abkürzungen ICTE: Intrakardiale Tumorau dehung NWTS: ationale WiIms-Tumor-Sludie rvC: Vena cava inferior EKG: Elektrokardiogramm VCR: Vincristin-Sulfat
Schlüsselwörter WiJms-Tumor - Nephroblasloma - Inlrakardiale .\letastase
Recei"ed February 19, 1991 Eur.J Pediatr Surg 2 (1992) 56-59 © Hippokrate \'erlag Stutlgart \tasson Editeur Pari
Downloaded by: NYU. Copyrighted material.
Summary
Wilms' 7lmwr with 1ntracardiac Extension
EurJ Pediatr Surg 2 (1992)
Introduction
In Mexico, nephrobla toma is the most common malignant olid tumor of the abdomen in childhood (10). Throughout the years, this tumor has been shown to have rather predictable behavior which will depend on a particular histologie subtype (5), however, in some instances this may change due to unusual events that can lead to a sudden cardiopulmonary arrest, secondary to an intracardiac lumor extension (ICTE); this has been reported to occur in less lhan 1 % (8). From 1971 10 1987, approximately 300 patienls \\'ilh this tumor ha\'e been regislered al our in lilulion and managed according 10 lhe 1 .W.T.S. crileria (USA.). The purpose of lhis paper is 10 describe lhe clinical presentation and final oulcome of lhe, in our experience, only lwo cases \\'ilh lCTE.
Case reports
57
Case2 A five-year-old male \\;th a large abdominal mass on the right flank was admitted. A Wilms' tumor of the right kidney was suspecled, after alilhe routine sludies; however, since the liver was elinicaJly enlarged, a liver scan was performed to rule oul infiltration of this gland. This method supported the original diagnosis of nephroblastoma of a large magnilude, displacing the liver downwards from behind and appparenUy fTee of lumor invasion (Fig. 4). The left kidney appeared normal but the venocavogram showed a lumor inlhe I\'C. Allhough lhe palienl's general condilion was apparenUy stahle, his blood pressure regislered high le\'els from the beginning (150/110 mm Hg) and lhe plalelel counts were quite low on repealed sam pIes (Iess lhan 30,000. mm 3 ); lhe rest of lhe coaguJalion tests were reported wilhin normallimits wilh lhe exceplion of prolonged prolhrombin and bleeding times. A bone marrow aspirale ruled oul lumor im'asion; therefore, we finally concluded lhal a plalelel "lrapping" phenomenon from the tumor lissue itself, was presenl. Allhis poinllhe palienl was considered as nol eligible for any kind of surgery. He therefore received a firsl dose of \'incrisline sulfale (\'CR) and, al lhe same lime, plalelel concenlrales and fresh plasma lransfusions. By lhe fourlh day, lhe plalelel counts re-
Case 1
At the presenl time (9 years from the original diagnosi ), the patient remains alive and fTee of di ease.
Fig, 1 Case 1. RadlonuclJde anglography Arrow shows the "cold spot" Image on the antenor wall 01 the nght ventnele
Downloaded by: NYU. Copyrighted material.
A e\'en-year-old female \\'as admitted \\ ilh an abdominal Illass in lhe left Oank. Alilhe com'enlional diagnoslic melhod poinled lo a Irilms'lumor. There was no e\'idence of lumor exlension lo lhe inferior \'ena ca\'a (I\'C). The palienl underwenllotal nephreclolllY and \\as lhen placed on a cheillolherapy/radiolherapy prolocol as for stage 111 of fa\'orable hislology, according lo lhe final palhologic repOJi. On lhe fiflh posl-operali\"e day lhe palienl suddenly de\"eloped severe hYPolension and irregular cardiac rh}ihm wilh Oattening ofT-wa\"es on lhe EKG. After a suspicious echocardiogralll, radionuclide angiography was performed, where a "cold spol" image was obsen'ed o\"er lhe wall of lhe righl \"enlriele (Fig. 1). The palienl was lhoughllo ha\"e an [CTE lo lhe righl chamber and hence was immedialely subjecled lo open hearl surgery. A lumor ma was found lo be lodged in lhe righl alrium as an exlension from the \\'C. The tumor was successfully remo\"ed altogether without complications (Figs. 2 and 3) and reported as lotally necrotic by the palhologist. The patient continued on her chemotherapy protocol for 15 months. Fig, 2
Case 1: Atnotomy with exposed tumoral thrombl.
Fig. 3
Case 1 Tumoral thrombl after extractlon.
EurJ Pediatr Surg 2 (1992)
Fig. 4 Case 2: Llver scan (lateral vlew). POlnted line marks the affected kidney.
G. Martinez-Guerra et al
Fig. 5 Case 2' Lung scan wlth pulmonary thromboembollsm. Arrow POints ou the umor In the rlgh a rlum
mained low and Lhe patient developed progressive edema of Lhe lower limbs and ehest pain wiLh dy pnea, suggesting a Lhromboembolie syndrome. A new ehest film supported Lhis eondition by howing cardiae enJargement wiLh pleural effusion. The patient was admitted to Lhe intensive-care unit for assisted ventilation among oLher relevant aids. A lung scan was eompatible wiLh severe pulmonary Lhrombo-embolism and showed a suspieious image of high density over Lhe right atrium (Fig.5). By now, all ECG's had failed to reveal any alteration and daetinomyein was initiated to potentiate Lhe VCR-effeet on Lhe primary a weil as Lhe pulmonary metastasis. Sinee an ICTE was now strongly su pected, surgica1 intervention became imperative. However, Lhe patient died a few hours later before any surgieal attempt could be offered. The post-mortem study disclosed Lhe primary tumor on the right kidney, apparentJy redueed in size and eontaining large areas of neerosis in the interior. The right renal vein and the entire IVC were exposed, revea1ing a massive tumor invasion and the right atrium totally oeeupied by asolid ma (Figs. 6 and 7). The left renal \'ein as weil as the suprahepatics eontained the ame tumor tissue. Both lungs were hea\i.ly eongested. The final pathologie-analysis eonfirmed a Wi/ms' tumor of favorable histology with large areas of neerosis_ The tumor Lissue within the atrium and the rest of the "essei was all necroLie \\'ith no identifiable tumor eells.
Fig. 6 Case 2' Post-mortem vlew of the affected rlght kldney and the Intracaval tumor extension
Downloaded by: NYU. Copyrighted material.
58
EurJ Pediatr Surg 2 (1992)
Wilms' TUmor with Intracardiac Extension
59
lions) and allleading to a critical condilion which ended in a few days with an irreversible state of shock and death, - despite all therapeutic efforts, including chemotherapy. From this experience we may conclude that every patient with a presumptive diagnosis of Wilms' tumor, regardless of the size of the primary of the clinical condition, has to be carefully studied with regard to the cardiopulmonary system, for the purpose of detecting a "silent" ICTE 01', ideally, to prevent it (if the IVC is already affected). We feelthat a simple echocardiogram is an excellent method to begin with, for that matter (1,3,4,7,9). An ICTE should be considered as areal emergency. We belieYe that, if any chemotherapy may help (by sterilizing the tumor, as it did with these two patients), it will e\identIy not be enough ta take care of the \\'hole problem, which is moslly mechanical. Surgery must therefore be performed as soon as possible before any major complication occurs (2, 6, 12).
,·Ic'il" E. .\l"rtinez GG I{adionuclide angiography' C\S a diagnostie method for \Yilms'tumor \\ith direet extension into the hearl. Pediatr I{adiol. 12 (IU82) 301-303 2 A)'I"e A. 71mcali T, TilwzlejJe I\., et al: .\letastatie \\'ilm5' tumor in the right atrium propagated through lhe inferior vena cava. \'ase Surg 10 (1976) 268 3 F"rookl ZQ. Henry JG, Green I\'E: Eehoeardiographie diagnosis of righl atrial extension of \\'ilms' lumor. Am J Cardiol 36 (1975) 363 1 Faroekl ZQ, Green \\'E, rlrcinlegas E: Eehocardiographie pallerns of righl atriallumor motion. BI' Ileart J 38 (1975) 580 5 Favara B, Ga/liani C, I\'akely P: c\dl'ances in lhe ca re of lhe child \\ilh cancer. Cancer 58 (1986) -126--1-11 6 Gasle GH, Gasle TB, Galanti S, M urphy S: Plalelet lumor cell inleractions in mice: The role of plalelels inthe spread of malignant disease. IntJ Cancer 11 (1973) 704 7 Murphy DA, RavillOvlch H, Chevalier L, et al: Wilms' tumor in right atrium. Am J Dis Child 126 (1973) 210-211 8 Nakayama D, Loremler A, O'Neill, et a1: lnlracardiac exlension of Wilms' tumor: Reporl of the Nalional Wilms' Tumor Study. Ann Surg 204 (1986)693-698 9 Ritchey ML, Kelalis PP, Breslow N, et a1: Int.racavaJ and alriaJ involvement with nephroblastoma: Review of national Wilms' tumor study - 3. J ofUrol J40 (1988) 1113-/1/8 10 Rlvera LR, Martinez GG: The problem of childhood cancer in a e1eveloping country. BuH Inl Ped Ass 7 (1987) 411-419 11 Schullinger ]N, Santuli TB, Casarella W], et al: The role of right hearl angiography in the management of seJecleei cases. Ann Surg 185 (1977) 451-455 12 Shunn S, Gauderer M, Dahms B, et a1: Fatal inlraoperalive puJmonary embolization of Wilms' lumor. J Peelialr 101 (1982) 559-563 13 \'augham ED, Crosby n
Wilms' Tumor with Intracardiac Extension G. Martinez-Guerra,]. Ruano-Aguilar, R. Rivera-Luna, R. Cardenas-Cardos, E. Avila-Ramirez, G. BraunRoth, E. Altamirano-Alvarez, A. Moreno-Hidalgo, E. L. Flamand-Rodriguez Departments of Pedialric Onco!og.l'. Surgical Onco!og.l'. Palhology. :"Juclear Medicine and Cardiovascular Surgery. Inslilulo NacionaJ de Pediatria. Mexico
Abreviations
Intracardiac tumor extension from nephroblastoma is a rare eyent. \\'e report on t\\'o cases with this peculiar condition who presented with a different set of signs and symptom. Both were diagnosed in life but onJy one could be properly managed on time. Emphasis is made upon the most reJiable methodology for early detection and the surgical approach as the only plausible way to solve lhis particuIar complication.
ICTE = Inlracardiac tumor extension .W.T.S. = National WiIms' Tumor Study rvc = Inferior Vena Cava EKG = EJeclrocardiogram VCR = Vincristine Sulfale
Keywords tumor
.\Iots-eles Tumeur de Wilms - Nephroblastome - Metastase inlracardiaque
Abbreviations
WiIms' Intracardiac metastasis
ICTE: Extension lumorale inlracardiaque X W.T.S.: Elude nalionale sur les lumeurs de Wilms 1\'C: \'eine ca,'e inferieure EKG: Electrocardiogramme \'CH: Sulfale de ,incrisline
ephroblastoma
Resume L'extension tumorale intracardia que a partir d'un nephroblastome est rarement rencontree. Les auteurs rapportent deux cas qui presentaient la particularite de regrouper groupement differenl des signes et des symptomes differents. Les deux cas ont ere diagnostiques au cours de la vie mais un seul a etre traite directement atemps. L'accent est mis sur Ia methodologie la plus efficac~ pour une detection precoce et un traitement chirurgicaJ eule solution plausible pous resoudre cette complication particuliere.
Zusammenfassung Eine intrakardiale Ausbreitung von 1 ephroblastomen iSl außerordenUich selten. Häufiger ist die Ausbreilung im Bereiche der Vena cava. In der vorliegenden Arbeit ,vird über 2 Fälle berichtet, bei denen der Tumor sich intrakardial ausbreitete. Bei einem Patienten gelang es, den Tumor nach Exstirpation des Nephroblastoms zu entfernen, der andere Patient verstarb. Auf die lypischen Symplome einer intraventrikulären ephroblastommetastase wird hingewiesen. Abkürzungen ICTE: Intrakardiale Tumorau dehung NWTS: ationale WiIms-Tumor-Sludie rvC: Vena cava inferior EKG: Elektrokardiogramm VCR: Vincristin-Sulfat
Schlüsselwörter WiJms-Tumor - Nephroblasloma - Inlrakardiale .\letastase
Recei"ed February 19, 1991 Eur.J Pediatr Surg 2 (1992) 56-59 © Hippokrate \'erlag Stutlgart \tasson Editeur Pari
Downloaded by: NYU. Copyrighted material.
Summary
Wilms' 7lmwr with 1ntracardiac Extension
EurJ Pediatr Surg 2 (1992)
Introduction
In Mexico, nephrobla toma is the most common malignant olid tumor of the abdomen in childhood (10). Throughout the years, this tumor has been shown to have rather predictable behavior which will depend on a particular histologie subtype (5), however, in some instances this may change due to unusual events that can lead to a sudden cardiopulmonary arrest, secondary to an intracardiac lumor extension (ICTE); this has been reported to occur in less lhan 1 % (8). From 1971 10 1987, approximately 300 patienls \\'ilh this tumor ha\'e been regislered al our in lilulion and managed according 10 lhe 1 .W.T.S. crileria (USA.). The purpose of lhis paper is 10 describe lhe clinical presentation and final oulcome of lhe, in our experience, only lwo cases \\'ilh lCTE.
Case reports
57
Case2 A five-year-old male \\;th a large abdominal mass on the right flank was admitted. A Wilms' tumor of the right kidney was suspecled, after alilhe routine sludies; however, since the liver was elinicaJly enlarged, a liver scan was performed to rule oul infiltration of this gland. This method supported the original diagnosis of nephroblastoma of a large magnilude, displacing the liver downwards from behind and appparenUy fTee of lumor invasion (Fig. 4). The left kidney appeared normal but the venocavogram showed a lumor inlhe I\'C. Allhough lhe palienl's general condilion was apparenUy stahle, his blood pressure regislered high le\'els from the beginning (150/110 mm Hg) and lhe plalelel counts were quite low on repealed sam pIes (Iess lhan 30,000. mm 3 ); lhe rest of lhe coaguJalion tests were reported wilhin normallimits wilh lhe exceplion of prolonged prolhrombin and bleeding times. A bone marrow aspirale ruled oul lumor im'asion; therefore, we finally concluded lhal a plalelel "lrapping" phenomenon from the tumor lissue itself, was presenl. Allhis poinllhe palienl was considered as nol eligible for any kind of surgery. He therefore received a firsl dose of \'incrisline sulfale (\'CR) and, al lhe same lime, plalelel concenlrales and fresh plasma lransfusions. By lhe fourlh day, lhe plalelel counts re-
Case 1
At the presenl time (9 years from the original diagnosi ), the patient remains alive and fTee of di ease.
Fig, 1 Case 1. RadlonuclJde anglography Arrow shows the "cold spot" Image on the antenor wall 01 the nght ventnele
Downloaded by: NYU. Copyrighted material.
A e\'en-year-old female \\'as admitted \\ ilh an abdominal Illass in lhe left Oank. Alilhe com'enlional diagnoslic melhod poinled lo a Irilms'lumor. There was no e\'idence of lumor exlension lo lhe inferior \'ena ca\'a (I\'C). The palienl underwenllotal nephreclolllY and \\as lhen placed on a cheillolherapy/radiolherapy prolocol as for stage 111 of fa\'orable hislology, according lo lhe final palhologic repOJi. On lhe fiflh posl-operali\"e day lhe palienl suddenly de\"eloped severe hYPolension and irregular cardiac rh}ihm wilh Oattening ofT-wa\"es on lhe EKG. After a suspicious echocardiogralll, radionuclide angiography was performed, where a "cold spol" image was obsen'ed o\"er lhe wall of lhe righl \"enlriele (Fig. 1). The palienl was lhoughllo ha\"e an [CTE lo lhe righl chamber and hence was immedialely subjecled lo open hearl surgery. A lumor ma was found lo be lodged in lhe righl alrium as an exlension from the \\'C. The tumor was successfully remo\"ed altogether without complications (Figs. 2 and 3) and reported as lotally necrotic by the palhologist. The patient continued on her chemotherapy protocol for 15 months. Fig, 2
Case 1: Atnotomy with exposed tumoral thrombl.
Fig. 3
Case 1 Tumoral thrombl after extractlon.
EurJ Pediatr Surg 2 (1992)
Fig. 4 Case 2: Llver scan (lateral vlew). POlnted line marks the affected kidney.
G. Martinez-Guerra et al
Fig. 5 Case 2' Lung scan wlth pulmonary thromboembollsm. Arrow POints ou the umor In the rlgh a rlum
mained low and Lhe patient developed progressive edema of Lhe lower limbs and ehest pain wiLh dy pnea, suggesting a Lhromboembolie syndrome. A new ehest film supported Lhis eondition by howing cardiae enJargement wiLh pleural effusion. The patient was admitted to Lhe intensive-care unit for assisted ventilation among oLher relevant aids. A lung scan was eompatible wiLh severe pulmonary Lhrombo-embolism and showed a suspieious image of high density over Lhe right atrium (Fig.5). By now, all ECG's had failed to reveal any alteration and daetinomyein was initiated to potentiate Lhe VCR-effeet on Lhe primary a weil as Lhe pulmonary metastasis. Sinee an ICTE was now strongly su pected, surgica1 intervention became imperative. However, Lhe patient died a few hours later before any surgieal attempt could be offered. The post-mortem study disclosed Lhe primary tumor on the right kidney, apparentJy redueed in size and eontaining large areas of neerosis in the interior. The right renal vein and the entire IVC were exposed, revea1ing a massive tumor invasion and the right atrium totally oeeupied by asolid ma (Figs. 6 and 7). The left renal \'ein as weil as the suprahepatics eontained the ame tumor tissue. Both lungs were hea\i.ly eongested. The final pathologie-analysis eonfirmed a Wi/ms' tumor of favorable histology with large areas of neerosis_ The tumor Lissue within the atrium and the rest of the "essei was all necroLie \\'ith no identifiable tumor eells.
Fig. 6 Case 2' Post-mortem vlew of the affected rlght kldney and the Intracaval tumor extension
Downloaded by: NYU. Copyrighted material.
58
EurJ Pediatr Surg 2 (1992)
Wilms' TUmor with Intracardiac Extension
59
lions) and allleading to a critical condilion which ended in a few days with an irreversible state of shock and death, - despite all therapeutic efforts, including chemotherapy. From this experience we may conclude that every patient with a presumptive diagnosis of Wilms' tumor, regardless of the size of the primary of the clinical condition, has to be carefully studied with regard to the cardiopulmonary system, for the purpose of detecting a "silent" ICTE 01', ideally, to prevent it (if the IVC is already affected). We feelthat a simple echocardiogram is an excellent method to begin with, for that matter (1,3,4,7,9). An ICTE should be considered as areal emergency. We belieYe that, if any chemotherapy may help (by sterilizing the tumor, as it did with these two patients), it will e\identIy not be enough ta take care of the \\'hole problem, which is moslly mechanical. Surgery must therefore be performed as soon as possible before any major complication occurs (2, 6, 12).
,·Ic'il" E. .\l"rtinez GG I{adionuclide angiography' C\S a diagnostie method for \Yilms'tumor \\ith direet extension into the hearl. Pediatr I{adiol. 12 (IU82) 301-303 2 A)'I"e A. 71mcali T, TilwzlejJe I\., et al: .\letastatie \\'ilm5' tumor in the right atrium propagated through lhe inferior vena cava. \'ase Surg 10 (1976) 268 3 F"rookl ZQ. Henry JG, Green I\'E: Eehoeardiographie diagnosis of righl atrial extension of \\'ilms' lumor. Am J Cardiol 36 (1975) 363 1 Faroekl ZQ, Green \\'E, rlrcinlegas E: Eehocardiographie pallerns of righl atriallumor motion. BI' Ileart J 38 (1975) 580 5 Favara B, Ga/liani C, I\'akely P: c\dl'ances in lhe ca re of lhe child \\ilh cancer. Cancer 58 (1986) -126--1-11 6 Gasle GH, Gasle TB, Galanti S, M urphy S: Plalelet lumor cell inleractions in mice: The role of plalelels inthe spread of malignant disease. IntJ Cancer 11 (1973) 704 7 Murphy DA, RavillOvlch H, Chevalier L, et al: Wilms' tumor in right atrium. Am J Dis Child 126 (1973) 210-211 8 Nakayama D, Loremler A, O'Neill, et a1: lnlracardiac exlension of Wilms' tumor: Reporl of the Nalional Wilms' Tumor Study. Ann Surg 204 (1986)693-698 9 Ritchey ML, Kelalis PP, Breslow N, et a1: Int.racavaJ and alriaJ involvement with nephroblastoma: Review of national Wilms' tumor study - 3. J ofUrol J40 (1988) 1113-/1/8 10 Rlvera LR, Martinez GG: The problem of childhood cancer in a e1eveloping country. BuH Inl Ped Ass 7 (1987) 411-419 11 Schullinger ]N, Santuli TB, Casarella W], et al: The role of right hearl angiography in the management of seJecleei cases. Ann Surg 185 (1977) 451-455 12 Shunn S, Gauderer M, Dahms B, et a1: Fatal inlraoperalive puJmonary embolization of Wilms' lumor. J Peelialr 101 (1982) 559-563 13 \'augham ED, Crosby n
