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A rare case of a Wilms tumor with intracardiac neoplastic extension is presented. The good prognosis of even extensive nephroblastoma is the reason for attempting a radical surgical approach to patients with intracardiac tumor thrombus. The exact preoperative delineation of the intracasal tumor extension is essential for a safe and successful operation. Ultrasonography in combination with echocardiography is the preferred modality for detecting intracardiac extension. Dr. med. Jürgen Pannek, Blütenweg 10, 44869 Bochum (FRG) Introduction Nephroblastoma (Wilms tumor) is one of the most common intra-abdominal tumors of childhood [1]. It grows by direct extension [2] and exhibits a marked tendency to invade vascular structures. A rare but dangerous complication is the extension of the nephroblastoma into the right atrium via the inferior vena cava (IVC). Caval tumor thrombosis occurred in 4.1% of 1,865 patients treated within the National Wilms Tumor Study 3; in 0.9% of the patients, this thrombus extended into the right atrium [3]. This extension may cause complications such as right heart inflow/outflow obstruction and pulmonary embolism. Due to modern combined therapeutic regimes, such as chemoradiotherapy and surgery, children suffering from nephroblastoma can be expected to have disease-free survival rates of between 50 and more than 90% depending on stage and histology [4]. This good prognosis of even extensive nephroblastoma is the reason for attempting a radical surgical approach in patients with intracardiac tumor thrombus. The level of vena caval involvement alone does not seem to be of prognostic significance [5]. Thus radical surgical excision of the tumor is recommended followed by chemotherapy. We present a case of a patient with nephroblastoma and intracardiac extension (ICE). Case Report In 1987, a 4.5-year-old girl was referred to our institution because of recurrent febrile episodes with abdominal pain of 4 weeks duration. Physical examination revealed a large upper left abdominal tumor. Physical ultrasound showed an inhomogenous partially cystic mass (10 × 8 × 7 D ow nl oa de d by : 54 .7 0. 40 .1 1 10 /3 1/ 20 17 9 :0 3: 19 P M cm) in the region of the left kidney, multiple masses of up to 2 cm in diameter on both sides of the abdominal aorta, and an almost complete occlusion of the IVC combined with a mass in the right atrium. The parenchyma of the liver appeared to be normal, the hepatic veins seemed to be congested. Intravenous pyelo-graphy showed no visualization of the left kidney 2 h after injection of contrast media. Distal cavography demonstrated obstruction of caval flow at the level of D 12 with collateral drainage via lumbar veins. Multiple pulmonary metastases were detected by X ray; skeletal or bone marrow metastases were ruled out by “Tc-diphosphonate scintigraphy and bone marrow cytology. Laboratory investigations were normal except for an elevated serum LDH (707 U/l). The presumptive diagnosis was Wilms tumor of the left kidney with tumor © 1994 S. KargerAG, Basel 0042-1138/94/0531-0038 $8.00/0 thrombosis in the IVC/right atrium and multiple pulmonary metastases (stage IV). First, left tumor nephrectomy and excision of all para-aortal lymph nodes was performed. Subsequently, the tumor thrombus in the IVC and right atrium was removed under the conditions of a cardiopulmonary bypass and a short period (10 min) of deep hypo-thermic circulatory arrest. The postoperative course was uneventful. Histopathological examination revealed a nephroblastoma with blastemal predominance. Five courses of a 9-week regimen including vincristine and pulses of cyclophosphamide, Adriamycin and actino-mycin D were given. The primary tumor region was irradiated with 19.2 Gy, both lungs received 12.0 Gy. The duration of postoperative treatment was 1 year. The pulmonary metastases regressed completely. At present, 4 years after the end of treatment, the girl is relapse-free and growing normally. Discussion The prerequisite for a well-planned, safe and successful operation is the exact delineation of the intravasal tumor extension [6]. This has been greatly facilitated by the newer techniques of noninvasive imaging. The preferred modality is ultrasonography [7] in combination with echocardiography [8]. Especially two-dimensional echocardi-ography with Doppler analysis and color flow mapping is presently considered as the most useful and reliable method for detecting ICE [9]. The computerized axial tomography scan seems to be of less diagnostic value [3]. The nuclear magnetic resonance scan as a new promising non-invasive imaging technique needs further experience [10]. In rare cases with inconclusive diagnostic results or specific questions, the invasive method of inferior cavogra-phy and/or brachial right heart catheterization is justified. With angiography of the IVC, as the most sensitive tool, the correct diagnosis of intracaval thrombus is achieved in nearly 100% [3]. Intracaval tumor extension below the level of the hepatic veins does not imply a particularly difficult surgical procedure. However, as the intravasal tumor extends to the diaphragm or right atrium and beyond, the hazards and difficulties of complete resection increase significantly [11]. Therefore, in the 1980s the use of the combined techniques of cardiopulmonary bypass and deep hypothermic circulatory arrest was introduced to assist in the resection of tumors that extend into the IVC above the level of the hepatic veins [6]. This approach enables the surgeon to operate in a totally bloodless field. Hypothermic arrest within given time limits is a safe and well-accepted procedure, especially in surgery for congenital heart disease [12]. D ow nl oa de d by : 54.70.40.11-10/31/20179:03:19PM Long-term survival of children with Wilms tumor and ICE is stage-dependent (2-year relapse-free survival in 78% in stage III, 72% in stage IV) [5]. These encouraging results can beachieved by surgery followed by a combined therapy with radiation and multiagentchemotherapy [4]. On the other hand, primary chemotherapy was shown to lead to a completeremission of the tumor in about 75% [3]. However, the extension of the tumor beyond thediaphragm confers particular risks, namely tricuspid valve obstruction [1], pulmonary embolism,tumor relapse or incomplete response to chemotherapy as illustrated by case reports in theliterature [3].Therefore, an aggressive surgical approach using the safe facilities of a cardiopulmonary bypassand hypothermic arrest is advocated in children with ICE reserving primary chemotherapy alonefor unresectable tumors.ReferencesSchullinger JN, Santulli TV, Casarella WJ, et al: Wilms tumor: The role of right heartangiography in the management of selected cases. Ann Surg 1977; 185:451. Slovis TL, CushingB, Reilly BJ, et al: Wilms tumor to the heart: Clinical and radiographic evaluation. Am JRoentgenol 1987;131:253. Ritchey ML, Kelalis PP, Breslow N, et al: Intracaval and atrialinvolvement with nephroblastoma: Review of National Wilms Tumor Study3.JUroll988;140:1113. Gutjahr P, Schwenger M, Spaar HJ, et al: Bedeutung der präoperativenChemotherapie und der Radiotherapie bei 373 Kindern mit Wilms-Tumor. Dtsch MedWochenschr 1990; 115:248. Nakayama DK, De Lorimier AA, O’Neill JA, et al: Intracardiac extension of Wilms tumor: Areport of the National Wilms tumor study. Ann Surgl986;204:693.Laas J, Schnid C, Allhoff E, et al: Tumor-related obstruction of the inferior vena cava extendinginto the right heart – A plea for surgery in deep hypothermic circulatory arrest. Eur J Car-diothoracSurgl991;5:653.Luck SR, De Leon S, Shkolnik A, et al: Intracardiac Wilms tumor: Diagnosis and management. JPediatr Surg 1982;17:551.Farooki ZQ, Henry JG, Green EW: Echocar-diographic diagnosis of right cardiac extension ofWilms tumor. Am J Cardiol 1975;36:363.9 Chia BL, Choo MH, Tan L, et al: Two-dimensional echocardiographic abnormalities of rightatrial metastatic tumors in hepatoma. Chest 1985;87:399.Pritchett TR, Raval JK, Benson RC, et al: Pre-operative magnetic resonance imaging of venacaval tumor thrombus. Experience with 5 cases. JUroll987;138:1220.Montie JE, Jackson CL, Cosgrove DM, et al: Resection of large inferior vena caval thrombi fromrenal cell carcinoma with the use of circulatory arrest. J Urol 1988;139:25.de Leval M: Perfusion techniques; in Stark J, de Leval M (eds): Surgery for Congenital HeartDefects. London, Grune & Stratton, 1983, pp 123-134.39 Downloadedby: 54.70.40.11-10/31/20179:03:19PM