Ann Thorac Surg 1995;60:1818-1820
© 1995 The Society of Thoracic Surgeons
Case Report
Resection of an Adult Intracardiac Wilms' Tumor Using Hypothermic Circulatory Arrest
Peter Fonseca, MD,
G. Hossein Almassi, MD,
Frank Begun, MD
Departments of Cardiothoracic Surgery and Urology, Medical College of Wisconsin, Milwaukee, Wisconsin
Accepted for publication July 3, 1995.
 |
Abstract
|
|---|
A 51-year-old woman underwent resection of a renal tumor with intracaval and intracardiac extension. Histologic examination demonstrated an adult Wilms' tumor. Cardiopulmonary bypass and deep hypothermic circulatory arrest were used in a combined abdominal and thoracic procedure to remove the tumor and extension in its entirety without complication.
|
Introduction
|
|---|
Adult Wilms' tumor is a rare entity among renal tumors, particularly with intracaval and intracardiac involvement. A case of a Wilms' tumor with extension into the inferior vena cava and right atrium is presented. Under deep hypothermic circulatory arrest, complete resection was performed. The advantage of this technique is discussed.
A 51-year-old woman was admitted to the hospital for evaluation of a right renal mass. She had initially noted a hardness in her lower abdomen several months earlier, with subsequent increased abdominal girth. On the day of admission she experienced the onset of intense back pain with associated nausea and vomiting.
Past medical history was significant for chronic hypertension controlled with medication. A hospital admission elsewhere 4 months earlier for pulmonary embolism was treated with anticoagulation. The physical examination reportedly was normal, with no lymphadenopathy and no definite abdominal masses palpable. All blood chemistries were within normal limits, as was the chest roentgenogram. Urinalysis showed evidence of microscopic hematuria.
A computed tomographic scan of the chest and abdomen showed a right renal mass displacing the aorta and mesenteric vessels to the left of the midline (Fig 1
). Intraluminal caval thrombus extended to the right atrium (Fig 2) and was confirmed by transesophageal echocardiography to be a right atrial mass 5.4 cm in diameter. The remainder of the chest computed tomographic scan was negative, as was the computed tomographic scan of the head and a bone marrow biopsy.
View larger version (145K):
[in this window]
[in a new window]
|
Fig 1.. Computed tomographic scan of the abdomen demonstrating large mass originating from the right kidney.
|
|
The tumor was removed in its entirety along with the right kidney and the right adrenal gland through a combined median sternotomy and midline abdominal incision. Intraabdominal dissection revealed the tumor to be adjacent to the colon and duodenum, but not invading these structures. The patient was placed on cardiopulmonary bypass with an arterial cannula in the ascending aorta and a single 26F venous cannula in the high right atrium; the usual venous cannulation was not possible because of the right atrial and inferior vena caval tumor. The patient was then actively cooled, with the heart vented via the right superior pulmonary vein. During the circulatory arrest time and aortic cross-clamping, the myocardial temperature was maintained at 10°C with additional blood cardioplegia.
Circulatory arrest was initiated at a systemic body temperature of 20°C (bladder temperature), and with the head packed in ice and additional barbiturates, an isoelectric electroencephalogram was obtained. A right atriotomy was performed and via bimanual palpation with the inferior vena cava open, the tumor was removed from the right atrium and inferior vena cava. The inferior vena cava was totally occluded by the tumor. The tricuspid valve was not involved and appeared normal. The freer elevator was required at times to remove tumor thrombus from the endothelial wall of the inferior vena cava including the hepatic branches. The stump of the proximal inferior vena cava was oversewn. The patient weaned from bypass after rewarming without difficulty. The total circulatory arrest time was 25 minutes.
Pathology revealed an adult Wilms' tumor of 3 kg (15.3 x 14.5 x 8.0 cm) with extension through the capsule and tumor thrombus throughout the inferior vena cava, but no involvement of surrounding structures or the right atrial myocardium. Histology was compatible with a favorable histology of epithelial-predominant-type Wilms' tumor. All resected lymph nodes and surrounding structures were negative for metastases, making this a stage II tumor [6]:
- Stage I: Tumor confined to the kidney and completely excised
- Stage II: Tumor penetrates capsule, but is completely excised
- Stage III: Residual tumor or positive lymph nodes
- Stage IV: Distant metastasis
The postoperative course was uneventful with the exception of lower extremity edema as the only morbidity, most likely secondary to inferior vena cava ligation. The patient received postoperative radiation to the tumor bed. She, however, did not tolerate the chemotherapy. She died a year later with widespread metastasis.
|
Comment
|
|---|
Wilms' tumor is rarely found in adults, although it is the most common renal neoplasm in children, accounting for approximately 20% of all malignant tumors in this age range [1]. Only 200 cases of adult Wilms' tumor have been reported in the world literature [1]. Due to the limited patient base, the incidence is difficult to ascertain and the most effective treatment is uncertain. Within the last 10 years some treatment regimens have been proposed based on the experience of the National Wilms' Tumor Study Project.
Hypothermic circulatory arrest is commonly used for repair of complex congenital heart malformations, adult aortic reconstruction, and neurosurgical procedures for tumors and aneurysms involving the central nervous system. Its use has also been reported in removing renal cell carcinoma with extension in the inferior vena cava [2–5]. We believe the advantage of a bloodless surgical field provides maximum exposure for tumor removal and is well suited to cases such as the one described. Removal of such tumors without the use of cardiopulmonary bypass is not safe and can result in tumor embolism to the lungs [6].
It is clear that surgical excision is the primary mode of treatment of adult Wilms' tumor, as it is in children. This acts to establish the diagnosis, debulk the tumor, and define the tumor bed for future irradiation and guidance in chemotherapy regimens [1].
Interestingly and importantly, the prognosis for adults with this neoplasm is significantly worse than for children. Several studies have shown a decrease in survival for adults compared with children (3-year survival of 24% for adults compared with 74% for children) [7]; perhaps because they present at a later stage than is typical of children [8].
Those adults who were treated aggressively with operation, chemotherapy, and radiation appeared to have fared better than adults treated in the prechemotherapy era. It has been concluded that aggressive therapy should be given to all adults with this tumor irrespective of size and involvement, despite their relatively poorer prognosis [7].
The incidence of caval and cardiac involvement has also been studied. Although the incidence of caval involvement in adults is unknown, the incidence in children was determined to be 0.7% to 10% [6, 9]. In one study [9] the level of caval involvement had no effect on survival, and 16 patients with atrial involvement survived 3 or more years with concomitant multimodal treatment of operation, radiation, and chemotherapy.
Histology has been determined to have the most prognostic value [6]. Patients with a favorable histology of predominantly epithelial cells have a better chance of longer survival. It should be noted that the risk of relapse was increased in the National Wilms' Tumor Study-2 in patients with intravascular tumor thrombus, however [10].
The value of a planned operative approach has also been stressed. Fifteen patients enrolled in the three National Wilms' Tumor Studies who had intracardiac tumor extension revealed no operative deaths, with fewer complications (3/6) when intracaval extension was recognized before operation than when it was not (8/9) [6]. This may represent the presence of a cardiac surgeon and availability of cardiopulmonary bypass and circulatory arrest during surgical resection. The use of cardiopulmonary bypass was necessitated for intracardiac involvement in a combined thoracoabdominal approach in these patients. In our patient a median sternotomy and a midline abdominal incision provided wide exposure for removal of the tumor.
We believe that the use of hypothermic circulatory arrest provides the additional advantage of a bloodless surgical field in which to allow thorough and definitive investigation and removal of the entire tumor thrombus. This technique, with its proven safety and within the limited time interval required for removal of intracardiac and extracardiac pathologies [11], can be used in a variety of conditions other than the repair of congenital heart diseases and aortic arch operations.
|
Footnotes
|
|---|
Address reprint requests to Dr Almassi, Department of Cardiothoracic Surgery, Medical College of Wisconsin, 8700 W Wisconsin Ave, Milwaukee, WI 53226.
|
References
|
|---|
- William G, Colbeck RA, Gowing NFC. Adult Wilms' tumor: review of 14 patients. Br J Urol 1992;70:230–5.[Medline]
- Klein EA, Kaye MC, Novick AC. Management of renal cell carcinoma with vena caval thrombi via cardiopulmonary bypass and deep hypothermic circulatory arrest. Urol Oncol 1991;18:445–7.
- Montie JE, Ammar RE, Pontes JE, et al. Renal cell carcinoma with inferior vena cava tumor thrombi. Surgery 1991;173:107–15.
- Montie JE, Jackson CL, Cosgrove DM, Streem SB, Novick AC, Pontes JE. Resection of large inferior vena caval thrombi from renal cell carcinoma with the use of circulatory arrest. J Urol 1988;139:25–8.[Medline]
- Shahian DM, Libertino JA, Zinman LN, Leonardi HK, Eyre RC. Resection of cavoatrial renal cell carcinoma employing total circulatory arrest. Arch Surg 1990;125:727–32.[Abstract/Free Full Text]
- Nakayama DK, Delorimier AA, O'Neil JA, Norkool P, D'Angio GJ. Intracardiac extension of Wilms' tumor. A report of the National Wilms' Tumor Study. Ann Surg 1986;204:693–7.[Medline]
- Grosfeld JL, Weber TR. Surgical considerations in the treatment of Wilms' tumor. In: Gonzalez-Crussi F, ed. Wilms' tumor and related renal neoplasms of childhood. Boca Raton, FL: CRC Press, 1984;263–83.
- Husea J, Grignon DJ, Ro JY, Ayala AG, Shannon RL, Papadopoulos NJ. Adult Wilms' tumor: a clinicopathologic study of 11 cases. Mod Pathol 1990;3:321–6.[Medline]
- Ritchey ML, Kelalis PP, Breslow N, Offord KP, Shochat SJ, D'Angio GJ. Intracaval and atrial involvement with nephroblastoma: review of National Wilms' Tumor Study-3. J Urol 1988;140:1113–8.[Medline]
- Breslow N, Churchill G, Beckwith JB, et al. Prognosis for Wilms' tumor patients with nonmetastatic disease at diagnosis. J Clin Oncol 1985;3:521–6.[Abstract]
- Kirklin JW, Barratt-Boyes BG. Cardiac surgery. 2nd ed. New York: Churchill-Livingstone, 1993;62–73.
Top
Abstract
Introduction
