ADRENAL CORTICAL CARCINOMA WITH TUMOR THROMBUS INVASION OF INFERIOR VENA CAVA
CHIN-YUEH WEI, M.D. KUANG-KUO CHEN, M.D., PH.D. MING-TSUN CHEN, M.D. HSIAO-TING LAI, M.D. LUKE S. CHANG, M.D.
From the Divisions of Urology and Cardiovascular Surgery, Department of Surgery, Veterans General Hospital-Taipei and National Yang-Ming University, School of Medicine, Taipei, Taiwan, Republic of China
ABSTRACT-A case of adrenal cortical carcinoma with inferior vena cava (IVC) involve- ment is presented. Ultrasonography, computed tomography, and venacavography all presumptively showed a large mass over the upper pole of the left kidney with tumor thrombus in the IVC. However, aortography demonstrated that this mass was receiving its blood supply from the left inferior phrenic artery, aorta, and left renal artery. Radi- cal surgery, including resection of the tumor and its adjacent organs (kidney, distal pan- creas, spleen) and the tumor thrombus in the IVC, with the aid of cardiopulmonary by- pass, was performed. We emphasize that adrenal cortical carcinoma can have tumor thrombi invading the IVC, and in such cases we suggest radical surgical removal of the tumor and the thrombus.
Carcinoma of the adrenal cortex is a rare malig- nancy with an estimated incidence of about 2 cases per million population.1 Further development of the tumor thrombi invasion of the inferior vena cava (IVC) is very rare. There were only 29 cases reported in English literature up to December 1990. Herein we report another case of adrenal cortical carcinoma with tumor thrombus in the IVC.
CASE REPORT
A 53-year-old woman was referred for evalua- tion of a palpable intra-abdominal mass. She had a 6-week history of pain in the left flank area. The patient was normotensive, and, except for the elevation of alkaline phosphatase and lactate dehydrogenase values, the general blood studies, including urea nitrogen and creatinine, were normal. The serum cortisol level and urinary 17- ketosteroid and 17-hydroxycorticosteroid were within normal limits. However, elevated serum testosterone (0.95 ng/ml) and progesterone (3.01 ng/ml) were detected.
An excretory urogram demonstrated a large mass in the left upper quadrant of the abdomen, causing downward displacement of the left kid- ney. Abdominal ultrasonography showed a large left retroperitoneal mass with a thrombus in the IVC. Venacavography revealed a tumor thrombus in the IVC at the level of the first lumbar vertebra (Fig. 1). Computed tomographic scan demon- strated a heterogeneous soft tissue mass about 12 × 12 × 10 cm in the upper pole of the left kidney, with central necrosis. This mass displaced the pancreas and spleen anteriorly. Also, a throm- bus was seen in the IVC and left renal vein (Fig. 2). However, aortography showed a hypervascu- lar suprarenal mass, about 14 × 11 cm, receiving blood supply from the left inferior phrenic artery, aorta, and left renal artery (Fig. 3).
Through a midline incision from the sternal notch to the lower abdomen and using extracor- poreal cardiac bypass, we found a large tumor mea- suring about 12 × 12 × 12 cm, fragile with cen- tral necrosis as well as hemorrhage, located over the left retroperitoneal space with adhesion to the upper pole of left kidney and distal portion of the pancreas. Also, a tumor thrombus, measuring
about 5 cm in length, was easily freed from the wall of the IVC. So, we performed the inferior venacavotomy with tumor thrombectomy, wide ex- cision of retroperitoneal tumor, left nephrectomy, hemipancreatectomy, and splenectomy. Histopatho- logic examination revealed the presence of adreno- cortical carcinoma with tumor thrombi in the left adrenal vein, renal vein, and IVC. The postopera- tive course was uneventful. She was in good physi- cal condition 2 months after the operation.
COMMENT
Adrenal cortical carcinoma with IVC invasion was first found at autopsy in 1963.2 Castleman et al.3 reported the first case found during surgery. Cahill and Sukov4 described the first case diag- nosed preoperatively and managed surgically. Since 1980, ultrasonography and computed tomography have been the first-line diagnostic methods for evaluating suprarenal masses.5 Schramek et al.6 re- ported on 1 case of adrenal cortical carcinoma with tumor thrombus extending to the right atrium of the heart, which was detected preoperatively. Pritchett et al.7 first used magnetic resonance imag- ing to access the presence and extent of vena caval tumor thrombus. The exact incidence of caval in-
vasion in adrenal cortical carcinoma at the time of diagnosis is uncertain. Nader et al.8 found that, of 60 patients with distant metastases from adrenal cortical carcinoma, 6 had caval invasion. In renal cell carcinoma, the incidence of caval invasion is approximately 9%.9
A review of the literature shows that 23 of 30 cases (including our case) of adrenal cortical car- cinoma with tumor thrombi in the IVC involve women.2-7,10-18 In these cases, the right side is in- volved more often than the left side. This fact is well explained because of the direct course of the short right adrenal vein into the IVC compared with the longer left adrenal vein that drains into the renal vein. In half of the reported cases with tumor thrombus in the IVC, the thrombus will extend further into right atrium. Bilateral tumors have not been reported to date.
Although there is general agreement that caval invasion is an ominous expression of the malig- nancy of a tumor, the results of surgical manage- ment suggest that this invasive feature should not be a grave prognostic indicator that prohibits at- tempt at operative eradication of the primary tu- mor and its caval extension.5 Subhepatic caval clamping provides a good and simple vascular con- trol method to remove the subhepatic intracaval tumor thrombus extending from adrenal cortical carcinoma. Extracorporeal cardiopulmonary by- pass may be used to give superior visualization and complete removal of intracaval thrombi.
In conclusion, we emphasize that adrenal corti- cal carcinoma should be considered as the pri- mary tumor in the differential diagnosis of the tu- mor thrombi invasion to the IVC. We advocate radical one-stage resection of adrenal tumors as well as tumor thrombectomy in IVC, provided no other evidence of disseminated or systemic dis- ease is present.
Kuang-Kuo Chen, M.D. Division of Urology Department of Surgery Veterans General Hospital-Taipei Taipei, Taiwan, 11217, Republic of China
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