Case Report
Adrenocortical carcinoma with tumor thrombus in the right hepatic vein
MARTHA A REYES,1 GAETANO CIANCIO,2 RAKESH SINGAL3 AND MURUGESAN MANOHARAN1 Departments of Urology and 2Surgery, Division of Transplantation and 3Department of Hematology and Oncology, University of Miami Miller School of Medicine, Miami, Florida, USA
Abstract Adrenocortical carcinoma is an unusual neoplasm with very poor prognosis. Patients present with an abdominal mass often exceeding 5 cm or as a functional tumor. Computed tomography is effective to demonstrate the neoplasm as an inhomoge- neous adrenal lesion with irregular margins, and magnetic resonance imaging is helpful to visualize invasion into large vessels as well. Reported herein is a case of large adrenocortical carcinoma with tumor thrombus extending into the right hepatic vein.
Key words adrenocortical carcinoma, hepatic vein, tumor thrombus.
Introduction
Adrenocortical carcinoma (ACC) is a rare malignancy with an estimated incidence of 0.5-2 new cases per million per year. Patients may present either with a hormonally active or inactive tumor. In non-functioning tumors, which are seen in 30-60% of patients,1,2 the tumor size often exceeds 10 cm at the time of diagnosis. They may be associated with tumor thrombus in the inferior vena cava (IVC).
We report a case of adrenal cortical carcinoma with tumor thrombus in the IVC extending into the right hepatic vein. Although the involvement of the IVC by the ACC is a known entity, there are no reports of right hepatic vein involvement in the literature. Hence we present the first case of ACC with thrombus in the right hepatic vein.
Case Report
A 42-year-old Hispanic woman presented with a 3-month history of right upper quadrant pain. Computed tomogra- phy (CT) showed a 12-cm heterogeneous mass consistent with a malignant tumor arising from the right adrenal gland displacing the kidney inferiorly.
Magnetic resonance imaging (MRI) demonstrated a thrombus in the IVC extending above the hepatic vein, terminating just below the level of the diaphragm without extension to the right atrium (Fig. la). Interestingly, a tumor thrombus was also demonstrated in the right hepatic vein (Fig. 1b).
The patient was normotensive, and routine laboratory studies were normal. The serum and urinary cortisol,
Correspondence: M Manoharan, MD, Department of Urology, University of Miami Miller School of Medicine, PO Box 016960 (M814), Miami, FL 33101, USA.
Email: mmanoharan@med.miami.edu
Received 21 September 2005; accepted 22 February 2006.
metanephrine, 17-ketosteroid and 17-hydroxycorticosteroid levels were within normal limits.
The patient underwent a right radical adrenalectomy along with a right nephrectomy. The liver and IVC were mobilized.
Tumor thrombus was present in the IVC extending just above the hepatic veins without involving the right atrium. The tumor thrombus was found to involve the right hepatic vein and was adherent to the liver parenchyma. There was no tumor thrombus seen within the left hepatic vein. Once the tumor was identified to be going to the cava and into one of the hepatic veins, the dissection was carried out to isolate the cava from the liver and also from the posterior wall so that a Pringle maneuver could be performed prox- imally and distally in order to open the cava. Cavotomy was performed and the tumor thrombus was completely removed from the IVC. The tumor thrombus was adherent to the wall of the right hepatic vein and hence it was completely excised along with a rim of adjacent liver parenchyma. The cavotomy was closed and good hemosta- sis was achieved. The cava was clamped, and the patient tolerated this procedure well. The postoperative course was uneventful.
Histopathology of the adrenal gland and the caval tumor thrombus confirmed ACC. The right hepatic vein was also involved by ACC. The margins were negative. The patient was in good physical condition, but a follow-up staging CT scan at 12 months showed an abnormal lesion (1 cm) within segment VI of the liver, and CT-guided needle biopsy confirmed the presence of recurrent carcinoma. Subsequently, a resection of segment VI of the right lobe of the liver was performed. At 18-month follow up the patient is disease free.
Discussion
Effective treatment for ACC requires early diagnosis and radical excision of the adrenal tumor. However, in most
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studies, despite radical surgery, the median survival is approximately 12-22 months due to local recurrence and metastatic disease.3,4 Diagnostic work-up in patients with suspected malignant adrenal tumor involves imaging studies such as ultrasound, CT scan and MRI. Serum and
urinary cortisol, metanephrine, 17-ketosteroid and 17- hydroxycorticosteroid are investigated to assess the hor- monal function of the adrenal tumor.
Intracaval extension of tumor by ACC was first described by Castleman et al. in 1972.5 Caval involvement by ACC is rare compared to renal cell carcinoma. The intimal layer of the IVC is resistant to direct tumor invasion and hence in the majority of cases the tumor extends by an intraluminal route.6 Although most IVC thrombi can be diagnosed with CT scan, we usually perform an MRI to accurately delineate the extent of the thrombus and the involvement of the wall of the IVC by the tumor. Transe- sophageal echography is invaluable to assess the suprahe- patic and intra-atrial extension of the tumor thrombus.7
Ekici and Ciancio described a classification system for adrenal masses with thrombus extending into the IVC to facilitate the surgical approach.8 Based on this, the present tumor thrombus was classified as level IIIa (Fig. 2). In the management of level III thrombus, the liver is mobilized off the IVC completely, until it is attached to the IVC only by the major hepatic veins (the piggyback technique).9 This allows excellent proximal and distal control of the IVC, and a complete thrombectomy is performed.
In the present patient, 1 year after surgical removal of the tumor, local recurrence occurred in the liver that required a resection of the segment of the liver involved. This demonstrates the aggressive nature of this tumor. With this in mind, we believe it is best to remove the liver segment involved around the hepatic vein for better cancer control.
Acknowledgment
The authors thank Claudia Gutierrez for her illustration expertise.
References
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