CASE REPORT
Large Adrenocortical Carcinoma
Kristian L. Brown, MD; Daniel Bacal, MD
Adrenal cortical carcinomas (ACCs) are rare, highly malig- nant tumors that carry a poor prognosis. The large size and possibility of adherence to adjacent structures can make these tumors difficult to excise. We present a patient who underwent successful resection of a massive 19-cm, non- functional ACC, which encased the right kidney. The goal of this report is to enrich the growing body of knowledge con- cerning the presentation, evaluation, and surgical interven- tion of these rare cancers.
Keywords: cancer tumor
J Natl Med Assoc. 2009;101:1287-1290
Author Affiliations: Detroit Medical Center/Wayne State University, Detroit, Michigan (Dr Brown); and Department of Surgery Oakwood Hospital, Dear- born, Michigan (Dr Bacal).
Corresponding Author: Kristian L. Brown, MD, Detroit Medical Center/ Wayne State University School of Medicine, 4201 St. Antoine Blvd, Detroit, MI 48201 (kbrown6@dmc.org).
INTRODUCTION
A drenal cortical carcinomas (ACCs) are rare malignancies that carry a poor prognosis. They make up only 1% of adrenal tumors and account for 0.2% of all cancer deaths.1 With an average size of 12 cm at presentation, 35% to 40% of these tumors will be nonfunctional.2,3 In all adrenal masses it is important to distinguish adenomas from ACCs, and due to the high potential for malignancy, tumors greater than 4 cm should be evaluated surgically.1,3
This case report attempts to add to the growing body of knowledge by documenting the presentation, evalua- tion, and surgical intervention of a patient presenting with a rare but highly malignant adrenal cancer.
CASE REPORT
A 64-year-old male with a past medical and surgical history significant for type I diabetes, hypertension, cor- onary artery disease (status: post-triple bypass 4 years prior) and cholecystectomy was evaluated after present- ing with a 1-month history of gradually increasing abdominal distention. This distention was accompanied by dull pain mainly in the right upper quadrant of his abdomen and early satiety. However, he denied any obstructive symptoms or appreciable weight loss. There was no report of headaches, visual deficits, or prior hos-
pitalizations for uncontrolled hypertension.
On physical examination the patient was afebrile, nor- motensive (117/67 mm Hg), with a resting heart rate of 76 beats per minute and no tachypnea. Abdominal exam was remarkable for significant distention with moderate ten- derness to palpation mainly over the right aspect of the abdomen. Basic laboratory values were significant for a slight hyponatremia (133 mMol/L; normal, 135-147). An adrenal panel revealed normal values for aldosterone (7.5 ng/dl; normal, 4-31), norepinephrine (343 pg/ml; normal, <420), epinephrine (51; normal, <84), dopamine (30 pg/ ml; normal, <60), and total cathecholamines (394 pg/ml; normal, <504). Urine total metanephrines were 642 µg/24 hours (normal, 140-820). The remainder of the complete blood count, electrolyte, coagulation, and liver function tests were within normal limits.
Computer tomography (CT) scan of the abdomen completed at an outside hospital revealed a large right- sided mass. Subsequent CT-guided biopsy demonstrated histopathology consistent with ACC (Figure 1A). Mag- netic resonance imaging (MRI) of the abdomen showed a 19×12×10 cm right-sided ACC with necrotic center (Figure 1B). The tumor was noted to be exerting a mass effect on but not invading the inferior vena cava (IVC) (Figure 1C) with displacement of the right kidney infe- riorly. There was no invasion into the right renal vein, but the tumor was affixed to the right kidney (Figure 1D). There was no lymphadenopathy. Positron emission tomography (PET) scan demonstrated a standard uptake value (SUV) of 6.4 (SUV levels above 2.5 are highly suggestive of malignancy in the proper clinical setting). There was no pathological uptake in paracervical or intrathoracic lymph nodes. Head, neck, and lungs were also negative.
The abdomen was approached through a right sub- costal incision (extending his prior cholecystectomy incision), providing good access to the right kidney and adrenal mass. After completing a Kocher maneuver the IVC was inspected and, although it was noted to be tented anteriorly by the large tumor, the IVC was not cir- cumferentially encased and the mass was easily dis- sected free from the adventitia of the cava. The tumor and the right kidney were mobilized laterally to medi- ally. (Figure 2). Upon manipulation of the mass, care was taken to define the right renal artery, renal vein, and adrenal vein, the latter of which was identified as com-
LARGE ADRENOCORTICAL CARCINOMA
ing directly off of the IVC. The renal artery and vein were controlled proximally and distally with suture ties and clips and then divided. In like fashion, the adrenal
vein was also controlled using ties and clips and divided, with no signs of accessory branches or continued bleed- ing. Pathologic examination revealed an 805-g partially
ACC
ACC
A
B
ACC
ACC
RK
C
D
A, Large abdominal right-sided mass via computed tomography (patient in prone position for adrenal biopsy). B, T2-weighted magnetic resonance imaging (MRI) of the abdomen (coronal view) showing the adrenal cortical carcinoma with necrotic center. C, T2-weighted MRI of the abdomen showing adrenal cortical carcinoma compressing inferior vena cava indicated by adrenal cortical carcinoma. D, T2-weighted MRI image of the abdomen showing adrenal cortical carcinoma affixed to adjacent right kidney annotated R.K.
Cardinal Health
KIDNEY
ACC
A
B
A, Excised adrenal cortical carcinoma encasing right kidney. B, Vascular pedicle of right kidney and adjacent adrenal cortical carcinoma (arrow)
encapsulated ACC, which approximated the renal cap- sule without parenchymal invasion. The postoperative recovery was uneventful, and the patient was discharged home after 8 days with plans to begin mitotane adjuvant chemotherapy. The follow-up surveillance strategy will include a PET scan at 1 month and a CT scan at 8 months to assess continued disease-free status.
DISCUSSION
Nonfunctional ACCs are more common in older adults, in which they are overall more aggressive.4 It is crucial to determine as early as possible whether these lesions are malignant or benign. Many centers routinely use core needle biopsies to help make this distinction. Lau and colleagues examined the histopathologic sensitivity and specificity of these core needle biopsies and found that they were correctly diagnosed as ACC or adrenocor- tical adenoma 76.2% and 75.6%, respectively.5 Indeed, histopathologic analysis of core needle biopsies has some role in the ACC diagnostic algorithm, but the weighted significance of this role must be further explored.
For malignant lesions, certain factors have been asso- ciated with a greater survival. Bilimoria and colleagues reported on a series involving 3982 ACC patients and noted that the most important predictors of survival for ACC are age, tumor grade, stage, and success of surgical resection.3,4,6 In addition to these 4 predictors, the litera- ture has also dealt with size of tumor at presentation and its prognostic potential. In a study involving 215 patients, size of tumor at presentation showed no correlation with survival.7 Moreover, a retrospective study of 457 patients with ACC suggest that the size of the tumor at presenta- tion was a good indicator of malignant potential, with tumors greater than 8 cm showing a 47% likelihood of being malignant.8 The marriage of these 2 studies sug- gests that although tumor size at presentation was not a good indicator of survival, it does offer valuable infor- mation concerning malignant potential and impacts the success of resection. Additionally, laparoscopic resec- tion was contraindicated due to the influence of tumor size on malignancy potential (possible metastatic seed- ing of the abdomen during resection) and respectability (given the tumor’s attachment to the right kidney).
The evolution of imaging modalities has had a direct effect on the diagnosis of adrenal tumors, as seen by the increase in incidentally found adrenal masses. The deci- sion to surgically remove these masses has pivoted on whether they were 4 cm or larger at the time of initial visualization. Ballian and colleagues reported on a series of 106 adrenalectomies performed between 1989 and 2007. They retrospectively found that adherence to a 4-cm size criteria as a sole indictor of adrenalectomy would have resulted in the resection of all primary malignant adrenal tumors in that series.9 When looking specifically at the success of resection as a prognostic indicator, one might postulate that advances in CT and
MRI technology and the lower threshold to utilize this technology in the workup of other nonrelated disease conditions would translate into earlier detection of smaller more contained tumors yielding improved resectability. However, a 15-year study by Paton et al suggests that despite the increasing sensitivity of imag- ing modalities there has not been a concomitant improve- ment in early-stage diagnosis of these tumors.3,10 Instead, the real benefit may lie in the improved preoperative planning and the assessment of IVC and renal vascula- ture involvement, yielding improvements in survival by increasing the likelihood of successful resection. The next generation of imaging modalities may come in the form of “fusion images” or radiologic studies that are a combination of PET-MRI or PET-CT. In a study of 175 adrenal masses that were evaluated using PET alone vs PET-CT, the sensitivity, specificity, negative predictive value, and positive predictive value for detecting malig- nant vs adenomatous lesions were found to be superior when using the fusion imaging methodology.11 This enhancement in imaging technology may eventually replace all other forms of radiologic staging and could provide a valuable tool in the surveillance of patients after surgical resection.12
According to the modified classification provided by Sullivan, this patient was determined to have a stage II (T2NOM0) cancer with a literature-supported 5-year survival of 60% with a median survival time of 101 months.13 The role of adjuvant therapy for completely resected nonmetastatic disease is controversial. Cur- rently, mitotane remains the acceptable choice of che- motherapeutic agents for recurrent and metastatic dis- ease. Several studies suggest that mitotane may delay or even prevent local recurrent disease but only shows a 20% to 35% patient response despite tropism for adre- nocortical tissue.1,14-16 Indeed the value of this adjuvant therapy must be further studied. One stumbling block has been the rarity of the disease and the lack of signifi- cant patient volume needed to conduct prospective tri- als. This has been partially addressed by pooling patient databases from national and international centers. These collaborations show much promise in bringing an effica- cious treatment protocol to ACCs.
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LARGE ADRENOCORTICAL CARCINOMA
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