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Adrenal Tumor with Caval Extension
Case Report and Review of the Literature
John P. Chesson1 and Dan Theodorescu1,2
From the 1Departments of Urology and 2Molecular Physiology and Biological Physics, University of Virginia Health Sciences Center, Charlottesville, Virginia, USA
(Submitted April 24, 2001. Accepted for publication May 29, 2001)
Scand J Urol Nephrol 36: 71-73, 2002
Extension of adrenal cortical carcinomas into the IVC is rare. We describe one such tumor that extended to the level of the right atrium. In an effort to aid recognition and guide work-up of an upper pole lesion, we review the literature comprised of 77 similar cases and analyze the data in terms of patient demographics, anatomic distribution, clinical and laboratory evidence of endocrine function. Our review suggests that over half of all adrenal lesions with IVC extension will be clinically nonfunctional, including up to 17% of pheochromocytomas.
Key words: adrenal neoplasms, outcomes.
Dan Theodorescu MD PhD, Department of Urology, Box 422, University of Virginia Health Sciences Center, Charlottesville, Virginia, 22908, USA. Tel: (804) 9240042. Fax: (804) 982 3652. E-mail: dt9d@virginia.edu.
Though widely recognized in the case of renal cell carcinoma (RCC), extension into the inferior vena cava (IVC) can be associated with a number of lesions in the upper pole area of the kidney (1-5). We present a case of a clinically silent adrenal cortical carcinoma (ACC) that extended via the renal vein and IVC to the level of the right atrium. In addition, we review the literature and compare patient demographics and clinical and biochemical tumor functionality among patients with adrenal lesions involving the IVC.
CASE REPORT
The patient is a 59-year-old white female who presented with a 2-week history of bilateral lower extremity edema. She denied flank pain and hematuria. Review of systems was positive for dyspnea. Blood pressure was normal. Physical examination was un- remarkable aside from marked pre-tibial edema. Initial laboratory examinations were unremarkable. CT re- vealed a 12 cm mass in the area of the upper pole of the left kidney (Fig. 1A) with MRI of the abdomen and pelvis revealing IVC extension via the renal vein to the level of the right atrium (Fig. 1B). Angiography demonstrated a well-vascularized lesion displacing the left kidney inferiorly (Fig. 1C). Metastatic work- up including a bone scan and CT of the head and chest was negative while a 24-h urine sample analysis for protein, catecholamines, VMA and metanephrine,
revealed only modest proteinuria, consistent with IVC syndrome. Heparin was infused at a therapeutic rate until 6 h before surgery.
Owing to right atrial involvement, we opted to perform surgery using cardiopulmonary bypass and complete circulatory arrest (6). Circulatory arrest lasted 29 min, during which a 15 cm cavotomy was made to reveal a freely mobile tumor thrombus (Fig. 1D). Bland thrombus was apparent entering the hepatic vein and proximal IVC. The caval tumor was fractured at the level of the renal vein, which was oversewn, followed by closure of the cavotomy. Circulatory arrest was then reversed and adrenalectomy and radical nephrectomy carried out. A 15 x 13 x 3 cm mass was removed and appeared grossly to represent an adrenal process (Figs 1E and 1F). Post-operatively, the patient did well and was discharged on the 11th post-operative day. Pathologically, the 1.28 kg mass was a Stage III adrenal cortical carcinoma with associated tumor thrombus. Surgical margins were negative.
REVIEW OF THE LITERATURE AND CONCLUSION
With an annual incidence of <2 per million, adrenal cortical carcinomas are rare tumors. Though the exact incidence of IVC extension is not known, the combined incidence in two larger series (11/142) (7,8) ap- proaches the rate reported for renal cell carcinoma of
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9% (9). Previously, Hedican & Marshall reviewed 29 cases of ACC with IVC extension (10). By emphasiz- ing IVC obstructive symptomatology as well as the crucial distinction between laboratory and clinical evidence of endocrine tumor function, we not only expand on but give a clinical focus to this review.
Using MEDLINE, we searched the English literature between years 1952 and 2000 with the headings “adrenal” and “IVC extension”. Additional cases were found using the references contained in the reports identified by MEDLINE. In all, we found 105 histologically confirmed adrenal tumors with IVC involvement: 78 ACC, 16 pheochromocytomas, 3 neuroblastomas, 3 leiomyosarcomas, 2 transitional cell carcinomas of the renal pelvis, 2 metastatic small cell carcinomas, and 1 Wilms’ tumor. In the 85 cases where anatomic detail was given, 74% of tumors were right-sided. All 16 of the IVC-extensive pheochromo- cytomas described occurred on the right, compared with 68% of ACC and 70% of other lesions. The average age of the patient with pheochromocytoma was 47 years for females and 49 years for males. For ACC, the average age for males and females was 34
and 39 years, respectively. The female:male prevalence ratio was different for pheochromocytoma (0.67:1), compared to ACC (4:1). For ACC as a whole, the ratio is 4:3 (11).
In order to characterize the way in which IVC extension affects presentation in these lesions, we took the following to indicate clinically evident IVC obstruction: lower extremity edema and/or shortness of breath; ascites; and increased abdominal girth in an infant. Overall, 45% of all patients had evidence of IVC obstruction (n=71). Among those with ACC, 48% presented with obstructive symptoms (n = 50). For tumor size, we used the greatest diameter on preoperative imaging. Where modalities differed as to preoperative size, we used the largest diameter attributed to the tumor. Pre-operative imaging showed the average ACC tumor to be 12 cm in its greatest diameter. Another 22 ACC lesions were described simply as large on preoperative work-up. At surgery, these “large” tumors averaged 15 cm. The miscella- neous (non-ACC, non-pheochromocytoma) lesion averaged 12 cm (n = 12), and half of these patients presented with evidence of obstruction. The average
pheochromocytoma was markedly smaller at 5 cm (n=7), and presented with obstructive symptoms in only 27% of cases (n= 11). Overall, among patients presenting with obstructive symptoms, the average tumor measured 11 cm pre-operatively (n = 16).
To clarify the way in which tumor function affects presentation, we distinguished not only between biochemically functional and non-functional tumors, but also between tumors in which function was readily apparent clinically and tumors in which function was clinically silent. We took the following to indicate clinical functionality: for pheochromocytoma, a SBP greater than 140 mmHg or a description of the patient as “hypertensive” on presentation; and for ACC, facial rounding associated with increased abdominal girth, hirsutism, sexual precocity or otherwise cushingoid preoperative picture.
Based on these criteria, 50% of ACC tumors were clinically functional (n =52). Females were more likely than males to have clinically functional tumors (56% vs 27%). Half of the clinically functional females were less than 31 years of age, compared to 2 of the 3 males, and notably, 57% suffered from hirsutism. Tumors were more likely to be clinically functional in young patients (71% of patients ≤30 years compared to 36% of patients >30 years). Interestingly, only half of clinically non-functional ACC tumors showed evidence of function when laboratory analysis was performed (n = 34). Preoperative tumor size did not seem to be related to clinical endocrine function. Among cases of IVC-extensive pheochromocytoma, 17% were clinically inapparent (n = 12). However, all of these tumors were functional on laboratory testing.
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