BERKELEY, CA (UroToday.com) -
Adrenocortical carcinomas (ACCs) are rare, aggressive tumors that may be functional and cause Cushing's syndrome and/or virilization, or nonfunctional, and present as an abdominal mass or incidental finding. We recently reported a case of nonfunctional adrenocortical carcinoma with varicocele and renal vein thrombosis.(1)
Approximately 60 percent of ACCs presented with functioning tumor.(2) Patients with hormone-secreting ACCs usually present with Cushing's syndrome alone (45 percent), or a mixed Cushing's and virilization syndrome, with overproduction of both glucocorticoids and androgens (25 percent).(3) The clinical symptoms associated with glucocorticoid excess, such as weight gain, weakness, and insomnia, usually develop over three to six months.
Conversely, the patients with nonfunctioning tumors present with clinical manifestations related to tumor growth (ie, abdominal or flank pain), or with an incidentally found adrenal mass detected on radiographic imaging performed for a different reason; constitutional symptoms (weight loss, anorexia) are frequent in this clinical context. Uncommonly, a varicocele or fever and leucocytosis from tumor necrosis or production of chemokines may occur.(4)
Diagnostic evaluation of ACCs including careful history and physical examination should be performed to exclude functioning tumors such as pheochromocytoma, hyperaldosteronism, hyperandrogenism, and Cushing's syndrome. The European Network for the Study of Adrenal Tumors (ENSAT) recommends performing the hormonal tests to determine the secretory activity of the tumor even in asymptomatic patients. The hormonal tests include fasting blood glucose, serum potassium, cortisol, ACTH, 24-hour urinary free cortisol, dexamethasone suppression test, adrenal androgens (DHEAS, androstenedione, testosterone, 17-OH progesterone), and serum estradiol in men and postmenopausal women.(5) ENSAT also recommends that plasma metanephrines or urinary metanephrines and catecholamines be obtained in all patients to exclude pheochromocytoma, and that plasma aldosterone and renin be obtained in patients with hypertension and/or hypokalemia.
Radiographic studies such as CT scanning can usually distinguish adenomas from ACCs. MRI is complementary to CT, in that local invasion and involvement of the vena cava are more readily identifiable. PET scanning with fluorodeoxyglucose is of value for identifying unilateral adrenal tumors with a higher index of suspicion for malignancy.(6) Fine-needle aspiration (FNA) biopsy cannot distinguish a benign adrenal mass from adrenal carcinoma. It can, however, distinguish between an adrenal tumor and a metastatic tumor. Thus, FNA is sometimes performed when there is a suspicion of cancer outside the adrenal gland, or in the patient undergoing a staging evaluation for a known cancer.(7) Pheochromocytoma should always be excluded by biochemical testing before attempting FNA biopsy of any adrenal mass.
Even when diagnostic material is available, the distinction between benign and malignant adrenocortical tumors may be difficult, and should only be made by a pathologist experienced in using the microscopic Weiss criteria.(8) The only definitive diagnostic criteria for a malignant adrenocortical tumor are distant metastasis or the presence of local invasion. In the absence of these findings, the Weiss histopathologic system is the most commonly used method for assessing the likelihood of malignant behavior because of its simplicity and reliability.(8)
- Cheungpasitporn W, Horne JM, Howarth CB. Adrenocortical carcinoma presenting as varicocele and renal vein thrombosis: a case report. J Med Case Reports. 2011 Aug 1; 5:337.
- Maurea S, Klain M, Mainolfi C, et al. The diagnostic role of radionuclide imaging in evaluation of patients with nonhypersecreting adrenal masses. J Nucl Med 2001; 42:884.
- Mackie GC, Shulkin BL, Ribeiro RC, et al. Use of [18F]fluorodeoxyglucose positron emission tomography in evaluating locally recurrent and metastatic adrenocortical carcinoma. J Clin Endocrinol Metab 2006; 91:2665.
- Tenenbaum F, Groussin L, Foehrenbach H, et al. 18F-fluorodeoxyglucose positron emission tomography as a diagnostic tool for malignancy of adrenocortical tumours? Preliminary results in 13 consecutive patients. Eur J Endocrinol 2004; 150:789.
- Schteingart DE, Giordano TJ, Benitez RS, et al. Overexpression of CXC chemokines by an adrenocortical carcinoma: a novel clinical syndrome. J Clin Endocrinol Metab 2001; 86:3968.
- Kocijancic K, Kocijancic I, Guna F. Role of sonographically guided fine-needle aspiration biopsy of adrenal masses in patients with lung cancer. J Clin Ultrasound 2004; 32:12.
- Groussin L, Bonardel G, Silvéra S, et al. 18F-Fluorodeoxyglucose positron emission tomography for the diagnosis of adrenocortical tumors: a prospective study in 77 operated patients. J Clin Endocrinol Metab 2009; 94:1713.
- Weiss LM, Medeiros LJ, Vickery AL Jr. Pathologic features of prognostic significance in adrenocortical carcinoma. Am J Surg Pathol 1989; 13:202.
Kunatum Prasidthrathsint, MD, Supawat Ratanapo, MD, and Wisit Cheungpasitporn, MD as part of Beyond the Abstract on UroToday.com. This initiative offers a method of publishing for the professional urology community. Authors are given an opportunity to expand on the circumstances, limitations etc... of their research by referencing the published abstract.