Adrenocortical carcinoma is a rare tumor that affects only 1 to 2 persons per one million population. It usually occurs in adults, and the median age at diagnosis is 44 years. Although adrenal carcinoma is potentially curable at early stages, only 30% of these malignancies are confined to the adrenal gland at the time of diagnosis. Radical surgical excision is the treatment of choice for patients with localized malignancies and remains the only method by which long-term disease-free survival may be achieved. Overall 5-year survival for tumors resected for cure is approximately 40%.
Retrospective studies have identified two important prognostic factors: completeness of resection and stage of disease. Patients without evidence of invasion into local tissues or spread to lymph nodes have an improved prognosis. The role of DNA ploidy as a prognostic indicator is controversial, with some  studies, such as ECOG-1879 for example, showing correlation between aneuploidy and prognosis, and other studies [2,4] showing no correlation.
Note: Some citations in the text of this section are followed by a level of evidence. The PDQ editorial boards use a formal ranking system to help the reader judge the strength of evidence linked to the reported results of a therapeutic strategy. (Refer to the PDQ summary on Levels of Evidence for more information.)
Surgical resection, i.e., adrenalectomy, is the definitive treatment for patients with localized pheochromocytoma. If preoperative imaging reveals an adrenal...
Approximately 60% of patients present with symptoms related to excessive hormone secretion, but hormone testing reveals that 60% to 80% of tumors are functioning.[5,6] Nonfunctioning carcinomas may be heralded by symptoms of local invasion by tumor or by metastases. Initial evaluation should include, in addition to appropriate endocrine studies, computed tomography and/or magnetic resonance imaging of the abdomen. Selective angiography and adrenal venography may be helpful in identifying smaller lesions and for distinguishing tumors of the adrenal gland from tumors of the upper pole of the kidney. Although the use of positron emission tomography may be effective in identifying unsuspected sites of metastases, its role as a staging tool is unclear. The detection of metastatic lesions may allow effective palliation of both functioning and nonfunctioning lesions.
The most common sites of metastases are the peritoneum, lung, liver, and bone. Palliation of metastatic functioning tumors may be achieved by resection of both the primary tumor and metastatic lesions. Unresectable or widely disseminated tumors may be palliated by antihormonal therapy with mitotane, systemic chemotherapy, or (for localized lesions) radiation therapy. However, survival for patients with stage IV tumors is usually less than 9 months unless a complete remission is achieved.[6,8,9,10] There is no convincing evidence to date that systemic therapy will improve the survival duration of patients with adrenal cancer.
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Lee JE, Berger DH, el-Naggar AK, et al.: Surgical management, DNA content, and patient survival in adrenal cortical carcinoma. Surgery 118 (6): 1090-8, 1995.
Camuto P, Schinella R, Gilchrist K, et al.: Adrenal cortical carcinoma: flow cytometric study of 22 cases, an ECOG study. Urology 37 (4): 380-4, 1991.
Haak HR, Cornelisse CJ, Hermans J, et al.: Nuclear DNA content and morphological characteristics in the prognosis of adrenocortical carcinoma. Br J Cancer 68 (1): 151-5, 1993.
Icard P, Chapuis Y, Andreassian B, et al.: Adrenocortical carcinoma in surgically treated patients: a retrospective study on 156 cases by the French Association of Endocrine Surgery. Surgery 112 (6): 972-9; discussion 979-80, 1992.
Luton JP, Cerdas S, Billaud L, et al.: Clinical features of adrenocortical carcinoma, prognostic factors, and the effect of mitotane therapy. N Engl J Med 322 (17): 1195-201, 1990.
Becherer A, Vierhapper H, Pötzi C, et al.: FDG-PET in adrenocortical carcinoma. Cancer Biother Radiopharm 16 (4): 289-95, 2001.
Brennan MF: Adrenocortical carcinoma. CA Cancer J Clin 37 (6): 348-65, 1987 Nov-Dec.