Abstract
Primary adrenal cortical carcinoma is a rare but highly malignant tumor with generally poor prognosis. Life expectancy of afflicted patients depends on early diagnosis and treatment. An early diagnosis of functioning adrenocortical carcinomas depends on the physician’s ability to recognize the clinical manifestations of excessive steroid-hormone production by the tumor. A definition of the biochemical abnormality present can be obtained with specific measurements of cortisol, aldosterone, androgen, or estrogen levels. Computerized tomography (T) or magnetic resonance imaging (MRI) helps to localize the tumor and define the presence or absence of local or distant metastases. For nonfunctioning tumors, their detection is frequently incidental to the investigation of nonspecific abdominal complaints. Attempts have been made to determine if an adrenal tumor is benign or malignant. Adrenal scintigraphy with 131I-6-β-iodomethyl-19-norcholesterol (NP-59) showing uptake in most benign tumors and lack of uptake in most malignant tumors can be helpful. However, it is important to recognize that a few cases with positive imaging may still be malignant. Surgical resection of the primary tumor and recurrences, as well as hepatic or pulmonary metastases, may help increase life expectancy. Other methods of treatment including radiation therapy and chemotherapy have been less effective. Mitotane appears effective in extending survival of patients with adrenal carcinoma, particularly when administered as early as adjuvant therapy or when combined with repeated debulking resection of recurrent tumor. The toxicity associated with mitotane administration limits the use of larger and probably more effective doses in these patients. The synthesis of analogs with more limited toxicity may ultimately provide more effective tools in the pharmacologic management of this tumor.
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Schteingart, D.E., Homan, D. (2001). Management of Adrenal Cancer. In: Margioris, A.N., Chrousos, G.P. (eds) Adrenal Disorders. Contemporary Endocrinology. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-59259-101-5_17
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DOI: https://doi.org/10.1007/978-1-59259-101-5_17
Publisher Name: Humana Press, Totowa, NJ
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