Adrenal Carcinoma

Adrenocortical cancers (ACs) are uncommon malignancies that can have protean clinical manifestations. Adrenocortical masses are common; autopsy studies show that approximately 5-15% of the general adult population may have adrenal incidentalomas. Adrenal incidentalomas are biochemically and clinically asymptomatic adrenal masses found incidentally as a result of unrelated imaging investigations such as abdominal CT or MRI scans. Findings from abdominal CT scans suggest that the prevalence rate is 1-5%. Only a small number of adrenal tumors are functional and an even smaller number (approximately 1%) are malignant.
Regardless of size, approximately 1 per 1500 adrenal tumors is malignant. The evaluation of these incidentalomas, therefore, focuses on (1) identifying functional masses and treating them appropriately (including surgical removal); (2) identifying adrenal carcinomas early, with the intent of attempting complete surgical extirpation; and (3) reassuring the patients who do not fit either of these classes and arranging for their subsequent follow-up.
Although the means of identifying ACs from this subpopulation still are controversial, virtually all authorities agree about removing all nonfunctional adrenal tumors larger than or equal to 6 cm because of the significant potential cancer risk. Authorities also generally agree that nonfunctional adrenal tumors (£3 cm) have a very low probability of being adrenal cancer; therefore, they can be removed safely.
The management strategy for adrenal masses larger than 3 cm and less than 6 cm is disputed. Some authorities suggest lowering the threshold for surgical removal of nonfunctional masses from 6 cm to 4-5 cm. Others individualize the follow-up of these patients depending on their clinical status, CT scan characteristics, and age. Particularly important is the fact that these criteria do not apply to children, who generally have smaller ACs. A review of the available data suggests that the incidence rate of malignancy is small Frequency
International
AC tumors are uncommon. The incidence is approximately 0.6-1.67 cases per million persons per year. Some reports suggest an inordinately high frequency (up to 10-fold higher) of cases among children in southern Brazil, for unknown reasons. Overall, AC accounts for 0.02-0.2% of all cancer-related deaths; therefore, it is relatively rare.
Race
AC has no specific racial predilection.
Sex
The female-to-male ratio is approximately 2.5-3:1. Male patients tend to be older and have a worse overall prognosis than female patients. Female patients are more likely than male patients to have an associated endocrine syndrome. Nonfunctional ACs are distributed equally between the sexes.
Age
AC occurs in 2 major peaks: in the first decade of life and again in the fourth to fifth decades. Approximately 75% of the children with AC are younger than 5 years. Functional tumors also are more common in children, while nonfunctional tumors are more common in adults.

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