Clinical Presentation

Women are affected twice as often as men. Three clinical patterns can be encountered.

1. A mass syndrome without any clinical evidence of hypersecretion (30% of cases). The patient complains of a large and sometimes huge flank tumor discovered by himself or herself. Alternatively, the tumor may be discovered by the patient's physician when the patient presents with a flank pain or pyrexia of unknown origin (possible tumor necrosis factor [TNF] secretion), asthenia, or weight loss. The erythrocyte sedimentation rate is often elevated. Subtle signs of hormonal secretion can be discovered, for instance, glycosuria or a shadow of a mustache above a woman's lip. In addition, there may be signs of inferior vena caval compression or

Merida Skull

FIGURE 69-1. A, Patient with a palpable left abdominal mass. B, Adrenocortical carcinoma specimen weighing 4.6 kg. C, Neoplastic thrombi extracted during operation from the inferior vena cava.

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FIGURE 69-1. A, Patient with a palpable left abdominal mass. B, Adrenocortical carcinoma specimen weighing 4.6 kg. C, Neoplastic thrombi extracted during operation from the inferior vena cava.

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obstruction (i.e., ankle edema) leading to MRI and intraoperative findings of neoplastic caval thrombus (Fig. 69-1). Tumor rupture or hemorrhage is rarely encountered.

2. An overt clinical syndrome of hypersecretion (60% of cases). Women younger than 40 years are more often affected. In patients with malignant adrenocortical tumors, the syndrome is of almost pure hypercortisolism in 30% of such cases, virilization in 22%, feminization in 10%, hyper-aldosteronism in 2.5%, and mixed secretions in 35%.1,12 Although adrenocortical carcinomas account for 5% to 10% of cases of hypercortisolism, 80% are due to corticotropin-secreting pituitary tumors. Notably, however, 40% of patients with Cushing's syndrome and adrenal neoplasms have malignant tumors. Virilizing tumors are malignant in 30% of cases, feminizing tumors in men are virtually always malignant, and pure aldosterone-secreting tumors are malignant in less than 1% of cases.14 Mixed secretion is highly suspicious of malignancy. Ectopic hyperinsulinism with hypoglycemia (Anderson's syndrome) or ectopic hyperparathyroidism with hypercalcemia has been reported, occasionally occurring synchronously in the same patient (Fig 69-2).15

3. The adrenal incidentaloma (10% of cases?). The smallest reported metastasizing adrenocortical carcinoma was 3 cm in diameter and weighed 25 g.16 Metastases occurred postoperatively. There is no evidence in the literature that solid, nonsecreting adrenal incidentalomas smaller than 3 cm in diameter are malignant (i.e., metastatic at presentation). Nevertheless, the referring physician may question whether benign adrenocortical adenomas could turn into malignant tumors. Current genetic and biochemical studies do not support this possibility. Most adrenocortical carcinomas are monoclonal, whereas the majority of adrenal adenomas are polyclonal.17 Conversely, genetic changes in locus 1 lpl5 are common in adrenocortical carcinomas and very rarely seen in adrenal adenomas.18 Point mutations of ras genes are equally encountered in 12% of adrenal carcinomas and adenomas.19 Subsequent studies may clarify whether a

FIGURE 69-2. Anderson's syndrome (ectopic hyperinsulinism) in a patient with a 2.2-kg adrenocortical carcinoma showing a confluent area of necrosis.

subset of adrenal adenomas are susceptible to malignant change or whether adrenocortical carcinomas begin de novo.

The likelihood of malignancy for tumors increases with size from 1.5 to 6 cm in diameter but remains limited because only 1 in 4000 cases (0.03%) are malignant.6 Operating on all patients with incidentalomas would probably result in more surgical deaths than patients cured by removing small adrenocortical carcinomas. However, in young patients, life-long observation may be unacceptable and may not be cost-effective, and benign adrenocortical adenomas are less common in young patients.

For patients with adrenal tumors larger than 6 cm in diameter, adrenocortical carcinomas account for up to 15% of cases.612,20'21 Surgery is therefore recommended.

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