Evaluation

The adrenal gland is one of the frequent sites of metastases, and virtually any malignancy may spread to it. A metastatic tumor should be highly suspected in the setting where an imaging study detects an adrenal mass in a patient with a known history of malignant disease. Common cancers that metastasize to the adrenal gland are from the lung, kidney, breast, gastrointestinal tract, and melanoma. Occasionally, an adrenal metastasis is discovered with an undetermined primary origin; thus, a thorough history and physical examination are essential for all patients (Figures 12-3 and 12-4). For example, melanomas can recur many years after previous surgical excision. The clinical assessment suggests what investigations are required to confirm the diagnosis, such as a chest radiograph and bronchoscopy for lung cancer or a mammogram and biopsy for breast cancer.

The next priority is to assess the functional status of the incidentaloma. Because these lesions are dis-

Figure 12-1. A 46-year-old woman had nonspecific right-sided abdominal discomfort and palpitation. An abdominal computed tomographic scan showed a low attenuation 3 cm x 1.8 cm x 1 cm right adrenal tumor (large white arrow) and a normal left adrenal gland (small white arrow). Biochemical workup showed no hyper-function. She underwent a laparoscopic right adrenalectomy. Pathology showed a 4 cm adrenocortical adenoma.

Figure 12-1. A 46-year-old woman had nonspecific right-sided abdominal discomfort and palpitation. An abdominal computed tomographic scan showed a low attenuation 3 cm x 1.8 cm x 1 cm right adrenal tumor (large white arrow) and a normal left adrenal gland (small white arrow). Biochemical workup showed no hyper-function. She underwent a laparoscopic right adrenalectomy. Pathology showed a 4 cm adrenocortical adenoma.

covered incidentally, some may secrete hormones without causing overt clinical syndromes. The most common functioning tumors are the adenomas causing Cushing's syndrome, primary hyperaldostero-nism, or pheochromocytoma. From an accurate history and physical examination, certain pertinent

Figure 12-2. A 64-year-old asymptomatic man had routine abdominal magnetic resonance imaging during a routine physical examination. A, The right adrenal tumor is heterogeneous and measured 4.3 cm x 4.2 cm x 3.5 cm (white arrow). The left adrenal gland is normal. Biochemical workup showed no hyperfunction. He underwent a laparoscopic adrenalectomy. B, Pathology showed a 5 cm adrenal cortical adenoma. The tumor was morsellated for removal. The adenoma appears golden-yellow.

Figure 12-2. A 64-year-old asymptomatic man had routine abdominal magnetic resonance imaging during a routine physical examination. A, The right adrenal tumor is heterogeneous and measured 4.3 cm x 4.2 cm x 3.5 cm (white arrow). The left adrenal gland is normal. Biochemical workup showed no hyperfunction. He underwent a laparoscopic adrenalectomy. B, Pathology showed a 5 cm adrenal cortical adenoma. The tumor was morsellated for removal. The adenoma appears golden-yellow.

Table 12-1. PATHOLOGIES OF ADRENAL INCIDENTALOMAS

Nonfunctioning benign tumors Cortical adenoma Cyst

Myelolipoma Ganglioneuroma

Other: angioma, granuloma, lipoma, hamartoma, fibroma, hemangioma Functioning benign tumors Pheochromocytoma

Cortical adenoma—Cushing's syndrome, primary hyperaldosteronism Malignant tumors

Primary adrenocortical carcinoma Metastatic cancers—melanoma, breast, lung, kidney, stomach, colorectal, pancreas, ovary clinical features such as hypertension—a common feature of all three conditions—or symptoms and signs of glucocorticoid excess in Cushing's syndrome can give clues to the diagnosis. However, biochemical tests, as listed in Table 12-2, remain the hallmark in accurately screening for and diagnosing these conditions.

Negative results usually indicate that the tumor is nonfunctioning. Most of these masses are cortical adenomas that vary in size, whereas others are adrenal cysts (Figure 12-5), myelolipoma, or adrenal hemorrhages that can be identified from certain CT characteristics and easily monitored on

Figure 12-3. Flow diagram for evaluating an incidentaloma.

imaging without removal. As it is unknown at what rate the cortical adenomas grow or whether these tumors can become hormonally active or cancerous, regular follow-up and surveillance are necessary if the adrenal tumor is not resected.

The evaluation process then centers on the likelihood of the adrenal mass being an adrenocortical carcinoma (Table 12-3). These tumors usually grow rapidly and present as large (> 5-6 cm) adrenal masses at the time of diagnosis (Figure 12-6). Clinical findings that support the diagnosis are abdom-

Figure 12-4. A 62-year-old man, who 3 years ago had a liver transplant because of end-stage liver disease owing to hepatitis C, had a computed tomographic (CT) scan because of left flank pain. A, An abdominal CT scan showed a 9 cm left adrenal tumor (white arrow). Fine-needle biopsy of this lesion was most consistent with a metastatic hepatoma. Other studies, including positron emission tomographic scan, showed no other obvious metastasis. He underwent a diagnostic laparoscopy, which showed no obvious other lesions and had a laparoscopic hand-assisted left adrenalectomy. B, The pathology showed a 16 cm x 9 cm x 7 cm metastasis from a hepatoma.

Figure 12-4. A 62-year-old man, who 3 years ago had a liver transplant because of end-stage liver disease owing to hepatitis C, had a computed tomographic (CT) scan because of left flank pain. A, An abdominal CT scan showed a 9 cm left adrenal tumor (white arrow). Fine-needle biopsy of this lesion was most consistent with a metastatic hepatoma. Other studies, including positron emission tomographic scan, showed no other obvious metastasis. He underwent a diagnostic laparoscopy, which showed no obvious other lesions and had a laparoscopic hand-assisted left adrenalectomy. B, The pathology showed a 16 cm x 9 cm x 7 cm metastasis from a hepatoma.

Table 12-2. BIOCHEMICAL SCREENING TESTS FOR THE DETECTION OF FUNCTIONAL TUMORS

Pheochromocytoma

Elevated urinary (24-hour) metanephrines and catecholamines Cushing's syndrome

Elevated urinary (24-hour) cortisol Unsuppressed dexamethasone suppression test Low serum adrenocorticotropic hormone Primary hyperaldosteronism Hypokalemia High serum aldosterone

High ratio of serum aldosterone/plasma renin activity inal or back pain, weakness, and an abdominal mass. Up to 75% of tumors can secrete hormones that cause Cushing's syndrome or virilization or

Figure 12-5. A 26-year-old woman had left upper quadrant pain. A, An abdominal computed tomographic scan showed a 6 cm x 5 cm x 4 cm left adrenal cyst (white arrow) with calcifications in the cystic wall. Biochemical workup showed no hyperfunction. She underwent a laparoscopic left adrenalectomy. B, Pathology showed a 6 cm endothelial cyst.

Figure 12-5. A 26-year-old woman had left upper quadrant pain. A, An abdominal computed tomographic scan showed a 6 cm x 5 cm x 4 cm left adrenal cyst (white arrow) with calcifications in the cystic wall. Biochemical workup showed no hyperfunction. She underwent a laparoscopic left adrenalectomy. B, Pathology showed a 6 cm endothelial cyst.

Table 12-3. FEATURES OF ADRENOCORTICAL CARCINOMA

Rapid growth

Abdominal pain, weakness, abdominal mass, anemia Functional in 75% of cases Large tumor > 5 cm in diameter

Computed tomography—heterogeneous with central necrosis and hemorrhage Magnetic resonance imaging—isotense on Ti-weighted and hyperintense on T2-weighted images Local invasion of adjacent structures

Invasion of vascular structures—renal vein, inferior vena cava Metastasis to regional lymph nodes Metastasis to liver, lung, bone feminization syndromes from excess sex steroid hormone. In particular, these tumors have a tendency to secrete multiple hormones. Abdominal CT or MRI scans, which often reveal a heterogeneous tumor (with an irregular capsule) invasion to surrounding structures such as kidney, renal vein, or inferior vena cava or metastases to the liver or lungs, give definite evidence of an adrenocortical carcinoma. Cytologic features cannot firmly distinguish between a carcinoma and a benign cortical adenoma. Local invasion, lymphadenopathy, or distant adrenal metastases confirms the diagnosis. For this reason, fine-needle biopsy is inadequate in differentiating between these two tumors on cytologic

Figure 12-6. A 60-year-old man had recent onset of severe right upper quadrant pain. A computed tomographic scan shows a 10 cm x 9 cm x 8 cm heterogeneous right adrenal tumor (white arrows). Biochemical workup showed mild hypercortisolism. He underwent open resection of the right adrenal tumor, with intraoperative findings of invasion into the liver and regional lymphadenopathy.

Figure 12-6. A 60-year-old man had recent onset of severe right upper quadrant pain. A computed tomographic scan shows a 10 cm x 9 cm x 8 cm heterogeneous right adrenal tumor (white arrows). Biochemical workup showed mild hypercortisolism. He underwent open resection of the right adrenal tumor, with intraoperative findings of invasion into the liver and regional lymphadenopathy.

criteria. However, fine-needle biopsy can be useful in diagnosing a metastatic lesion of unknown primary origin or for the therapeutic aspiration of a large, symptomatic adrenal cyst. A needle biopsy is contraindicated in a patient with pheochromocy-toma. All functioning tumors should be excluded by biochemical screening and not by biopsy because of the potential risk of lethal, hypertensive crises.

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