Adrenal malignancies

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CHAPTER 29

Adrenal malignancies

1. What types of cancers occur in the adrenal glands?

2. Do adrenocortical carcinomas produce hormones?

3. What are the clinical features of functioning adrenocortical carcinomas?

4. What are the clinical features of nonfunctioning adrenocortical carcinomas?

5. What imaging procedure is best for evaluating an adrenal mass?

Computed tomography (CT) is the method of choice to determine the size and physical characteristics of the mass. Features that most strongly suggest ACC are size greater than 6 cm, heterogeneity, calcifications, irregular borders, local invasion, lymphadenopathy, and decreased lipid content. The last feature is assessed by signal attenuation, expressed in Hounsfield units (HU). An adrenal mass CT protocol is employed in many institutions; this consists of a non-contrast CT, a contrast-enhanced CT, a delayed contrast-enhanced CT, and calculation of the relative washout percentage (RWP) of enhancement. Table 29-1 shows the typical CT findings for some commonly seen adrenal masses. MRI can also be used to assess the size, features, and lipid content of adrenal masses but is more expensive. Fluorodeoxyglucose (18F) positron emission tomography (FDG-PET) or PET-CT fusion scanning may also be useful, especially for masses with high HU or low RWP on CT. High sensitivities and specificities have been reported with this technique using a standard uptake value (SUV) cutoff of 3.1. Other PET tracers, such as metomidate 11C, may offer even better sensitivity in the future.

6. What hormone tests should be used to evaluate an adrenal mass?

The goal is to determine whether the mass is producing hormones that may cause symptoms or that may be indicative of malignancy (androgens). Many experts recommend a focused evaluation consisting of an overnight 1-mg dexamethasone suppression test, measurement of plasma free metanephrines or fractionated urinary catecholamines and metanephrines, and, for hypertensive patients, measurement of plasma aldosterone and renin. Tests currently recommended by the European Network for the Study of Adrenal Tumors (ENSAT) are shown in Table 29-2.

TABLE 29-2.

HORMONAL EVALUATION OF THE INCIDENTAL ADRENAL MASS PROPOSED BY ENSAT

Cortisol testing (3 of 4 tests) Dexamethasone suppression test (1-mg)
  Urine cortisol (24-hour)
  Serum cortisol, basal
  Plasma ACTH, basal
Sex steroid testing (all) Serum testosterone
  Serum dehydroepiandrosterone sulfate (DHEA-S)
  Serum androstenedione
  Serum 17-OH progesterone
  Serum estradiol (men, postmenopausal women)
Aldosterone testing (if hypertension present) Plasma aldosterone
  Plasma renin activity
  Serum potassium
Pheochromocytoma testing (1 or 2 tests) Plasma metanephrines
  Urine metanephrines (24-hour)

ENSAT (European Network for the Study of Adrenal Tumors).

Adapted from Lacroix A: Approach to the patient with adrenocortical carcinoma. J Clin Endocrinol Metab 95: 4812-4822, 2010

7. How should the incidentally discovered adrenal mass be managed?

8. Describe a useful staging system for adrenocortical carcinoma.

9. Describe the initial treatment for an adrenocortical carcinoma.

Surgery is the initial treatment of choice for ACC. If the tumor resection is complete, adjuvant therapy with mitotane, an adrenocorticolytic agent, is recommended because of the high recurrence rate (∼60%). After an incomplete resection, adjuvant mitotane alone or combined with streptozotocin is recommended. Adjuvant tumor bed radiation therapy is also advised by some investigators. Follow-up imaging, with or without tumor marker measurement, should be performed every 3 months for 1 year, then every 6 months for 5 years, and then annually thereafter. In patients receiving mitotane, serum mitotane levels should be monitored and maintained in the 14 to 20 μg/mL range; concomitant glucocorticoid with or without mineralocorticoid replacement therapy is necessary in all patients except those with hypercortisolism. Patients with persistent hypercortisolism despite mitotane use may need additional therapy with an enzyme inhibitor (ketoconazole, metyrapone).

10. How should advanced, metastatic, and recurrent adrenal cortical carcinoma be managed?

11. What is the prognosis for patients with adrenocortical carcinoma?

12. How often are pheochromocytomas malignant?

13. What are the clinical features of a malignant pheochromocytoma?

14. What clues suggest that a pheochromocytoma is malignant?

15. Which of the familial pheochromocytoma syndromes is most commonly associated with malignant pheochromocytomas?

Table 29-5 lists the four well-recognized familial pheochromocytoma and paraganglioma syndromes. Only the succinate dehydrogenase B mutation, an autosomal-dominantly inherited condition, is associated with malignant pheochromocytomas and paragangliomas.

TABLE 29-5.

GENETIC SYNDROMES ASSOCIATED WITH PHEOCHROMOCYTOMAS AND/OR PARAGANGLIOMAS

SYNDROME GENE MUTATION
Multiple endocrine neoplasia 2 Ret
Von Hippel–Lindau syndrome VHL
Neurofibromatosis 1 NF-1
Succinate dehydrogenase B mutation SDH

16. What are the best tests to localize metastatic pheochromocytomas?

17. What is the treatment for a malignant pheochromocytoma?

18. What is the prognosis for malignant pheochromocytoma?

19. What tumors metastasize to the adrenal glands?

20. What is the clinical significance of metastatic disease to the adrenal glands?

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