eMedicine Specialties > Oncology > Carcinomas of Endocrine Organs
Adrenal Carcinoma: Treatment & Medication
Updated: Dec 4, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
Treatment
Medical Care
- Because ACs are so rare, clinical series are small and there is only limited prospective evaluation of treatment strategies. Current practices are strongly influenced by expert consensus opinion from a few medical centers that specialize in ACs. The establishment of national tumor registries offers the potential for gathering more comprehensive evidence.
- Medical management encompasses (1) the treatment of endocrine excess syndromes; (2) the use of mitotane or several multiagent chemotherapy regimens; (3) the treatment and prevention of potential complications; and (4) strategies for palliative and terminal care issues, including symptom relief and management.
- Mitotane
- This drug remains the major chemotherapeutic option for the management of AC because it is a relatively specific adrenocortical cytotoxin. It is used as primary therapy, as adjuvant therapy, and as therapy in recurrent or relapsing disease
- Mitotane apparently causes adrenal inhibition without cellular destruction. The exact mechanism of action is unknown. It inhibits cholesterol side-chain cleavage and 11-beta-oxyhydrase reactions. It also appears to reduce the peripheral metabolism of steroids. Alteration of extra-adrenal metabolism of cortisol reduces measurable 17-hydroxy corticosteroid while stimulating the formation of 6-beta-hydroxy cortisol. Plasma levels of corticosteroids do not fall.
- This drug may be considered in the treatment of inoperable adrenal cortical carcinoma (functional, nonfunctional). It controls endocrine hypersecretion in 70-75% of patients. While objective tumor responses often are cited in as many as 20-25% of patients, a study has yet to be conducted with modern imaging techniques and response criteria accepted by clinical oncologists. Tumor response has been reported to correlate with serum levels and often requires several months of continuous therapy. Assaying mitotane levels during therapy is valuable because therapeutic efficacy depends on achieving serum levels of at least 15 mcg/mL.
- Approximately 40% of the drug is absorbed, and approximately 10% of the dose is recovered in the urine as a water-soluble metabolite. Active metabolite excreted in the bile varies from 1-17%. The balance apparently is stored in tissues. Autopsy data indicate that fat tissue is the primary storage site, but it is found in most tissues.
- After therapy, plasma terminal half-life varies from 18-159 days.
- Experience suggests that the best approach is continuous treatment with the maximum possible dosage.
- If tolerated and an improved clinical response appears possible, increase the dose until adverse reactions interfere. The aim is to achieve doses as high as 10-20 g daily.
- The major beneficial effect is on symptoms rather than a specific antitumor or mortality benefit. Generally, treatment benefits are short-lived, and long-term survivors on this therapy are rare.
- The potential benefit of postoperative adjuvant therapy with mitotane is still controversial.
- Terzolo et al conducted a retrospective study of adjuvant mitotane in patients with adrenocortical cancer who had undergone radical surgery for AC. The study included 47 Italian patients who received mitotane postoperatively and control groups of 55 Italian patients and 75 German patients.
- In the Italian patients, baseline features were similar in the treatment and control groups; the German patients were significantly older and had more stage I or II disease than did patients in the mitotane group. Median recurrence-free survival was 42 months in the mitotane group, as compared with 10 months in the Italian control group and 25 months in the German control group. Hazard ratios for recurrence were 2.91 (95% confidence interval [CI], 1.77 to 4.78; P<0.001) and 1.97 (95% CI, 1.21 to 3.20; P=0.005), respectively. Multivariate analysis indicated that mitotane treatment had a significant advantage for recurrence-free survival.6
- In contrast, a retrospective study by Grubbs et al in 28 patients with AC who underwent primary resection found that although the overwhelming majority did not receive adjuvant mitotane, the recurrence rate was 50%—indistinguishable from the 49% recurrence rate reported by Terzolo et al for patients who received adjuvant mitotane.7
- Terzolo et al conducted a retrospective study of adjuvant mitotane in patients with adrenocortical cancer who had undergone radical surgery for AC. The study included 47 Italian patients who received mitotane postoperatively and control groups of 55 Italian patients and 75 German patients.
- Some reports exist of the potential utility of streptozotocin in combination with mitotane (at a dose of 1 g qd for first 5 d followed by 2 g q3-4wk thereafter). This regimen has been reported to be associated with a significantly better disease-free interval and with more long-term survivors.
- Suramin: Although a few reports suggest the potential utility of suramin as an additional chemotherapeutic agent in the treatment of AC, this drug is not recommended for AC.
- Gossypol
- Gossypol also has been tried for metastatic adrenal cancer, with limited experience and success.
- It was originally developed as a spermatotoxin and was derived from cottonseed oil. It has been used widely in China as a male contraceptive with few adverse effects. While the exact mechanism for its action is unclear, it is known to cause selective mitochondrial destruction by the uncoupling of oxidative phosphorylation.
- Cisplatin-based chemotherapy
- In cases where mitotane fails, chemotherapeutic regimens containing cisplatin alone or in combination often are used. It also is used often in combination with ongoing mitotane administration.
- Cyclophosphamide, Adriamycin (doxorubicin), and cisplatin (CAP); 5-fluorouracil, Adriamycin, and cisplatin (FAP); and cisplatin with etoposide-16 have been tried.
- Ronchi et al found that, as with other types of cancer, expression of excision repair cross complementing group 1 (ERCC1) by ACs predicts resistance to platinum-based chemotherapy. Median overall survival after platinum treatment was 8 months in patients with high ERCC1 versus 24 months in those with low ERCC1 expression (hazard ratio 2.95, 95% confidence interval 1.4-6.2, P=0.004).8
- An international phase III trial comparing the most promising regimens (etoposide, doxorubicin, cisplatin plus mitotane, and streptozotocin plus mitotane) is currently under way.9
- Further improvements in therapy may utilize novel chemotherapy agents, vascular growth inhibitors, or small-molecule therapy based on a better understanding of the molecular pathways involved in tumorigenesis.
- Management of endocrine syndromes
- In functional tumors, management of the endocrine syndromes is often important because the associated systemic effects may significantly impact patient well-being.
- Therapeutic options for Cushing syndrome include mitotane, ketoconazole, metyrapone, aminoglutethimide, RU 486 (mifepristone), and intravenous etomidate, alone or in various combinations.
- For hyperaldosteronism, the major therapeutic options are spironolactone, elpreninone (presently in phase 3 trials), amiloride, triamterene, and various antihypertensives, especially long-acting dihydropyridine calcium channel blockers.
- For hyperandrogenism or hyperestrogenism, several options are available if adverse effects from these hormones significantly impact patient well-being. Antiestrogens may include clomiphene citrate, tamoxifen, toremifene, and danazol. Potential antiandrogens include flutamide, cyproterone acetate, bicalutamide (Casodex), nilutamide, and megestrol acetate. Ketoconazole, spironolactone, and cimetidine also have a significant antiandrogen effect. The various aromatase inhibitors (eg, testolactone, anastrozole, letrozole, fadrozole) also have some antiandrogen effect; therefore, they may be used. Controlled studies have not yet been performed to assess which of these agents, either alone or in combination, achieves the best metabolic control. The choice of medication often is guided by cost, availability, patient preference, adverse effects, and tolerance.
- In the rare setting of mixed carcinoma associated with pheochromocytoma components, high-dose radiolabeled metaiodobenzylguanidine (MIBG) has a potential role.
- The management of blood pressure elevation in this setting is similar to that in pheochromocytoma, using long-acting alpha-blockers (usually with phenoxybenzamine), followed by long-acting beta-blockers (eg, propranolol) and, finally, metyrosine. Evidence does not exist that suggests a combination of radiotherapy with mitotane (or any other chemotherapeutic regimen for that matter) confers any survival benefit. The only place for radiotherapy in the management of this condition is for palliation of painful bone disease and local luminal obstructive disease.
- Patients treated with mitotane may present with features of both glucocorticoid and aldosterone insufficiency requiring replacement therapy.
- Radiation therapy
- Some experts recommend that the use of radiation therapy should be restricted to palliation of local disease such as bony metastases.
- A meta-analysis by Polat et al suggests that radiotherapy to the tumor bed may be considered in patients at high risk for local recurrence. These researchers recommend administering a total dose of > 40 gray (Gy) with single fractions of 1.8 Gy to 2 Gy (including a boost volume to reach from 50 Gy to 60 Gy in individual patients).10
- A few reports suggest that a potential use for radiation therapy is the palliative management of symptomatic metastases to bone or brain, or vena cava obstruction.10
Surgical Care
- Preoperative screening
- Include a full evaluation to determine the extent of disease and staging, which has implications for the ultimate prognosis.
- The most common sites for metastases are the lungs, liver, bone, and lymph nodes. Contiguous spread to the kidney and liver (if the primary is on the right side) and tumor extension into the venous drainage system of the adrenals and the inferior vena cava are common.
- Surgical resection
- When feasible, total resection remains the management modality of choice for the definitive management of AC. It also remains the only potentially curative therapeutic modality.
- Recurrent local and metastatic disease is common, even among patients who undergo a successful complete resection. In such settings, the only effective treatment is attempted reoperation.
- Preoperative diagnostic accuracy should improve in the following years with improved MRI technology, percutaneous core needle biopsy technology, and advances in molecular, genetic, and immunotyping interpretation.
- While open laparotomy for adrenalectomy remains the standard of care, several reports suggest a role for laparoscopic resection in those cases where the adrenal tumor is small and where no evidence of metastatic disease is present preoperatively.11,12 Aggressive resection of locally recurrent disease may prolong survival in some patients, while percutaneous radiofrequency ablation (RAF) may have a place in providing local symptom control related to local compression by an invasive tumor.
Medication
The goal of pharmacotherapy is to reduce morbidity, prevent complications, and eradicate the carcinoma if possible.
Chemotherapeutic agents
These agents inhibit cell growth and proliferation.
Mitotane (Lysodren)
An option for the management of AC because it is a relatively specific adrenocortical cytotoxin.
Adult
2-6 g/d PO divided tid/qid, titrate to 9-10 g/d PO
Begin with 2-3 g/d and advance as tolerated in increments of 0.5 g/wk if tolerated
Use serum levels to guide therapy; target to levels of 10-15 mcg/mL; in most patients, this is achieved after several weeks to months of 5-6 g/d
Maintenance: Usually 2-16 g/d PO but maintenance dose is that which achieves a steady serum level of 10-15 mcg/mL
Smaller patients generally require lower doses; those receiving long-term therapy may require dose reduction to as low as 0.5-1 g/d
After 2 wk of therapy, add replacement hydrocortisone and fludrocortisone
Pediatric
Not established
CNS depressants may increase toxicity; may increase metabolism of warfarin, causing a decrease in levels; spironolactone may decrease effects
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Because primary effect is adrenal suppression, discontinue in case of trauma or shock and institute adrenal replacement therapy; caution in severe hepatic impairment, CNS impairment (lethargy, sedation, vertigo), and neuropsychiatric syndromes; speech impairment, gynecomastia, and hematologic abnormalities also may occur
More on Adrenal Carcinoma |
| Overview: Adrenal Carcinoma |
| Differential Diagnoses & Workup: Adrenal Carcinoma |
 Treatment & Medication: Adrenal Carcinoma |
| Follow-up: Adrenal Carcinoma |
| References |
| Further Reading |
| « Previous Page | Next Page » |
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Further Reading
Related eMedicine topics
Adrenal Carcinoma (Pediatrics)
Adrenal Carcinoma (Radiology)
Adrenal Metastases (Radiology)
Keywords
adrenal carcinoma, AC, adrenocortical carcinoma, adrenal cancer, adrenocortical cancer, primary adrenocortical malignancies, malignant adrenocortical neoplasms, malignant adrenal tumors, malignant adrenocortical tumors, adrenocortical masses, adrenal incidentalomas
