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Case History

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Image not available. A 55-year-old woman has been diagnosed with metastatic adrenocortical carcinoma. She is unwell and cushingoid.

What should be the medical care? What treatments are available for her?

What is the role of mitotane?

What is the management of adrenal suppression and replacement?

Is there a role for chemotherapy?

How do you control Cushing's syndrome?

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Background

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What should be the medical care? What treatments are available for her?

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Image not available. Adrenal cortical carcinoma (ACC) is an uncommon tumour which occurs at all ages and in both sexes, accounting for approximately 0.02% of all cancer.1 The average age at presentation ranges from approximately 40 to 50 years and it occurs equally frequently in men and women (1:1). Most cases represent non-functioning tumours.2 About two-thirds of all patients with ACC have tumours that hypersecrete glucocorticoids and or androgens, and one-third of patients have classic Cushing's syndrome. In addition to symptoms of hormonal excess, patients with ACC commonly present with an abdominal mass, weight loss and other constitutional symptoms.

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The mainstay of current treatment for ACC is complete surgical excision at the time of initial evaluation.3 If metastatic disease is limited, there remains an apparent benefit of surgery aimed at rendering the patient free of measurable disease. The role of radiotherapy in ACC has not been well defined and is usually regarded as of limited benefit.4 However, palliative radiotherapy for metastatic disease has been shown to be effective in a considerable proportion of patients, and it is the treatment choice for bone metastases (30–40 Gy). More importantly, adjuvant postoperative radiation therapy may have a role in patients at high risk for local recurrence.

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Medical treatment aims to control hormone hypersecretion and - more importantly - partial or complete remission of tumour spread (Figure 49.1).

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Figure 49.1

Algorithm of management of metastatic adrenal cortical carcinoma.

Graphic Jump Location
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Discussion

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What is the role of mitotane?

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Image not available. Mitotane is the only adrenal-specific agent available for the treatment of ACC. Mitotane (1,1 dichloro-2(O-chlorophenyl)-2-(p-chloro-phenyl) ethane) is an isomer of the insecticide p,p′-DDD and a chemical congener of the insecticide DDT. It is an adrenolytic compound with specific activity on the adrenal cortex.5 Mitotane's therapeutic effects depend on intra-adrenal metabolic transformation, and its clinical efficacy remains disputed. Response rate of 35% has been reported, with mostly partial and transient responses and only an occasional complete remission. More recent series have reported lower response rates.6 The role of mitotane as adjuvant therapy after complete surgical removal of ACC remains a matter of debate.

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Mitotane is either given as tablets (Lysodren, Bristol Myers Squibb, Princeton, USA) usually at a dose of >3 g/day or as capsules of micronized mitotane mixed ...

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