Cushing’s Disease: Preconception Management

Abstract

The desire for future pregnancy affects the management of recurrent Cushing’s syndrome (CS). In contrast to the initial presentation, where surgery is the optimal treatment, there are several reasonable choices for treatment of recurrent Cushing’s disease (CD). These include repeat surgery, radiation therapy with adjunctive cortisol-lowering medical agents, medical therapy alone, and bilateral adrenalectomy. The patient’s age and potential fecundability influence the decision, as do the advantages and disadvantages of each potential treatment. When pregnancy is desired in the near term, a surgical method provides the fastest time to eucortisolism, with 100% certainty for bilateral adrenalectomy, and 42–73% chance of remission with transsphenoidal exploration. However, adrenalectomy requires lifelong replacement of glucocorticoids and mineralocorticoids, with the risk of adrenal crisis, and complicates the management of hyperemesis gravidarum. Radiation therapy can take 10 years or more to achieve eucortisolism and carries a risk of hypogonadism. There is relatively little or no published experience with most medical treatments. In general, medical therapy is not recommended for women who wish to attempt pregnancy with the caveat that metyrapone has been used off-label to control hypercortisolism in pregnancy. Recurrence in a patient with ectopic ACTH secretion is nearly always caused by metastasis. Preconception management of recurrent Cushing’s syndrome requires shared decision-making with the patient/couple based on a frank discussion of the advantages and disadvantages of the options for each individual patient and the natural history of the causal tumor.