ISSN 1849-9031 (Online)

ISSN 1849-8922    (Print)

Recurrence of Cushing’s disease after bilateral adrenalectomy: A myth or reality?
Ivan Vičić, Ivan Kruljac, Miroslav Ćaćić, Jelena Marinković Radošević, Gorana Mirošević, Hrvoje Ivan Pećina, Vatroslav Čerina, Leo Pažanin, Milan Vrkljan.


Bilateral adrenalectomy usually results in lifelong primary adrenal insufficiency. Evidence exists that up to 34% of patients with Cushing’s disease (CD) have some degree of endogenous cortisol secretion after treatment; however, it is unusual that overt recurrence persists even after the removal of the replacement therapy. We present a case of a patient with an atypical corticotropinoma/carcinoma and CD recurrence after bilateral adrenalectomy. A 59-year-old man presented with CD in 2010 and underwent a transsphenoidal adenomectomy. Pathohistological evaluation suggested an 8 mm × 8 mm atypical corticotropinoma. CD recurred 8 months after surgery. A total hypophysectomy was performed, which led to complete remission, followed by recurrence 5 months later. Subsequently, a bilateral two-stage adrenalectomy was performed along with radiosurgical treatment. Postoperatively, the patient received glucocorticoid replacement therapy. 2 years after the adrenalectomy, the patient was diagnosed with Nelson’s syndrome. Fractionated radiotherapy was given, and ACTH levels slightly decreased, but urinary free cortisol (UFC) continued to increase. Glucocorticoid therapy was stopped, but UFC increased to 1400 nmol/24 h (normal range 54-319 nmol/24 h) 3 years after the adrenalectomy, accompanied by the recurrence of signs and symptoms of CD. Abdominal computed tomography showed a 4 cm large mass in the left adrenal bed suggestive of adrenal tissue, along with multiple liver lesions, without signs of another primary tumor. The patient died 5 years after initial diagnosis. This is the first case of recurrent CD after bilateral adrenalectomy. This report highlights the importance of long-term patient monitoring after total bilateral adrenalectomy and individual dosing of replacement therapy.