Bilateral adrenalectomy usually results in lifelong primary adrenal insufficiency. Evidence exists that up to 34% of patients with Cushings disease (CD) have some degree of endogenous cortisol secretion after bilateral adrenalectomy due to ACTH driven hyperplasia of residual cortical tissue. We present a case of a patient with atypical corticotropinoma/carcinoma and recurrence of CD after bilateral adrenalectomy.
A 59-year-old man presented with Cushings disease (CD) in 2010. An MRI revealed intrasellar mass 8×8 mm suggestive of a microadenoma. After the surgery pathohistological evaluation was suggestive of atypical corticotropinoma (Ki-67 proliferation index 7%, positive nuclear staining for p53). CD recurred eight months after the surgery. Total hypophysectomy led to complete remission, followed by recurrence 5 months later. We performed radiosurgical treatment of the remnant tumor mass, along with bilateral two-stage adrenalectomy. Pathohistological evaluation confirmed complete removal of the left adrenal gland, and was inconlusive about the right one. Postoperatively, patient required replacement therapy. Two years after the adrenalectomy patient was diagnosed with Nelsons syndrome due to enlargement of the pituitary mass. Fractionated radiotherapy was performed, after which ACTH slightly decreased, but UFC levels continued to increase, while taking hydrocortisone replacement. Replacement therapy was stopped, but urinary free cortisol increased to 1400 nmol/24 h 3 years after the bilateral adrenalectomy, accompanied by the recurrence of signs and symptoms of CD. Computed tomography showed 4-cm large mass in the left adrenal bed suggestive of an adrenal tissue, along with the multiple liver lesions and without the signs of another primary tumor. Patient died five years after the initial diagnosis and his family refused an autopsy.
This is the first case of a recurrent CD after bilateral adrenalectomy. This report highlights the importance of long-term monitoring of the patient and individual dosing of replacement therapy.
20 - 23 May 2017
European Society of Endocrinology