Introduction: Nelson’s syndrome (NS) represents a rare but severe, potentially life-threatening condition, displayed as a complication after bilateral adrenalectomy was done for Cushing’s disease (CD), involving corticotropinoma increase and ACTH (Adrenocorticotropic Hormone) elevation. Case report: This is the case of a 65-year old male diagnosed in 1987 with CD caused by a macrocorticotropinoma of 1 cm. Bilateral adrenalectomy was performed during the same year (classic approach with one month between sides) and external pituitary radiotherapy during next year. The patient was further under substitution for chronic adrenal insufficiency. In 2003 the adenoma was larger by 0.2 cm maximum diameter. In 2010, an extremely ACTH increase to 1250 pg ml (Normal levels between 7.2 and 62.3 pg mL) was consistent for NS without significant increase of the pituitary mass. For the next four years the annual assays showed a progression of ACTH with stationary pituitary imagery scan and normal eye field exam. The patient refused surgery so external pituitary radiation therapy was done once again. One year later, he was re-examined: normal body mass index was found in association with a blood pressure of 130 80 mmHg under daily prednisone 7.5 mg and fludrocortisone 0.1 mg; still associating skin and oral hyperpigmentation at the gingival level, palatal mucosa, and oral mucosa. ACTH responded to therapy and decreased to 715 pg mL, showing a partial control of NS thus close follow-up is advised.
Conclusion: Currently, bilateral adrenalectomy is rarely used for CD compared to what was done three – four decades ago; the consecutive adrenal insufficiency in association with remaining hypophyseal tumor may complicate with NS, a condition with less described protocols of follow-up and treatment. However, if pituitary surgery which remains the first option is not feasible, radiotherapy, as mentioned case, improves the ACTH levels, especially if the tumor mass does not associate severe local anatomical compression.Full text sources