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Endocrine Abstracts (2023) 90 EP680 | DOI: 10.1530/endoabs.90.EP680

Endocrinology Research Centre, Moscow, Russia


Background: Nelson syndrome is a rare complication of Cushing disease (CD), which occurs either in patients with very aggressive CD or as a result of misdiagnosis. Clinical characterization of patients with Nelson syndrome could provide important insights in management of the disorder.

Methods: We analyzed medical records of patients with CD between 2015 and 2021 to identify those who developed Nelson syndrome.

Results: Nine patients developed Nelson syndrome: 2 males and 7 females. Median age of CD onset 27.0 years [20.8;29;8]. Prior to adrenalectomy, 3 patients presented with macroadenomas, 5 – microadenomas, in 1 patient adenoma was not visualized. Seven patients received CD treatment before adrenalectomy: 5 patients underwent multiple transsphenoid neurosurgeries and radiation therapy, 2 were treated only surgically. Mean duration of CS before adrenalectomy was 62 months [48;89]. Mean duration between adrenalectomy and confirmation of Nelson syndrome was 25.5 months [18.5;36.3]. All nine patients developed Nelson syndrome: all had elevated morning ACTH levels – 1176 pg/ml [434;1713], pituitary tumor regrowth or visualization was seen in 6 patients, 4 patients presented with darkened skin tone, 3 – with severe headaches. Median follow-up duration after Nelson diagnosis was 28 months [17;75]. All patients received glucocorticoid replacement therapy – median dose 35 mg/day [25;35]. Three patients received medical therapy, either with cabergoline, long-acting octreotide or lanreotide. Combination of neurosurgery an pituitary radiation was used to treat 3 patients, 2 patients underwent only surgery and 2 received only radiation therapy. At last follow-up visit, 7 patients had pituitary adenoma remnant (5 macro-, 2 microadenoma): without further progression in 6 cases, 1 patient with tumor progression was referred for pituitary irradiation. In 2 patients no signs of regrowth was found on pituitary MRI. Median ACTH during last follow-up was 715.5 pg/ml [453.4;1173].

Conclusion: Nelson syndrome is a severe complication of CD which requires a strict long-term follow-up and all the treatment options to increase the chances of stabilization. In our cohort, combination of treatment modalities led to stabilization of tumor growth in 8 cases, however, ACT remained elevated in all patients. Further reports on this complication could help to identify optimal management strategies and improve the outcomes for Nelson syndrome.

Volume 90

25th European Congress of Endocrinology

Istanbul, Turkey
13 May 2023 - 16 May 2023

European Society of Endocrinology 

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