Nelson Syndrome: A Case Report and Literature Review

Abstract

Objective: Nelson syndrome (NS) is a rare clinical disorder that can occur after total bilateral adrenalectomy (TBA), performed as a treatment for Cushing disease. NS is defined as the accelerated growth of an adrenocorticotropic hormone-producing pituitary adenoma. Our objective is to describe a case of NS and discuss it based on existing knowledge of this syndrome. Methods: We describe the case of a woman diagnosed with NS at our facility in the Instituto Nacional de Ciencias Medicas y Nutricion Salvador Zubiran and review published cases of NS. Results: The patient, a 35-year-old woman with Cushing disease, had been diagnosed in 2006 at the endocrinology department in the Instituto Nacional de Ciencias Medicas y Nutricion Salvador Zubiran. In 2007, a laparoscopic TBA was performed, and 2 years later, she presented with hyperpigmentation and adrenocorticotropic hormone levels of up to 11 846 pg/mL. NS was suspected, and as magnetic resonance imaging showed macroadenoma, transsphenoidal surgery was performed. The patient remained asymptomatic until 2012, when she presented with a right hemicranial headache, photophobia, and phonophobia. A fresh magnetic resonance imaging was performed, which documented tumor growth. She was referred to the Instituto Nacional de Neurologia y Neurocirugia, where she underwent surgery. Conclusion: NS develops as a complication of TBA, which is used as a treatment of Cushing disease. The main treatment is surgery and radiotherapy.

Petroclival Aggressive Pituitary Adenoma in Nelson's Syndrome: 2-Dimensional Operative Video

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Nelson's syndrome (corticotroph tumor progression after bilateral adrenalectomy/Nelson syndrome) is a complication that occurs in 8% to 47% of cases following bilateral total adrenalectomy in patients with refractory Cushing disease, resulting in rapid development of aggressive macroadenomas, associated with elevated plasma adrenocorticotropin hormone (ACTH) levels and skin pigmentation. [1][2][3] From a histopathological point of view, aggressive pituitary adenoma (PA) does not differ from pituitary carcinoma except for the presence of metastases and the higher mortality rate in the latter. [4][5][6][7] Currently, the management of these subgroups of tumors represent a challenge, and a multidisciplinary team approach plays a crucial role in the care of these patients, whose outcome usually remains unfavorable.

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