Category: Adrenal Disorders

Monitor: 30

30 - A UNIQUE PRESENTATION OF NELSON SYNDROME DUE TO PARTIAL ADRENAL INSUFFICIENCY WITHOUT BILATERAL ADRENALECTOMY

Thursday, Apr 25
11:30 AM – 12:00 PM

Objective : Nelson syndrome refers to a spectrum of symptoms and signs arising from an adrenocorticotropin (ACTH) secreting pituitary macroadenoma after a therapeutic bilateral adrenalectomy. The first case was reported by Nelson et al in 1958. We present a case of 58-year-old Asian female with facial hyperpigmentation and ACTH secreting pituitary adenoma who had clinical features similar to Nelson syndrome due to partial adrenal insufficiency, without bilateral adrenalectomy.


Methods : We describe the clinical presentation including the laboratory, imaging and pathology findings as a case report.


Results :
Case presentation:
Patient is a 58-year-old Asian female who presented with severe facial and neck hyperpigmentation. She has history of pituitary macroadenoma, for which she underwent transsphenoidal resection of pituitary tumor. Microscopic examination of specimen with immunostain profile revealed positive ACTH and focally positive prolactin. She had residual tissue extending into right cavernous sinus and received 2 sessions of radiation postoperatively. About 1 year after surgery she noticed hyperpigmentation of her face and neck, for which she was seen by multiple dermatologists and was prescribed multiple medications and topical creams which did not improve her pigmentation. On presentation to Endocrinology, work up revealed high levels of ACTH and low normal AM cortisol. She did not have any other signs or symptoms of adrenal insufficiency. She was started on hydrocortisone which improved her hyper-pigmentation significantly, and her ACTH level is now back to normal reference range.


Discussion : Nelson syndrome may be seen in anywhere from 8-44% of patients who have undergone bilateral adrenalectomy for Cushing disease. The spectrum of clinical features observed relates to the local effects of the tumor on surrounding structures, the secondary loss of other pituitary hormones, and the effects of the high serum concentrations of ACTH on the skin. Almost all cases of Nelson syndrome follow bilateral adrenalectomy in patients who have Cushing disease due to an ACTH-secreting pituitary adenoma. Generally, these cases are managed by radiation to the pituitary or medically with cabergoline, octreotide, cyproheptadine. In our patient use of low dose hydrocortisone decreased the ACTH levels and improved the skin hyperpigmentation significantly suggesting primary adrenal insufficiency as possible cause of excess ACTH production.


Conclusion : Primary adrenal insufficiency should be considered as a cause of excessive ACTH production resulting in hyperpigmentation and ACTH secreting adenoma.

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Raveena Nalla

PGY2, Department of Internal medicine
Monmouth Medical Center, Long Branch, NJ
Long Branch, New Jersey

I was born in 1992 in the state of Telangana in India. With the passion to be a doctor, I entered into my medical school, Osmania medical college, Hyderabad, India after securing the top rank in the State Level Common Medical Entrance Test. I graduated from my medical school in 2016. I joined Monmouth Medical Center, Long branch, NJ for Internal medicine residency in 2017 and I am currently pursuing my second year of residency.

Smita Kargutkar

Owner and Medical Director ACE Endocrinology Associates PC
Monmouth Medical Center, Long Branch NJ 07740
Red Bank, New Jersey

Education: M.B.B.S. - Seth G.S. Medical College and K.E.M. Hospital, Mumbai, India
Residency: M.D. Internal Medicine, Monmouth Medical Center, Long Branch, NJ
Fellowship: M.D. Endocrinology, Diabetes & Metabolism, Albert Einstein Medical Center, Philadelphia, PA
Actively training in Fellowship in Anti-aging and Regenerative Medicine Dr. Smita Kargutkar's Vision: I provide comprehensive and personalized treatment with compassion, in harmony with patient's personal and cultural beliefs. I complement traditional evidence based Western Medicine ( Allopathy) with Traditional Indian medicine (Ayurveda), Natural supplements, Nutraceuticals, Vitamins and Bio-identical hormones when necessary to bring the Best results for my patients.

Raveena Nalla

PGY2, Department of Internal medicine
Monmouth Medical Center, Long Branch, NJ
Long Branch, New Jersey

I was born in 1992 in the state of Telangana in India. With the passion to be a doctor, I entered into my medical school, Osmania medical college, Hyderabad, India after securing the top rank in the State Level Common Medical Entrance Test. I graduated from my medical school in 2016. I joined Monmouth Medical Center, Long branch, NJ for Internal medicine residency in 2017 and I am currently pursuing my second year of residency.