Long-term outcome after bilateral adrenalectomy in Cushing's disease with focus on Nelson's syndrome

ABSTRACT Objective We analyzed the clinical, biochemical, and imaging findings of adrenalectomized patients with Cushing's disease (CD) in order to compare the characteristics of those who developed Nelson's syndrome (NS) versus those who did not develop this complication (NNS), aiming to identify possible predictive factors for its occurrence. Subjects and methods We performed a retrospective review of the clinical records of a group of patients with CD who underwent TBA between 1974 and 2011. Results Out of 179 patients with CD, 13 (7.3%) underwent TBA. NS occurred in 6 of them (46%) after a mean of 24 months from the total bilateral adrenalectomy (TBA). Age at diagnosis, duration of Cushing's syndrome (CS) until TBA, and steroid replacement doses were similar in both groups. Initial urinary cortisol levels (24-hour urinary free cortisol [UFC]) were significantly higher in the NS group than in the NNS group (p = 0.009). Four patients in the NS group and three of those in the NNS group received radiotherapy before TBA (p = 0.26). Three patients in the NS group presented residual tumors before TBA, compared with none in the NNS group (p = 0.04). At 1 year after TBA, the median ACTH level was 476 ng/L (240-1500 ng/L) in the NS group and 81 ng/L (48-330 ng/L) in the NNS group (p = 0.0007). Conclusion In conclusion, a residual tumor before TBA, higher 24-hour UFC at diagnosis, and increasing ACTH levels within 1 year after TBA emerged as predictive factors of development of NS.

Diagnosis and treatment of Cushing's syndrome caused by bilateral solitary adrenal neoplasma / 中华内分泌代谢杂志

_ Objective_ To evaluate the efficacy of unilateral subtotal adrenalectomy in the treatment of bilateral adrenal solitary neoplasma causing Cushing's syndrome and to elaborate the therapeutic principle. Methods From 2007 to 2013, a total of ten patients were diagnosed with Cushing's syndrome caused by bilateral solitary adrenal neoplasma. We compared patients'clinical symptoms, hormone profiles, biochemical and metabolic parameters, and imaging data before and after the surgery. Five of them chose the optimal neoplasma based on the lateralization ratio of adrenal venous sampling result and the other 5 patients chose the optimal neoplasma based on the diameter of the mass reflected by the computed tomography result and were then operated. Results After the unilateral subtotal adrenalectomy,the24-hour urinary free cortisol decreased significantly(P<0.05)and the midnight serum cortisol level also significantly reduced(P<0. 01). Plasma adrenocorticotropic hormone level increased significantly(P<0. 01). Nine patients of them did not need contralateral adrenalectomy and one patient received contralateral adrelectomy because of the remnant of Cushingnoid symptoms. Conclusion Unilateral subtotal adrenalectomy is an effective and safe way to treat Cushing's syndrome caused by bilateral solitary neoplasma.

Surgical Outcomes of Bilateral Adrenalectomy / 대한내분비외과학회지

PURPOSE: Because the main drawback of bilateral adrenalectomy is permanent adrenal insufficiency and the subsequent risks of life-long steroid use, adrenal preserving partial adrenalectomy is being accepted as its alternative. The aim of this study is to investigate the indications for bilateral adrenalectomy and to assess the postoperative outcomes and steroid replacement according to operative methods. METHODS: From May 1996 through July 2013, a total of 25 patients who underwent bilateral adrenalectomy in our institution were reviewed retrospectively. Surgical outcomes were compared between total and partial adrenalectomy, and postoperative steroid hormone replacement were examined according to the volume of remnant adrenal gland. RESULTS: The median follow-up duration of 25 patients was 55.8 months. The most common indication for bilateral adrenalectomy was bilateral pheochromocytoma (n=16), which was associated with genetic mutation of RET or VHL gene in 11 cases. Cushing's syndrome (n=8) and hyperaldosteronism (n=1) were another indications of bilateral adrenalectomy. Total adrenalectomy was performed in 8 patients and adrenal preserving partial adrenalectomy in 17 patients. Among the 17 patients, only 5 patients needed adrenal hormone replacement and 2 patients had a recurrence at remnant adrenal tissue. CONCLUSION: Adrenal preserving partial adrenalectomy might be a better option for bilateral adrenal tumor than total adrenalectomy because it can reduce complications associated with adrenal insufficiency and recurrence of the disease is not common.

A Case of Bilateral Adrenal Cortical Adenomas Causing Cushing's Syndrome and Primary Aldosteronism

A 38-year old female was admitted to our hospital for further evaluation of an incidentally found cardiac murmur. She had been in a hypertensive state for 5 years but had taken antihypertensive drugs intermittently on her own. Her history revealed that she had become amenorrheic for 7 months and had, had headaches for a few months. Physical examination revealed central obesity and a moon face, but no hirsuitism. When she first visited our hospital, her blood pressure was 260/170 mmHg and grade 2 systolic murmur was audible along the left sternal border. Laboratory studies revealed high levels of 24-hour urine-free cortisol and plasma aldosterone, but a very low level of plasma adrenocorticotropic hormone and plasma renin activity. A low-dose and a high-dose dexamethasone suppression tests, adrenal venous sampling, inferior petrosal sinus sampling, and a renin stimulation test were performed. Bilateral adrenal masses were found on computerized tomographic scanning and magnetic resonance imaging but there was no abnormality of the pituitary gland. The uncontrollable blood pressure and the elevated 24-hour urine-free cortisol and plasma aldosterone levels were corrected by a right-total and left-subtotal adrenalectomy. Pathologic findings were bilateral adrenal cortical adenomas of different cell types. These findings indicate that the adrenal cortical adenomas were tumors that functioned differently, causing Cushing's syndrome and primary aldosteronism in the same patient. A review of, the literature published in English showed that this is the first reported case, of bilateral adrenal adenomas functioning differently.

A Case of Persistent Cushing's Syndrome

A case of persistent Cushing's syndrome in a 25 year-old male is presented. Inspite of bilateral total adrenalectomy twice during two years, there was no clinical improvement. Adrenal scanning revealed no adrenal remnants. ACTH secreting pituitary microadenoma is demonstrated by computed tomographic brain scan and hormonal studies. Selective transsphenoidal microadenomectomy results in clinical and endocrinological improvement.

A Case of Persistent Cushing's Syndrome

A case of persistent Cushing's syndrome in a 25 year-old male is presented. Inspite of bilateral total adrenalectomy twice during two years, there was no clinical improvement. Adrenal scanning revealed no adrenal remnants. ACTH secreting pituitary microadenoma is demonstrated by computed tomographic brain scan and hormonal studies. Selective transsphenoidal microadenomectomy results in clinical and endocrinological improvement.