Skip to main content
Key Specialties
Cardiology Diabetology Endocrinology Gastroenterology Geriatrics and Gerontology Gynecology and Obstetrics Hematology Infectious Disease Internal Medicine Nephrology Neurology Oncology Pediatrics Respiratory Medicine Rheumatology Surgery
Congress Coverage
2024 ACC Congress 2023 ESMO Congress 2023 EASD Congress 2023 ERS Congress 2023 ESC Congress 2023 EHA Congress 2023 ASCO Annual Meeting
Clinical Collections
Advances in Alzheimer’s

At a glance: The ONWARDS insulin icodec trials

A round-up of the ONWARDS phase 3 clinical trials evaluating the novel weekly insulin icodec in people with diabetes.
ADVANCED SEARCH
Log in
Top
World Journal of Surgery
Published in: World Journal of Surgery 7/2013

01-07-2013

The Role of Unilateral Adrenalectomy in Corticotropin-Independent Bilateral Adrenocortical Hyperplasias

Authors: Yunze Xu, Wenbin Rui, Yicheng Qi, Chongyu Zhang, Juping Zhao, Xiaojing Wang, Yuxuan Wu, Qi Zhu, Zhoujun Shen, Guang Ning, Yu Zhu

Published in: World Journal of Surgery | Issue 7/2013

Login to get access

Abstract

Background

The objective of the present study was twofold: to demonstrate our experience with unilateral adrenalectomy in the treatment of adrenocorticotropic hormone (ACTH)-independent Cushing syndrome (CS) caused by bilateral adrenocortical hyperplasias, and to evaluate the long-term results as evidenced by the main laboratory and clinical findings.

Methods

From February 2000 to August 2009, unilateral adrenalectomy was performed on 27 patients with ACTH-independent CS and bilateral adrenocortical hyperplasias, including 14 patients with ACTH-independent macronodular adrenal hyperplasia (AIMAH) and 13 patients with primary pigmented nodular adrenocortical disease (PPNAD). Signs and symptoms of CS, endocrine examinations, and radiographic imaging were evaluated preoperatively and postoperatively.

Results

At a median follow-up of 69 months (range: 23–120 months) for AIMAH and 47 months (range: 16–113 months) for PPNAD, 25 patients were cured by unilateral adrenalectomy. Serum cortisol level, daily urinary free cortisol (UFC), and plasma ACTH regained the normal range in both AIMAH and PPNAD patients at monthly follow-up visits; the circadian serum cortisol rhythm returned to normal, and a normal responsiveness to overnight low-dose dexamethasone administration (LDDST) became obvious. Both systolic and diastolic blood pressure (BP) levels were significantly reduced: 85 % of patients recovered normal BP levels, and the remaining patients need antihypertensive drugs, but at a reduced dose. No surgery-related morbidity occurred, and there was no sign of further enlargement of the residual adrenal gland after successful unilateral adrenalectomy. One patient with PPNAD and another patient with AIMAH with similar weights and sizes of the bilateral adrenals needed contralateral adrenalectomy.

Conclusions

Unilateral adrenalectomy may be the suitable treatment for selected patients with AIMAH and PPNAD. It can achieve long-term remission of CS and improve glycemic control and BP values.
Literature
1.
Lacroix A, Bourdeau I (2005) Bilateral adrenal Cushing’s syndrome: macronodular adrenal hyperplasia and primary pigmented nodular adrenocortical disease. Endocrinol Metab Clin North Am 34:441–458 xPubMedCrossRef
2.
3.
Stratakis CA, Kirschner LS (1998) Clinical and genetic analysis of primary bilateral adrenal diseases (micro- and macronodular disease) leading to Cushing syndrome. Horm Metab Res 30:456–463PubMedCrossRef
4.
Lamas C, Alfaro JJ, Lucas T et al (2002) Is unilateral adrenalectomy an alternative treatment for ACTH-independent macronodular adrenal hyperplasia?: long-term follow-up of four cases. Eur J Endocrinol 146:237–240PubMedCrossRef
5.
Arnaldi G, Angeli A, Atkinson AB et al (2003) Diagnosis and complications of Cushing’s syndrome: a consensus statement. J Clin Endocrinol Metab 88:5593–5602PubMedCrossRef
6.
Bertagna X, Guignat L, Groussin L et al (2009) Cushing’s disease. Best Pract Res Clin Endocrinol Metab 23:607–623PubMedCrossRef
7.
Ilias I, Torpy DJ, Pacak K et al (2005) Cushing’s syndrome due to ectopic corticotropin secretion: twenty years’ experience at the National Institutes of Health. J Clin Endocrinol Metab 90:4955–4962PubMedCrossRef
8.
Nieman LK, Biller BM, Findling JW et al (2008) The diagnosis of Cushing’s syndrome: an Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab 93:1526–1540PubMedCrossRef
9.
Gunther DF, Bourdeau I, Matyakhina L et al (2004) Cyclical Cushing syndrome presenting in infancy: an early form of primary pigmented nodular adrenocortical disease, or a new entity? J Clin Endocrinol Metab 89:3173–3182PubMedCrossRef
10.
Kirschner MA, Powell RD Jr, Lipsett MB (1964) Cushing’s syndrome: nodular cortical hyperplasia of adrenal glands with clinical and pathological features suggesting adrenocortical tumor. J Clin Endocrinol Metab 24:947–955PubMedCrossRef
11.
Aiba M, Hirayama A, Iri H et al (1991) Adrenocorticotropic hormone-independent bilateral adrenocortical macronodular hyperplasia as a distinct subtype of Cushing’s syndrome. Enzyme histochemical and ultrastructural study of four cases with a review of the literature. Am J Clin Pathol 96:334–340PubMed
12.
Lacroix A, Ndiaye N, Tremblay J et al (2001) Ectopic and abnormal hormone receptors in adrenal Cushing’s syndrome. Endocr Rev 22:75–110PubMedCrossRef
13.
Zeiger MA, Nieman LK, Cutler GB et al (1991) Primary bilateral adrenocortical causes of Cushing’s syndrome. Surgery 110:1106–1115PubMed
14.
Stratakis CA, Kirschner LS, Carney JA (2001) Clinical and molecular features of the Carney complex: diagnostic criteria and recommendations for patient evaluation. J Clin Endocrinol Metab 86:4041–4046PubMedCrossRef
15.
Goodarzi MO, Dawson DW, Li X et al (2003) Virilization in bilateral macronodular adrenal hyperplasia controlled by luteinizing hormone. J Clin Endocrinol Metab 88:73–77PubMedCrossRef
16.
Lacroix A (2009) ACTH-independent macronodular adrenal hyperplasia. Best Pract Res Clin Endocrinol Metab 23:245–259PubMedCrossRef
17.
Doppman JL, Nieman LK, Travis WD et al (1991) CT and MR imaging of massive macronodular adrenocortical disease: a rare cause of autonomous primary adrenal hypercortisolism. J Comput Assist Tomogr 15:773–779PubMedCrossRef
18.
Doppman JL, Chrousos GP, Papanicolaou DA et al (2000) Adrenocorticotropin-independent macronodular adrenal hyperplasia: an uncommon cause of primary adrenal hypercortisolism. Radiology 216:797–802PubMed
19.
20.
Doppman JL, Travis WD, Nieman L et al (1989) Cushing syndrome due to primary pigmented nodular adrenocortical disease: findings at CT and MR imaging. Radiology 172:415–420PubMed
21.
Sahdev A, Reznek RH, Evanson J et al (2007) Imaging in Cushing’s syndrome. Arq Bras Endocrinol Metab 51:1319–1328CrossRef
22.
Shen WT, Lee J, Kebebew E et al (2006) Selective use of steroid replacement after adrenalectomy: lessons from 331 consecutive cases. Arch Surg 141:771–774 discussion 774–776PubMedCrossRef
23.
Storr HL, Mitchell H, Swords FM et al (2004) Clinical features, diagnosis, treatment and molecular studies in paediatric Cushing’s syndrome due to primary nodular adrenocortical hyperplasia. Clin Endocrinol (Oxf) 61:553–559CrossRef
24.
Sato M, Soma M, Nakayama T et al (2006) A case of adrenocorticotropin-independent bilateral adrenal macronodular hyperplasia (AIMAH) with primary hyperparathyroidism (PHPT). Endocr J 53:111–117PubMedCrossRef
25.
Vezzosi D, Cartier D, Regnier C et al (2007) Familial adrenocorticotropin-independent macronodular adrenal hyperplasia with aberrant serotonin and vasopressin adrenal receptors. Eur J Endocrinol 156:21–31PubMedCrossRef
Metadata
Title
The Role of Unilateral Adrenalectomy in Corticotropin-Independent Bilateral Adrenocortical Hyperplasias
Authors
Yunze Xu
Wenbin Rui
Yicheng Qi
Chongyu Zhang
Juping Zhao
Xiaojing Wang
Yuxuan Wu
Qi Zhu
Zhoujun Shen
Guang Ning
Yu Zhu
Publication date
01-07-2013
Publisher
Springer-Verlag
Published in
World Journal of Surgery / Issue 7/2013
Print ISSN: 0364-2313
Electronic ISSN: 1432-2323
DOI
https://doi.org/10.1007/s00268-013-2059-9

Other articles of this Issue 7/2013

World Journal of Surgery 7/2013 Go to the issue

OriginalPaper

Clinical Implication of Cancer Adhesion in Papillary Thyroid Carcinoma: Clinicopathologic Characteristics and Prognosis Analyzed with Degree of Extrathyroidal Extension

OriginalPaper

Notch1 Contributes to Chemoresistance to Gemcitabine and Serves as an Unfavorable Prognostic Indicator in Pancreatic Cancer

OriginalPaper

Utilisation of a Level 1 Trauma Centre in KwaZulu-Natal: Appropriateness of Referral Determines Trauma Patient Access

OriginalPaper

Prophylactic Lymph Node Dissection in Papillary Thyroid Carcinoma: Is There a Place for Lateral Neck Dissection?

Erratum

Erratum to: History of Biliary Surgery

OriginalPaper

Differentiated Thyroid Cancer Presenting with Distant Metastases: A Population Analysis Over Two Decades