1. M Iacobone, G Viel, S Zanella, M Frego, G Favia
THE ROLE OF UNILATERAL ADRENALECTOMY IN ACTH-INDEPENDENT MACRONODULAR ADRENAL HYPERPLASIA (AIMAH)
M Iacobone,
G Viel, S Zanella, M Frego,
G Favia
Department of Endocrine Surgery
University of Padua, Italy

2. - ACTH-Independent Macronodular Adrenal Hyperplasia (AIMAH)
Cushing’s Syndrome
ACTH dependent (80%)
Pituitary
Ectopic
ACTH independent (20%)
Unilateral
(Adenoma, Carcinoma)
Bilateral
- PPNAD
- ACTH-Independent Macronodular Adrenal Hyperplasia (AIMAH)

3. ACTH-Independent Macronodular Adrenal Hyperplasia (AIMAH)
Slowly Progressing Disease
Mild Hypercortisolism
Bilateral Macronodules
Ectopic Receptors
(GIP, Catecholamines, LH/hCG)

4. Bilateral Adrenalectomy Partial adrenalectomies
AIMAH - Therapy
Lifetime steroid
replacement
Bilateral Adrenalectomy
Octreotide
Propanolol
Leuprolide
Medical Treatment
Ectopic Receptors
Subtotal
Unilateral
Partial adrenalectomies

5. of Unilateral Adrenalectomy for AIMAH
AIM of the STUDY
Long-term results
of Unilateral Adrenalectomy for AIMAH

6. PATIENTS and METHODS (1)
Jan 01-Dec 05
Unilateral Adrenalectomy and AIMAH
(n=7)
Males: 2 ; Females: 5
Age: 55 yr (36 – 71)
DIAGNOSIS:
Clinical, Laboratory, Imaging, Pathology
Cushing’s Syndrome

7. CT/MRI + I-131 norcholesterol Scintigraphy
PATIENTS ET METHODS (2)
CT/MRI + I-131 norcholesterol Scintigraphy
Asymmetric involvement
Unilateral adrenalectomy

9. Glycometabolic Parameters Body Mass Index (BMI)
PATIENTS ET METHODS (3)
Serum ACTH and Cortisol
UFC
Circadian rhythm
DMX suppression test
Hormonal Parameters
Blood Pressure
Glycometabolic Parameters
Body Mass Index (BMI)
Subjective Perception of Quality of Life (SF-36: MCS + PCS)
OGTT
HbA1c

10. CURE: 86% Persistent hypercortisolism: 1 case RESULTS (1)
Morbidity: 0%
CURE: 86%
Persistent hypercortisolism: 1 case
Completion contralateral adrenalectomy
Large contralateral remaining adrenal
Symmetric uptake at scintigraphy

11. Normal range CURE: 6 PATIENTS RESULTS (2)
Follow-up: 53 months (range 27-68)
CURE: 6 PATIENTS
HORMONAL PARAMETERS
Normal range
Serum ACTH
Serum Cortisol
Urinary free Cortisol
Normal Circadian rhythm
Normal DMX suppression test

12. CURE: 6 PATIENTS RESULTS (2) Follow-up: 53 months (range 27-68)
“Small” contralateral remaining adrenal
Asymmetric uptake at scintigraphy

13. HORMONAL PARAMETERS RESULTS (3) ACTH UFC 0,006 0,04 ACTH
PRE-OP
POST-OP p
ACTH
(nv ng/L)
7 + 2
0,006
Urinary Free Cortisol
(nv nmol/day)
0,04
ACTH
UFC

14. HYPERTENSION RESULTS (4) 0,006 0,001 RECOVERY: 50% DRUG REDUCTION: 50%
BLOOD PRESSURE
PRE-OP
POST-OP p
Systolic BP (mmHg)
0,006
Diastolic BP (mmHg)
92 + 5
79 + 4
0,001
HYPERTENSION
RECOVERY: 50%
DRUG REDUCTION: 50%

15. DIABETES 0,005 0,002 RESULTS (5) OGTT (nv <7.1 mmol/L)
GLUCOSE METABOLISM
PRE-OP
POST-OP p
OGTT (nv <7.1 mmol/L)
0,005
HbA1c (nv 3,7–6,1%) % %
0,002
DIABETES
RECOVERY: 40%
DRUG REDUCTION: 40%

16. RESULTS (6) BMI (nv<25 kg/mq) 0,03
BODY MASS INDEX
PRE-OP
POST-OP p
BMI (nv<25 kg/mq)
28,7 + 5
25,8 + 3,5
0,03

17. Physical Component Summary
RESULTS (7)
SF-36 QUALITY OF LIFE * *
* p<0,01
Physical Component Summary
Mental Component
Summary

18. (case selection) Unilateral adrenalectomy EFFECTIVE TREATMENT
CONCLUSIONS (1) AIMAH
Asymmetric adrenal involvement
“Small” remaining gland
(case selection)
Unilateral adrenalectomy
EFFECTIVE TREATMENT

19. Postoperative adrenal insufficiency
Table 3. Literature review: unilateral adrenalectomy for AIMAH.
Author
Number of cases
Follow-up (months)
Postoperative adrenal insufficiency
(length)
Outcome
Lamas [9] 4 74
(range )
2 cases
(60 and 14 months)
Cure of CS (4 cases)
·   Normalization of serum cortisol, UFC and ACTH
·   Abnormal circadian cortisol rhythm
·   Abnormal responsiveness to dexamethasone suppression test
·   No enlargement of the remaining gland
Vezzosi [12] 1 7
1 case
(6 months)
Cure of CS
·   Normalization of UFC, serum cortisol
·   Normal responsiveness to dexamethasone suppression test
Lacroix [2, 6] 36
(15 months)
·   Subnormal ACTH
Sato [13] 8
(8 months)
·   Subnormal serum cortisol and UFC
Ogura [11] 24 -
·   Normal ACTH, serum cortisol
·   Normal circadian cortisol rhythm
·   Subnormal UFC
·   Unchanged BMI and BP
·   Improvement of glycemic control
Imohl [10] 27
·   Normalization of serum cortisol and circadian cortisol rhythm
N’Diaye [7] 12
1 cases
(12 months)
·   Subnormal, serum cortisol and UFC
·   Mild enlargement (3 mm) of the remaining gland
Doppmann [8] 64
Present series
(WJS 2008) 53
(range 7-68)
(7 and 24 months)
Persistent CS (1 case)
C Cure of CS (6 cases)
·   Normalization of ACTH, serum cortisol, circadian cortisol rhythm and UFC
·   Improvement of BP, glycemic control and BMI

20. Unilateral adrenalectomy for AIMAH
CONCLUSIONS (2)
Unilateral adrenalectomy for AIMAH
Cure of hypercortisolism
BP improvement
BMI decrease
Glycemic control improvement
Quality of life improvement