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
- 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)
ACTH-Independent Macronodular Adrenal Hyperplasia (AIMAH) Slowly Progressing Disease Mild Hypercortisolism Bilateral Macronodules Ectopic Receptors (GIP, Catecholamines, LH/hCG)
Bilateral Adrenalectomy Partial adrenalectomies AIMAH - Therapy Lifetime steroid replacement Bilateral Adrenalectomy Octreotide Propanolol Leuprolide Medical Treatment Ectopic Receptors Subtotal Unilateral Partial adrenalectomies
of Unilateral Adrenalectomy for AIMAH AIM of the STUDY Long-term results of Unilateral Adrenalectomy for AIMAH
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
CT/MRI + I-131 norcholesterol Scintigraphy PATIENTS ET METHODS (2) CT/MRI + I-131 norcholesterol Scintigraphy Asymmetric involvement Unilateral adrenalectomy
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
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
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
CURE: 6 PATIENTS RESULTS (2) Follow-up: 53 months (range 27-68) “Small” contralateral remaining adrenal Asymmetric uptake at scintigraphy
HORMONAL PARAMETERS RESULTS (3) ACTH UFC 0,006 0,04 ACTH PRE-OP POST-OP p ACTH (nv 10-50 ng/L) 7 + 2 28 + 11 0,006 Urinary Free Cortisol (nv 28-214 nmol/day) 1206 + 1038 59 + 16 0,04 ACTH UFC
HYPERTENSION RESULTS (4) 0,006 0,001 RECOVERY: 50% DRUG REDUCTION: 50% BLOOD PRESSURE PRE-OP POST-OP p Systolic BP (mmHg) 152 + 15 130 + 8 0,006 Diastolic BP (mmHg) 92 + 5 79 + 4 0,001 HYPERTENSION RECOVERY: 50% DRUG REDUCTION: 50%
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) 8.6 + 2 6.7 + 1 0,005 HbA1c (nv 3,7–6,1%) 7.4 + 1% 5.8 + 1% 0,002 DIABETES RECOVERY: 40% DRUG REDUCTION: 40%
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
Physical Component Summary RESULTS (7) SF-36 QUALITY OF LIFE * * * p<0,01 Physical Component Summary Mental Component Summary
(case selection) Unilateral adrenalectomy EFFECTIVE TREATMENT CONCLUSIONS (1) AIMAH Asymmetric adrenal involvement “Small” remaining gland (case selection) Unilateral adrenalectomy EFFECTIVE TREATMENT
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 30-137) 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
Unilateral adrenalectomy for AIMAH CONCLUSIONS (2) Unilateral adrenalectomy for AIMAH Cure of hypercortisolism BP improvement BMI decrease Glycemic control improvement Quality of life improvement