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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 dependent (80%) Pituitary Ectopic. ACTH independent (20%)

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M Iacobone , G Viel, S Zanella, M Frego, G Favia

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

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

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

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

  8. 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

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

  10. 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

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

  12. RESULTS (3) HORMONAL PARAMETERS ACTH UFC

  13. RESULTS (4) BLOOD PRESSURE HYPERTENSION RECOVERY: 50% DRUG REDUCTION: 50%

  14. RESULTS (5) GLUCOSE METABOLISM DIABETES RECOVERY: 40% DRUG REDUCTION: 40%

  15. RESULTS (6) BODY MASS INDEX

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

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

  18. 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 Persistent CS (1 case) C Cure of CS (6 cases) ·Normalization of ACTH, serum cortisol, circadian cortisol rhythm and UFC ·Normal responsiveness to dexamethasone suppression test ·Improvement of BP, glycemic control and BMI ·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] 1 36 1 case (15 months) Cure of CS ·Normalization of UFC, serum cortisol ·Subnormal ACTH ·Abnormal circadian cortisol rhythm ·No enlargement of the remaining gland Sato [13] 1 8 1 case (8 months) Cure of CS ·Subnormal serum cortisol and UFC ·Subnormal ACTH Ogura [11] 1 24 - Cure of CS ·Normal ACTH, serum cortisol ·Normal circadian cortisol rhythm ·Subnormal UFC ·Unchanged BMI and BP ·Improvement of glycemic control ·No enlargement of the remaining gland Imohl [10] 1 27 1 case (6 months) Cure of CS ·Normalization of serum cortisol and circadian cortisol rhythm ·Abnormal responsiveness to dexamethasone suppression test ·No enlargement of the remaining gland N’Diaye [7] 1 12 1 cases (12 months) Cure of CS ·Subnormal, serum cortisol and UFC ·Subnormal ACTH ·Mild enlargement (3 mm) of the remaining gland Doppmann [8] 1 64 - Cure of CS Present series (WJS 2008) 7 53 (range 7-68) 2 cases (7 and 24 months)

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

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