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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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the role of unilateral adrenalectomy in acth independent macronodular adrenal hyperplasia aimah

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

cushing s syndrome
ACTH dependent (80%)

Pituitary

Ectopic

ACTH independent (20%)

Unilateral

(Adenoma, Carcinoma)

Bilateral

- PPNAD

- ACTH-Independent Macronodular Adrenal Hyperplasia (AIMAH)

Cushing’s Syndrome
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)

aimah therapy
AIMAH - Therapy
  • Lifetime steroid

replacement

  • Bilateral Adrenalectomy
  • Octreotide
  • Propanolol
  • Leuprolide
  • Medical Treatment

Ectopic Receptors

  • Subtotal
  • Unilateral
  • Partial adrenalectomies
aim of the study
AIM of the STUDY
  • Long-term results

of Unilateral Adrenalectomy for AIMAH

patients and methods 1
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

patients et methods 2
PATIENTS ET METHODS (2)

CT/MRI + I-131 norcholesterol Scintigraphy

Asymmetric involvement

Unilateral adrenalectomy

patients et methods 3
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
results 1
RESULTS (1)
  • Morbidity: 0%
  • CURE: 86%
  • Persistent hypercortisolism:1 case
  • Completion contralateral adrenalectomy
  • Large contralateral remaining adrenal
  • Symmetric uptake at scintigraphy
results 2
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
results 21
RESULTS (2)

Follow-up: 53 months (range 27-68)

CURE: 6 PATIENTS

  • “Small” contralateral remaining adrenal
  • Asymmetric uptake at scintigraphy
results 3
RESULTS (3)

HORMONAL PARAMETERS

ACTH

UFC

results 4
RESULTS (4)

BLOOD PRESSURE

HYPERTENSION

RECOVERY: 50%

DRUG REDUCTION: 50%

results 5
RESULTS (5)

GLUCOSE METABOLISM

DIABETES

RECOVERY: 40%

DRUG REDUCTION: 40%

results 6
RESULTS (6)

BODY MASS INDEX

results 7
RESULTS (7)

SF-36 QUALITY OF LIFE

*

*

* p<0,01

Mental Component

Summary

Physical ComponentSummary

conclusions 1 aimah
CONCLUSIONS (1)AIMAH

Asymmetric adrenal involvement

“Small” remaining gland

(case selection)

Unilateral adrenalectomy

EFFECTIVE TREATMENT

slide19

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)

slide20

CONCLUSIONS (2)

Unilateral adrenalectomy for AIMAH

  • Cure of hypercortisolism
  • BP improvement
  • BMI decrease
  • Glycemic control improvement
  • Quality of life improvement