J Endocrinol Metab

Journal of Endocrinology and Metabolism, ISSN 1923-2861 print, 1923-287X online, Open Access

Article copyright, the authors; Journal compilation copyright, J Endocrinol Metab and Elmer Press Inc

Journal website http://www.jofem.org

Case Report

Volume 3, Number 1-2, April 2013, pages 29-37

Pitfalls in the Diagnosis of Ectopic Adrenocorticotropic Hormone Syndrome: A Case Report

Figure 1. Octreoscan showing a single nodular lesion in the inferior third of the left hemithorax medial surface.

Figure 2. Thoracic MRI showing a 1.8 cm of diameter, nodular lesion in the inferior left lobe of the pulmonary parenchyma.

**Table 1A.** Laboratory Testing on Admission
Variable	Reference Range, Adults	Basal values
Hemogram
Hemoglobin (g/dL)	13.0 - 17.5	13.8
Hematocrit (%)	40.0 - 50.0	39.4
Mean Cell Volume (fL)	80.0 - 97.0	93.1
White-cell count (per µL)	4,000 - 11,000	8,900
Neutrophils (%)	57 - 67	90
Lymphocytes (%)	25 - 33	6.5
Eosinophils (%)	1-3	0.1
Platelet count (per µL)	150,000 - 450,000	119,000
Biochemistry
Glucose (mg/dL)	70 - 110	288
Urea nitrogen (mg/dL)	10 - 50	28
Creatinine (mg/dL)	0.7 - 1.2	0.6
Sodium (mmol/L)	135-145	150
Potassium (mmol/L)	3.5 - 5.1	2.0
Chloride (mmol/L)	98 - 107	96
Urate (mg/dL)	200 - 420	136
Aspartate Transaminase (U/L)	0 - 37	24
Alanine Aminotransferase (U/L)	10 - 49	46
Gamma-glutamyl transferase (U/L)	< 73	21
Alkaline Phosphatase (U/L)	45 - 129	264
Bilirubin (mg/dL)	0.1 - 1.1	0.9
Proteins (g/L)	66 - 87	63
Albumin (g/L)	34 - 48	42
Lactate dehydrogenase (U/L)	208 - 378	1,009
Creatine Kinase (U/L)	32 - 294	55
Calcium (mg/dL)	8.6 - 10.2	8.3
Phosphorus (mg/dL)	2.7 - 4.5	1.5
Magnesium (mg/dL)	1.6 - 2.6	2.1
Triglycerides (mg/dL)	< 200	164
Total Cholesterol (mg/dL)	< 200	154
HDL (mg/dL)	< 35 (high risk)	44
C-reactive protein (mg/dL)	< 0.5	0.05

Table 1A. Laboratory Testing on Admission

Variable

Reference Range, Adults

Basal values

Hemogram

Hemoglobin (g/dL)

13.0 - 17.5

13.8

Hematocrit (%)

40.0 - 50.0

39.4

Mean Cell Volume (fL)

80.0 - 97.0

93.1

White-cell count (per µL)

4,000 - 11,000

8,900

Neutrophils (%)

57 - 67

Lymphocytes (%)

25 - 33

6.5

Eosinophils (%)

1-3

0.1

Platelet count (per µL)

150,000 - 450,000

119,000

Biochemistry

Glucose (mg/dL)

70 - 110

288

Urea nitrogen (mg/dL)

10 - 50

Creatinine (mg/dL)

0.7 - 1.2

0.6

Sodium (mmol/L)

135-145

150

Potassium (mmol/L)

3.5 - 5.1

2.0

Chloride (mmol/L)

98 - 107

Urate (mg/dL)

200 - 420

136

Aspartate Transaminase (U/L)

0 - 37

Alanine Aminotransferase (U/L)

10 - 49

Gamma-glutamyl transferase (U/L)

< 73

Alkaline Phosphatase (U/L)

45 - 129

264

Bilirubin (mg/dL)

0.1 - 1.1

0.9

Proteins (g/L)

66 - 87

Albumin (g/L)

34 - 48

Lactate dehydrogenase (U/L)

208 - 378

1,009

Creatine Kinase (U/L)

32 - 294

Calcium (mg/dL)

8.6 - 10.2

8.3

Phosphorus (mg/dL)

2.7 - 4.5

1.5

Magnesium (mg/dL)

1.6 - 2.6

2.1

Triglycerides (mg/dL)

< 200

164

Total Cholesterol (mg/dL)

< 200

154

HDL (mg/dL)

< 35 (high risk)

C-reactive protein (mg/dL)

< 0.5

0.05

**Table 1B.** Laboratory Testing on Admission
Variable	Reference Range, Adults	Basal Values
Endocrinology
Insulin (mU/L)	3 - 25	26.4
C peptide (ng/mL)	0.8 - 3.9	6.16
Glycated haemoglobin (%)	< 6.0%	6.3
Parathyroid Hormone (pg/mL)	14 - 72	40.2
Adrenocorticotropic Hormone (pg/mL)	0 - 46	212.0
Cortisol (µg/dL)	4.3 - 23 (8 a.m)	77.9
Hydroxyprogesterone (ng/mL)	0.3 - 2.0	4.30
Dehydroepiandrosteronesulfate (µg/dL)	80 - 560	375.0
Delta 4-androstenedione (ng/mL)	0.6 - 3.1	16.90
11 Desoxycortisol - Compound S (ng/mL)	Baseline: < 7.2	40.30
Aldosterone (pg/mL)	Standing: 40 - 310 Lying: 10 - 160	88.4
Renin (pg/mL)	Standing: 2 - 50 Lying: 1.6 - 15	2.2
Thyroxin (µg/dL)	4.5 - 10.9	6.6
Thyroid Stimulating Hormone (µU/mL)	0.35 - 5.50	0.70
Free Thyroxin (ng/dL)	0.89 - 1.80	1.20
Triiodothyronine (ng/mL)	0.60 - 1.81	0.68
Follicle-stimulating hormone (U/L)	> 16 years: 1.4 - 18.1	0.94
Luteinizing Hormone (U/L)	1.5 - 9.3	2.37
Prolactin (ng/mL)	2.1 - 17.7	12.8
Estradiol (pg/mL)	Man: < 52	< 10.0
Total Testosterone (ng/dL)	241 - 827	198.0
Free Testosterone (pg/mL)	8.8 - 27	15.30
Free Androgen Index	14.8 - 94.8	53.8
Sex-hormone Binding Globulin (nmol/L)	13 - 71	14.7
Growth Hormone (ng/mL)	0 - 1.0	0.09
Insulin-like Growth Factor 1 (ng/mL)	117 - 329	118.0
Tumoral markers
CEA (ng/mL)	< 5.0	17.6
CA 15.3 (U/mL)	< 32	43.4
Chromogranin A (µg/L)	< 100	1473
Glycoprotein Free alpha-subunit (µg/L)	< 0.8	0.2
Calcitonin (pg/mL)	< 8.4	< 5.00
Human chorionic gonadotropin (ng/mL)	0 - 2.5	< 0.1
Somatostatin (µg/L)	< 5.6	1.4
Serotonin (µg/L)	117.5 - 193.3	186
Urinary 5-Hydroxyindoleacetic acid (mg/24h)	0.7 - 8.2	12.8

Table 1B. Laboratory Testing on Admission

Variable

Reference Range, Adults

Basal Values

Endocrinology

Insulin (mU/L)

3 - 25

26.4

C peptide (ng/mL)

0.8 - 3.9

6.16

Glycated haemoglobin (%)

< 6.0%

6.3

Parathyroid Hormone (pg/mL)

14 - 72

40.2

Adrenocorticotropic Hormone (pg/mL)

0 - 46

212.0

Cortisol (µg/dL)

4.3 - 23 (8 a.m)

77.9

Hydroxyprogesterone (ng/mL)

0.3 - 2.0

4.30

Dehydroepiandrosteronesulfate (µg/dL)

80 - 560

375.0

Delta 4-androstenedione (ng/mL)

0.6 - 3.1

16.90

11 Desoxycortisol - Compound S (ng/mL)

Baseline: < 7.2

40.30

Aldosterone (pg/mL)

Standing: 40 - 310
Lying: 10 - 160

88.4

Renin (pg/mL)

Standing: 2 - 50
Lying: 1.6 - 15

2.2

Thyroxin (µg/dL)

4.5 - 10.9

6.6

Thyroid Stimulating Hormone (µU/mL)

0.35 - 5.50

0.70

Free Thyroxin (ng/dL)

0.89 - 1.80

1.20

Triiodothyronine (ng/mL)

0.60 - 1.81

0.68

Follicle-stimulating hormone (U/L)

> 16 years: 1.4 - 18.1

0.94

Luteinizing Hormone (U/L)

1.5 - 9.3

2.37

Prolactin (ng/mL)

2.1 - 17.7

12.8

Estradiol (pg/mL)

Man: < 52

< 10.0

Total Testosterone (ng/dL)

241 - 827

198.0

Free Testosterone (pg/mL)

8.8 - 27

15.30

Free Androgen Index

14.8 - 94.8

53.8

Sex-hormone Binding Globulin (nmol/L)

13 - 71

14.7

Growth Hormone (ng/mL)

0 - 1.0

0.09

Insulin-like Growth Factor 1 (ng/mL)

117 - 329

118.0

Tumoral markers

CEA (ng/mL)

< 5.0

17.6

CA 15.3 (U/mL)

< 32

43.4

Chromogranin A (µg/L)

< 100

1473

Glycoprotein Free alpha-subunit (µg/L)

< 0.8

0.2

Calcitonin (pg/mL)

< 8.4

< 5.00

Human chorionic gonadotropin (ng/mL)

0 - 2.5

< 0.1

Somatostatin (µg/L)

< 5.6

1.4

Serotonin (µg/L)

117.5 - 193.3

186

Urinary 5-Hydroxyindoleacetic acid (mg/24h)

0.7 - 8.2

12.8

**Table 2.** Low and High-Dose Dexamethasone Suppression Tests; CRH Stimulation Test
	Reference Range, Adults	Basal Values	Post- Low-Dose Dexamethasone Suppression Test	Post- High-Dose Dexamethasone Suppression Test/Pre-CRH	Post-CRH 15’
ACTH (pg/mL)	0 - 46	197		115	112
Cortisol (µg/dL)	4.3 - 23 a.m.	110	107	52	59
Urinary Cortisol (µg/24h)/Urinary Creatinine (g/24h)	28.5 - 214/ 0.8 - 2.0	771	1,022	1,200

Table 2. Low and High-Dose Dexamethasone Suppression Tests; CRH Stimulation Test

Reference Range, Adults

Basal Values

Post- Low-Dose Dexamethasone
Suppression Test

Post- High-Dose Dexamethasone Suppression Test/Pre-CRH

Post-CRH
15’

ACTH (pg/mL)

0 - 46

197

115

112

Cortisol (µg/dL)

4.3 - 23 a.m.

110

107

Urinary Cortisol (µg/24h)/Urinary Creatinine (g/24h)

28.5 - 214/ 0.8 - 2.0

771

1,022

1,200

**Table 3.** Inferior Petrosal Sinus Sampling With 1 µg/kg of Endovenous CRH at 0’
	Inferior Petrosal Sinus Sampling with 1µg/kg endovenous CRH at 0’
ACTH 0’	136	140	126
ACTH 15’	143	159	128

Table 3. Inferior Petrosal Sinus Sampling With 1 µg/kg of Endovenous CRH at 0’

Inferior Petrosal Sinus Sampling with 1µg/kg endovenous CRH at 0’

Left Inferior Petrosal Sinus

Right Inferior Petrosal Sinus

Peripheral blood

ACTH 0’

136

140

126

ACTH 15’

143

159

128