1. Introduction

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Adrenal adenomas are responsibility of approximately 10-15% of cases of Cushing’s syndrome. It can be either ACTH-dependent (pituitary adenoma or ACTH independent (extra pituitary adenoma). Multidisciplinary approach: endocrine manipulation, surgery, specific anesthetic procedure is needed in management of this case.

2. Case Report

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A 20-year-old man was admitted for evaluation of Cushing’s syndrome. He presented history of headache, fatique, mood disorder, hypertension (Blood Pressure 170/120 mmHg), moon face, buffalo hump, striae rubrae. Cortisol serum laboratory increased 33,53 µgr/dl (Normal range: 3,09 – 16,6µgr/dl). Abdominal CT Scan showed a right adrenal mass diameter 10,53 x 6,83 cm, heterogeneous pattern with calcified and necrotized area.

Level of ACTH < 5 pg/ml (Normal range: 6 – 50 pg/ml), absence hypothalamus pituitary defect in brain MRI angiography led to the primary site on adrenal (figure 1, bottom-left).

Figure 1

2.1. Treatment
Manipulation with ketoconazole 600 mg daily to treat hypercortisolemia. The patient underwent laparoscopic right adrenalectomy. Preparation of hydrocortisone 100 mg during anesthesia-surgery to prevent occurrence adrenal crisis.

Patient position LLD, 11mm trocar port with 0, 30-degree optic, 2 port 5mm for working element. Harmonic ultrasoundshear used to dissection, hemoLock clip to control vascular. Right subcostal incision makes to remove adrenal gland. EBL 1000 cc, close monitoring in ICU ward. 5

Hydrocortison continued 5 days after surgery. Ventilatory support remove in 2 day after surgery. Fifth day condition stabil without sign of adrenal crisis, patient send to elective ward. Gross specimen appear giant adrenal, 15 x 10 cm (figure1,right). Cortex area has a solid elasticity consistency. Some areas of necrosis are suspected of malignancy. But systematic microscopic investigations shown no visible mitosis or malignant signs (figure 3 A)

Figure 2    Figure 3

2.2. Outcome and Follow-up
On the fifth day after surgical intervention, postoperative cortisol level at 12 µgr/dl. Seventh day, surgical wound healing well with minimum dose NSAID orally. Striae thining, gynecomastia, buffalo hump, moon face reduced.

The patient was regularly followed up at Endocrine division, Internal medicine department. Moon face have been eliminated, no striae and good mood condition. Blood pressure was 130/ 70 mmHg (without antihypertensive drugs) and cortisol serum was 4,52 µgr/dL and independent from steroid medication.

Figure 4

3. Discussion

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We manipulated with ketoconazole by considering the hypothalamic adrenal pituitary axis to overcome due to excess cortisol. Endocrinologist can make a normal cortisol titer, and laparoscopic surgery can be performed. Another problem is that both clinical and heterogeneous physical imaging and large > 8 cm should be suspected and managed as a case of malignancy. Ibrahim, Agrusa also Zografos reported that laparoscopic adrenalectomy for giant size adrenal to be feasible and safe so we did this procedure.

Gross specimens also show heterogeneous features, there is a solid chewy part and calcification microscopic histopathology examination does not show specific malignancy signs. Also the area of necrosis that was originally thought to be strong focus malignancy we examined carefully only showed a dead cell collection.

Post-operative Cushing's symptom disappears, normotension without antihypertension, weakness, bad emotion, thinning striae. Buffalo hump, obesity, gynaecomasti in next follow-up seems to be reduced. This convinced us that these patients were purely functional benign lessions of the giant adrenal.

4. Conclusion

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Multidisciplinary approach including endocrine treatment, prevention adrenal crisis and laparoscopic adrenalectomy procedure have good result for Cushing’s syndrome due to adenoma of giant adrenal gland.

References

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