異位ACTH症候群患者接受腹腔鏡雙側腎上腺切除手術 － 案例報告

林巧文¹、鍾孝仁¹²³ 、林亮羽⁴⁵、呂仕彥⁶ 、黃志賢¹²³

¹臺北榮民總醫院 泌尿部; ²國立陽明大學醫學院泌尿學科; ³書田泌尿科學研究中心;
⁴臺北榮民總醫院 內分泌新陳代謝科; ⁵國立陽明大學 醫學系 內科學科;

⁶台北榮民總醫院新竹分院外科部

Chyau-Wen Lin¹、Hsiao-Jen Chung¹²³、Liang-Yu Lin⁴⁵、Shih-Yen Lu⁶、William Ji-Shien Huang¹²³

¹Department of Urology, Taipei Veterans General Hospital;

²Department of Urology, School of Medicine, National Yang-Ming University, Taipei, Taiwan;

³Shu-Tien Urological Institute, National Yang-Ming University, Taipei, Taiwan;

⁴Department of Endocrinology, Taipei Veterans General Hospital;

⁵Department of internal Medicine, School of Medicine, National Yang-Ming University, Taipei, Taiwan;

⁶Department of Surgery, Taipei Veterans General Hospital Hsinchu branch, Hsinchu, Taiwan

 

Ectopic ACTH syndrome is a minor cause of ACTH-dependent hypercortisolism, and bilateral adrenalectomy is an acceptable mean for treatment of hypercortisolism symptoms. Here we presented a case of ectopic ACTH syndrome who received bilateral laparoscopic adrenalectomy.

A 69-year-old male had obesity, hypertension and diabetes. His initial presentation was bilateral pitting lower leg edema with pain and erythema along with increasing speech amount, bizarre thinking and self-talking in 2018. Comprehensive work-up was done, and ACTH dependent Cushing syndrome was diagnosed. However, brain MRI revealed no pituitary mass. Biopsy to left lower lung lesion and nodule at gastro-splenic fossa mass both revealed no definite source of ACTH.

Due to deterioration of psychiatric symptoms and shortage of medication, urologist was consulted for surgical intervention.

Oral Metyrapone 1# QID and Fluconazole 1# BID were given for one week, and intravenous infusion of Etomidate was given one day prior to the surgery. Bilateral laparoscopic adrenalectomy was performed.

The surgery was performed under general anesthesia with arterial canulation and central venous catheter. The patient was placed on left lateral decubitus position, and the primary 12-mm port at right midclavicular line 2 cm under the 10th rib with Veress needle. The subsequent three trocars were placed at near subxiphoid area, right anterior axillary line under the rib and over right posterior axillary line respectively. Right adrenalectomy was performed, and the gland was retrieved with tissue retrieval bag. The trocar wounds were closed layer by layer, and the patient was re-positioned in right lateral decubitus. The left adrenalectomy was performed in the similar fashion, and two 15Fr JP drains were placed at bilateral supra-renal fossa. Total operative duration was 8 hours and 50 minutes, and the blood loss was 1000 ml. The patient didn’t receive any blood transfusion during the peri-operative period, and extubation was performed in the operative room right after the surgery ended.

Cortisol replacement was given since the post-operative day (POD) 1, and oral diet resumed on POD 2.

The pathology revealed diffuse hyperplasia with no malignancy. Hypertension and hyperglycemia greatly improved after the surgery, while psychiatric symptoms remain stationary. No electrolyte imbalance that requiring parenteral supplement was detected during admission, and there were no symptoms of adrenal insufficiency. The patient was discharged on POD 11 with oral prednisolone.

Ectopic ACTH syndrome is a minor cause of ACTH-dependent hypercortisolism, which was responsible for 12-17% of Cushing syndrome. Ectopic ACTH syndrome is a common paraneoplastic syndrome, and it is often associated with lung or bronchial tumors. Other origins of ectopic ACTH had been mentioned in literature include pancreatic tumor, pheochromocytomas, medullary thyroid carcinoma, thymus tumor, and ovarian adenocarcinomas. Minor portion of the source of ectopic ACTH remains occult after comprehensive work-up. In those cases, medical adrenolytic treatments should be considered first, which include metyrapone, ketoconazole, mitotane and etomidate. Bilateral adrenalectomy may be considered when medical treatment is not effective or intolerable. Life-long steroid replacement should be given.