Is Adrenal  Venous Sampling Enough for ACTH Independent Cushing’S  Syndrome in Patient with Bilateral Adrenal Mass?

Presentation Number: MON 413
Date of Presentation: April 3rd, 2017

Meral Mert*1, Hamide Piskinpasa2 and Pinar Karakaya3
1Bakirkoy Dr. Sadi Konuk Training and Research Hospital, Istanbul, Turkey, 2Genx Pharmaceuticals, 3Bakirkoy Dr. Sadi Konuk Training and Research Hospital, Istanbul

Abstract

Background: Bilateral functioning adenomas are exceedingly rare and diagnosis is very challenging and there is no way to decide the best therapeutic approach. We aimed to present bilateral adrenal functioning mass which underwent adrenal venous sampling.

Case Report: 43 years old woman with a history of hypertension and hypothyroidism applied to our hospital with a weight gain of 40 kg and abdominal strias over abdomen. she had buffalo hump, abdominal obesity, and purple strias on her physical examination.

Her laboratory findings showed an elevated basal cortisol levels was 22 µg/dl and ACTH was less than 5 pg/ml The 24 - hour urine free cortisol excretion increased to 198 µg/day (normal range, 23 to 135) and low dose dexamethasone supression test (DST) with overnight DST was 20.9 µg/dl, 48 hour DST was performed after administration of dexamethasone at 0,5 mg every 6 hours for 48 hours was 16,4 µg/dl. The patient underwent tests to exclude adrenocorticotropin independent macronodular adrenal hyperplasia (AIMAH). The results were compatible with ACTH independent Cushing syndrome. The MRI of abdomen had documented the presence of two adrenal adenomas, which was 16*25 mm on the right side and 18*24 mm on the left side. There was no abnormal finding in the sella MRI. PET CT showed no malign FDG affinity in adrenal mass.

We used aldosterone levels to make the correction between both sides [2] Samples of both adrenal veins and inferior vena cava vein were taken. Successful catheterisation was confirmed on the basis of following aldosterone levels: right adrenal vein, 83,6 pg/ml and left adrenal vein 38,4 pg/ml. The cortisol levels are: right adrenal vein 354 µg/dl and left adrenal vein 277 µg/dl, inferior vena cava 22 µg/ dl . The adrenal vein to peripheral vein cortısol ratio (AV/PV gradient) was 16 on the right side and 12,5 on the left side, with a right-to-left lateralisation ratio 1,3 .

The test suggest that the patient has an adrenal hyperplasia with predominance in the production of cortisol from the right side. Right adrenal vein cortisol levels and diameter of adenom on abdomen MR were higher from left adrenal. So we decided to perform right adrenalectomy. The patient underwent laparoscopic right adrenalectomy.

The blood cortisol concentration was 20 µg/dl and 1 mg DST results was found 12 µg/dl in the 6th month follow-up period affter right adrenalectomy. Therefore we decided to perfom left adrenalectomy.

 

 

Nothing to Disclose: MM, HP, PK