Pheochromocytoma and sub-clinical Cushing’s syndrome during pregnancy: Diagnosis, medical pre-treatment and cure by laparoscopic unilateral adrenalectomy

Abstract

The coexistence of pheochromocytoma and primary adrenal Cushing's syndrome of the same adrenal gland has rarely been reported. We describe here the case of a female patient presenting with mild Cushing's stigmata, hypertension and diabetes mellitus in whom we diagnosed a pheochromocytoma of the left adrenal gland with coexisting non-ACTH-dependent cortisol hypersecretion. While hormonal work-up was still in progress, the patient became pregnant and wanted to carry her pregnancy to full-term. A laparoscopic adrenalectomy in the 17th week of gestation was decided upon and the patient accordingly prepared for surgery by pre-treatment with phenoxybenzamine. Successful surgery--the first ever reported laparoscopic resection of a pheochromocytoma in pregnancy--without perioperative complications was performed under general anesthesia, with the patient receiving peri- and post-operative hydrocortisone substitution. Pathohistological examination revealed a pheochromocytoma with positive immunostaining for interleukin-6 (IL-6) and negative immunostaining for ACTH, vasoactive intestinal polypeptide (VIP) and cytochrome P450, and with no signs of malignancy. A paracrine stimulation of the ipsilateral adrenal cortex by IL-6 produced by the pheochromocytoma, leading to cortical hyperplasia and subclinical Cushing's syndrome, is suggested by the positive immunostaining for IL-6 and the MRI findings. Post-operatively, secondary adrenal insufficiency ensued, necessitating continuing hydrocortisone replacement over 12 months. Hypertension resolved after surgery, and diabetes after the uncomplicated vaginal delivery at term.

A Case of ACTH-Producing Pheochromocytoma Associated with Pregnancy

“…Considering the fact that the pregnancy in this case was possible and maintained without any fetal problem until early third trimester, we can postulate the cortisolemic effect might have been minimal at the time of conception. To the best of our knowledge, only one case with pheochromocytoma and subclinical Cushing's syndrome during pregnancy has ever been reported [9]. However, the tumor was negative for ACTH immunostaining, and thus the increased level of interleukin-6 produced by the tumor was suggested as the mechanism of increased cortisol production in that case.…”

Guidelines for diagnosis, treatment, and use of laparoscopy for surgical problems during pregnancy

“…Laparoscopic adrenalectomy during pregnancy has proven effective in the management of primary hyperaldosteronism [129], Cushing's syndrome [130][131][132], and pheochromocytoma [133][134][135][136][137][138]. Laparoscopic splenectomy has also become an increasingly accepted surgical approach in pregnancy [139].…”

Diagnosis and laparoscopic treatment of surgical diseases during pregnancy: an evidence-based review

“…After successful laparoscopic management of appendicitis and gallbladder disease in pregnancy, surgeons began exploring laparoscopy in the management of other surgical diseases that arise in pregnancy. Laparoscopic adrenalectomy during pregnancy has proven effective in the management of primary hyperaldosteronism [122], Cushing's syndrome [123][124][125], and pheochromocytoma [126][127][128][129][130][131]. Laparoscopic splenectomy has also become an increasingly accepted surgical approach in pregnancy [132].…”