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

The coexistence of pheochromocytoma and primary adrenal Gushing's syndrome of the same adrenal gland has rarely been reported. We describe here the ca se of a female patient presenting with mild Gushing's stigmata, hypertensio n 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 adrenalectom y in the 17th week of gestation was decided upon and the patient accordingl y prepared for surgery by pre-treatment with phenoxybenzamine. Successful s urgery - the first ever reported laparoscopic resection of a pheochromocyto ma in pregnancy - without perioperative complications was performed under g eneral anesthesia, with the patient receiving peri- and post-operative hydr ocortisone substitution. Pathohistological examination revealed a pheochrom ocytoma with positive immunostaining for interleukin-6 (IL-6) and negative immunostaining for ACTH, vasoactive intestinal polypeptide (VIP) and cytoch rome P450, and with no signs of malignancy. A paracrine stimulation of the ipsilateral adrenal cortex by IL-6 produced by the pheochromocytoma, leadin g to cortical hyperplasia and subclinical Gushing's syndrome, is suggested by the positive immunostaining for IL-6 and the MRI findings. Post-operativ ely, secondary adrenal insufficiency ensued, necessitating continuing hydro cortisone replacement over 12 months. Hypertension resolved after surgery, and diabetes after the uncomplicated vaginal delivery at term. (C) 1999, Ed itrice Kurtis.