Pheochromocytoma and sub-clinical Cushing's syndrome during pregnancy: Diagnosis, medical pre-treatment and cure by laparoscopic unilateral adrenalectomy
G. Finkenstedt et al., Pheochromocytoma and sub-clinical Cushing's syndrome during pregnancy: Diagnosis, medical pre-treatment and cure by laparoscopic unilateral adrenalectomy, J ENDOC INV, 22(7), 1999, pp. 551-557
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.