title: 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.
authors.affiliation: Department of Internal Medicine, University of Innsbruck, Austria. Gerd.Finkenstedt@uibk.ac.at
authors.forename: G
authors.initials: G
authors.lastname: Finkenstedt
authors.forename: R W
authors.initials: RW
authors.lastname: Gasser
authors.forename: G
authors.initials: G
authors.lastname: Höfle
authors.forename: K
authors.initials: K
authors.lastname: Lhotta
authors.forename: D
authors.initials: D
authors.lastname: Kölle
authors.forename: A
authors.initials: A
authors.lastname: Gschwendtner
authors.forename: G
authors.initials: G
authors.lastname: Janetschek
mesh_terms: D000310:Adrenal Gland Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D000315:Adrenalectomy; D000328:Adult; D003480:Cushing Syndrome / Q000150:complications / Q000175:diagnosis*; D048909:Diabetes Complications; D005260:Female; D005865:Gestational Age; D006801:Humans; D006854:Hydrocortisone / Q000627:therapeutic use; D006973:Hypertension / Q000150:complications; D015850:Interleukin-6 / Q000032:analysis; D008279:Magnetic Resonance Imaging; D010673:Pheochromocytoma / Q000150:complications / Q000175:diagnosis* / Q000601:surgery; D011247:Pregnancy; D011252:Pregnancy Complications, Neoplastic / Q000175:diagnosis*
chemical_list: D015850:Interleukin-6; D006854:Hydrocortisone