title: A case of ACTH-producing pheochromocytoma associated with pregnancy.
abstract: Ectopic ACTH syndrome is rarely caused by pheochromocytoma. We report a case of a 28-year-old woman with Cushing's syndrome due to ACTH-producing adrenal pheochromocytoma. She had delivered preterm baby at 32nd week of gestation with 'severe preeclampsia'. After delivery, persistent hypertension accompanied by severe headache led her to being misdiagnosed as Cushing's syndrome due to right adrenal adenoma (normal plasma ACTH level) and cerebral vasculitis of unknown etiology. She was referred to our hospital for surgical treatment. Repeated biochemical studies suggested coexistence of ectopic ACTH syndrome and pheochromocytoma. To reverse her clinical deterioration, right total and left subtotal adrenalectomy was performed with presumptive diagnosis of 1) right adrenal pheochromocytoma causing ectopic ACTH syndrome or 2) coexistence of ACTH-dependent Cushing's syndrome and right adrenal pheochromocytoma. Pathologic examination of right adrenal mass revealed pheochromocytoma which showed strong immunostaining for ACTH. Plasma ACTH and urinary cortisol excretion normalized after surgery, but she succumbed to multiple cerebral infarcts and disseminated intravascular coagulation. Pregnancy and inappropriately low plasma ACTH at initial evaluation might have hampered early diagnosis. To our knowledge, this is the first description of a case with ectopic ACTH syndrome due to pheochromocytoma associated with pregnancy.
authors.affiliation: Division of Endocrinology and Metabolism, Department of Internal Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
authors.forename: Hyoung Chul
authors.initials: HC
authors.lastname: Oh
authors.forename: Jung-Min
authors.initials: JM
authors.lastname: Koh
authors.forename: Min Seon
authors.initials: MS
authors.lastname: Kim
authors.forename: Joong Yoel
authors.initials: JY
authors.lastname: Park
authors.forename: Young Kee
authors.initials: YK
authors.lastname: Shong
authors.forename: Ki-Up
authors.initials: KU
authors.lastname: Lee
authors.forename: Ghi Su
authors.initials: GS
authors.lastname: Kim
authors.forename: Suck Joon
authors.initials: SJ
authors.lastname: Hong
authors.forename: Hyun Lyoung
authors.initials: HL
authors.lastname: Koo
authors.forename: Won Bae
authors.initials: WB
authors.lastname: Kim
mesh_terms: D000182:ACTH Syndrome, Ectopic; D000310:Adrenal Gland Neoplasms / Q000150:complications / Q000000981:diagnostic imaging / Q000378:metabolism* / Q000473:pathology; D000328:Adult; D003480:Cushing Syndrome / Q000209:etiology*; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D010673:Pheochromocytoma / Q000150:complications / Q000000981:diagnostic imaging / Q000378:metabolism* / Q000473:pathology; D011247:Pregnancy; D011252:Pregnancy Complications, Neoplastic / Q000000981:diagnostic imaging / Q000378:metabolism* / Q000473:pathology; D014057:Tomography, X-Ray Computed