title: Aberrant hormone production from ovarian neoplasms: strategies for diagnosis and therapy.
abstract: Syndromes involving peptide or nonsex steroid hormone secretion due to aberrantly located tumors are rare. We report a collected series of 16 patients with ectopic hormone production from ovarian neoplasms, including 3 patients recently encountered at our institution as well as 13 additional cases identified in the recent literature. These tumors included 2 insulin-producing ovarian carcinoids, 1 ACTH-producing pituitary adenoma within a benign ovarian cystic teratoma, 2 cortisol-producing ovarian neoplasms, 8 gastrin-producing ovarian cystadenomata or cystadenocarcinomata, and 3 thyroxine-producing ovarian strumal carcinoids. All patients presented with syndromes of hormone excess. Only 62% of all tumors were localized preoperatively. Following ovarian resection, 87% of patients remained disease-free with a median follow-up period of 1.5 years. In addition to ovariectomy, 8 additional unnecessary ablative procedures were performed in 7 patients. These included distal pancreatectomy, pancreaticoduodenectomy, adrenalectomy, total gastrectomy, selective vagotomy, and subtotal thyroidectomy. Failure to localize the ovarian neoplasm preoperatively was associated with a significantly higher risk of subsequent unnecessary ablative procedures. Because of the potential for the ovary to act as a source of aberrant hormone secretion, we recommend complete preoperative evaluation of the pelvis in female patients presenting with nonlocalizable endocrine tumors.
authors.affiliation: Department of Surgery, Yale University School of Medicine, New Haven, Connecticut 06510.
authors.forename: S D
authors.initials: SD
authors.lastname: Leach
authors.forename: A I
authors.initials: AI
authors.lastname: LaMorte
authors.forename: L D
authors.initials: LD
authors.lastname: True
authors.forename: S D
authors.initials: SD
authors.lastname: Flynn
authors.forename: P E
authors.initials: PE
authors.lastname: Schwartz
authors.forename: C E
authors.initials: CE
authors.lastname: Cahow
authors.forename: B K
authors.initials: BK
authors.lastname: Kinder
mesh_terms: D000324:Adrenocorticotropic Hormone / Q000378:metabolism; D000328:Adult; D005260:Female; D005755:Gastrins / Q000378:metabolism; D006729:Hormones, Ectopic / Q000378:metabolism*; D006801:Humans; D006854:Hydrocortisone / Q000378:metabolism; D007328:Insulin / Q000378:metabolism; D000078790:Insulin Secretion; D010051:Ovarian Neoplasms / Q000378:metabolism*; D009384:Paraneoplastic Endocrine Syndromes / Q000175:diagnosis*; D012189:Retrospective Studies; D013974:Thyroxine / Q000378:metabolism
chemical_list: D005755:Gastrins; D006729:Hormones, Ectopic; D007328:Insulin; D000324:Adrenocorticotropic Hormone; D013974:Thyroxine; D006854:Hydrocortisone