title: Ectopic adrenocorticotropic hormone-syndrome in medullary carcinoma of the thyroid: a retrospective analysis and review of the literature.
abstract: Cushing's syndrome (CS) in medullary thyroid carcinoma (MTC) is rare. Only 50 cases have been reported. We report 10 cases of MTC with ectopic adrenocorticotropic hormone (ACTH)-dependent syndrome (EAS), analyzed retrospectively. Among 1640 patients with MTC, 13 developed EAS (0.7%). In 10 patients CS could unequivoqually be related to MTC (0.6%). CS was always clinically obvious. It revealed MTC in 3 cases and followed diagnosis by an average of 34.5 months in the others. Metastases were often present at diagnosis. Immunohistochemistry with ACTH antibodies was positive in one case. Diagnosis of ectopic CS was established according to clinical and biologic features, and absence of corticotropic adenoma as well as parallel evolution between tumor and CS. Therapy was medical and surgical: anticortisolic drugs alone or in association with somatostatin analogue, somatostatin analogue alone, and bilateral adrenalectomy. Eight patients died within 2 to 30 months, 4 of hypercortisolism complications (3 peritonitis and 1 hypokalaemia), 4 of MTC progression. EAS is a rare complication of MTC. The prognosis is poor because of frequency of metastasis at diagnosis. Persistent hypercortisolism can, by itself, lead to death, and has to be treated specifically.
authors: Endocrinologie, Médecine C, Centre Hospitalier Universitaire, Angers, France.
authors: Sandrine Laboureau-Soares
authors: SL
authors: Barbosa
authors: Patrice
authors: P
authors: Rodien
authors: Sophie
authors: S
authors: Leboulleux
authors: Patricia
authors: P
authors: Niccoli-Sire
authors: Jean-Louis
authors: JL
authors: Kraimps
authors: Philippe
authors: P
authors: Caron
authors: Françoise
authors: F
authors: Archambeaud-Mouveroux
authors: Bernard
authors: B
authors: Conte-Devolx
authors: Vincent
authors: V
authors: Rohmer
mesh_terms: D000324:Adrenocorticotropic Hormone / Q000378:metabolism*; D000328:Adult; D000368:Aged; D018276:Carcinoma, Medullary / Q000473:pathology* / Q000503:physiopathology; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009384:Paraneoplastic Endocrine Syndromes / Q000473:pathology* / Q000503:physiopathology; D011379:Prognosis; D012189:Retrospective Studies; D013964:Thyroid Neoplasms / Q000473:pathology* / Q000503:physiopathology
chemical_list: D000324:Adrenocorticotropic Hormone