title: Atypical thymic carcinoid associated with Cushing's syndrome.
abstract: A 56-year-old Japanese woman with adrenocorticotropic hormone (ACTH)-dependent Cushing's syndrome (CS) was admitted to hospital, where she was diagnosed as having a mediastinal tumor with ectopic ACTH production. The tumor and associated lymph node metastases were resected endoscopically, and the pathological diagnosis was atypical thymic carcinoid. Radiation therapy and administration of metyrapone, an inhibitor of 11b-hydroxylase to decrease the cortisol level, were attempted, but the levels of ACTH and cortisol were unresponsive. Bilateral adrenalectomy and hydrocortisone replacement were performed to ameliorate the patient's hypercortisolism. She subsequently developed multiple vertebral metastases, but was unwilling to undergo chemotherapy. Her condition deteriorated progressively, and she died of heart and respiratory failure 3 years and 6 months after the first admission. Immunostaining for ACTH, chromogranin A, synaptophysin, and neuron-specific enolase was positive in the carcinoid cells. Since somatostatin (SS) and SS analogues inhibit the growth of carcinoid via the SS receptor (SSTR) 2, we evaluated the expression of SSTR2 in the carcinoid cells using reverse transcription-polymerase chain reaction, and this confirmed the expression of SSTR2 in the carcinoid cells. Our experience of this patient with CS due to an ectopic ACTH-producing atypical thymic carcinoid suggests that SS analogues may be useful for treatment of carcinoid showing expression of SSTR2.
authors.affiliation: Department of Medicine, Tokai University School of Medicine, 143 Shimokasuya, Isehara, Kanagawa 259-1193, Japan. hrhrsato@is.icc.u-tokai.ac.jp
authors.forename: Haruhiro
authors.initials: H
authors.lastname: Sato
authors.forename: Hanako
authors.initials: H
authors.lastname: Kajiya
authors.forename: Genta
authors.initials: G
authors.lastname: Kanai
authors.forename: Takashi
authors.initials: T
authors.lastname: Hirukawa
authors.forename: Hisae
authors.initials: H
authors.lastname: Tanaka
authors.forename: Takatoshi
authors.initials: T
authors.lastname: Kakuta
authors.forename: Chie
authors.initials: C
authors.lastname: Inomoto
authors.forename: Robert Yoshiyuki
authors.initials: RY
authors.lastname: Osamura
mesh_terms: D000324:Adrenocorticotropic Hormone / Q000378:metabolism; D002276:Carcinoid Tumor / Q000150:complications / Q000473:pathology; D003480:Cushing Syndrome / Q000209:etiology*; D018450:Disease Progression; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008479:Mediastinal Neoplasms / Q000150:complications / Q000473:pathology; D008875:Middle Aged; D017481:Receptors, Somatostatin / Q000235:genetics / Q000378:metabolism; D013953:Thymus Neoplasms / Q000150:complications / Q000473:pathology
chemical_list: D017481:Receptors, Somatostatin; D000324:Adrenocorticotropic Hormone; C093733:somatostatin receptor 2