title: Metastatic Pancreatic Neuroendocrine Tumor that Progressed to Ectopic Adrenocorticotropic Hormone (ACTH) Syndrome with Growth Hormone-releasing Hormone (GHRH) Production.
abstract: The patient was a 61-year-old woman who had a well-differentiated pancreatic neuroendocrine tumor (PNET) with lymph node metastasis. After 15 months of octreotide treatment, glucose control deteriorated and pigmentation of the tongue and moon face developed, leading to the diagnosis of ectopic adrenocorticotropic hormone (ACTH) syndrome. An abnormal secretion of growth hormone (GH) was identified, and the plasma growth hormone-releasing hormone (GHRH) level was elevated. A tumor biopsy specimen positively immunostained for ACTH and GHRH. Ectopic hormone secretion seems to have evolved along with the progression of the PNET.
authors.affiliation: Department of Medicine, Division of Diabetes, Metabolism and Endocrinology, Showa University Fujigaoka Hospital, Japan.
authors.forename: Rie
authors.initials: R
authors.lastname: Tadokoro
authors.forename: Shotaro
authors.initials: S
authors.lastname: Sato
authors.forename: Fumiko
authors.initials: F
authors.lastname: Otsuka
authors.forename: Makoto
authors.initials: M
authors.lastname: Ueno
authors.forename: Shinichi
authors.initials: S
authors.lastname: Ohkawa
authors.forename: Hideki
authors.initials: H
authors.lastname: Katakami
authors.forename: Matsuo
authors.initials: M
authors.lastname: Taniyama
authors.forename: Shoichiro
authors.initials: S
authors.lastname: Nagasaka
mesh_terms: D000182:ACTH Syndrome, Ectopic / Q000209:etiology*; D000324:Adrenocorticotropic Hormone / Q000378:metabolism; D018931:Antineoplastic Agents, Hormonal / Q000627:therapeutic use; D005260:Female; D013007:Growth Hormone-Releasing Hormone / Q000378:metabolism*; D006801:Humans; D008875:Middle Aged; D018358:Neuroendocrine Tumors / Q000188:drug therapy / Q000378:metabolism* / Q000556:secondary; D015282:Octreotide / Q000627:therapeutic use; D010190:Pancreatic Neoplasms / Q000188:drug therapy / Q000378:metabolism* / Q000473:pathology
chemical_list: D018931:Antineoplastic Agents, Hormonal; D000324:Adrenocorticotropic Hormone; D013007:Growth Hormone-Releasing Hormone; D015282:Octreotide