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Cushing's syndrome due to an ectopic ACTH-secreting pituitary tumour mimicking occult paraneoplastic ectopic ACTH production.
Clin Endocrinol (Oxf). 1999 Dec; 51(6):809-14.CE

Abstract

A 32-year-old man presenting with typical features of Cushing's syndrome showed baseline cortisol and ACTH values indicating ACTH-dependent disease. Dynamic function tests (dexamethasone, corticotropin releasing hormone (CRH), desmopressin), were suggestive of paraneoplastic ectopic ACTH production. However, inferior petrosal sinus (IPS) ACTH sampling demonstrated a maximum baseline central (363 pmol/l)-peripheral (19 pmol/l) ACTH gradient of 19.1 for the right IPS, conventionally suggestive of Cushing's disease. However, again, IPS ACTH level did not increase after CRH stimulation. Magnetic resonance imaging, while showing no evidence of an intrasellar tumour, revealed an 1.5 x 1.0 cm mass in the left sphenoid sinus which was initially interpreted as most probably being a mucosal polyp. After neurosurgical removal of the tumour, transient secondary adrenal insufficiency was present. The structure and immunostaining characteristics of the tumour demonstrated an ACTH cell adenoma of the pituitary. Ectopic ACTH-secreting pituitary adenomas may cause significant difficulties in differential diagnosis, localisation and appropriate therapy. Thus, although these tumours are rare, they should be included in the list of possible causes of ACTH-dependent Cushing's syndrome.

Authors+Show Affiliations

Department of Internal Medicine III, Municipal Hospital, Darmstadt, University of Würzburg, Germany.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

10619988

Citation

Bethge, H, et al. "Cushing's Syndrome Due to an Ectopic ACTH-secreting Pituitary Tumour Mimicking Occult Paraneoplastic Ectopic ACTH Production." Clinical Endocrinology, vol. 51, no. 6, 1999, pp. 809-14.
Bethge H, Arlt W, Zimmermann U, et al. Cushing's syndrome due to an ectopic ACTH-secreting pituitary tumour mimicking occult paraneoplastic ectopic ACTH production. Clin Endocrinol (Oxf). 1999;51(6):809-14.
Bethge, H., Arlt, W., Zimmermann, U., Klingelhöffer, G., Wittenberg, G., Saeger, W., & Allolio, B. (1999). Cushing's syndrome due to an ectopic ACTH-secreting pituitary tumour mimicking occult paraneoplastic ectopic ACTH production. Clinical Endocrinology, 51(6), 809-14.
Bethge H, et al. Cushing's Syndrome Due to an Ectopic ACTH-secreting Pituitary Tumour Mimicking Occult Paraneoplastic Ectopic ACTH Production. Clin Endocrinol (Oxf). 1999;51(6):809-14. PubMed PMID: 10619988.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Cushing's syndrome due to an ectopic ACTH-secreting pituitary tumour mimicking occult paraneoplastic ectopic ACTH production. AU - Bethge,H, AU - Arlt,W, AU - Zimmermann,U, AU - Klingelhöffer,G, AU - Wittenberg,G, AU - Saeger,W, AU - Allolio,B, PY - 2000/1/5/pubmed PY - 2000/5/20/medline PY - 2000/1/5/entrez SP - 809 EP - 14 JF - Clinical endocrinology JO - Clin Endocrinol (Oxf) VL - 51 IS - 6 N2 - A 32-year-old man presenting with typical features of Cushing's syndrome showed baseline cortisol and ACTH values indicating ACTH-dependent disease. Dynamic function tests (dexamethasone, corticotropin releasing hormone (CRH), desmopressin), were suggestive of paraneoplastic ectopic ACTH production. However, inferior petrosal sinus (IPS) ACTH sampling demonstrated a maximum baseline central (363 pmol/l)-peripheral (19 pmol/l) ACTH gradient of 19.1 for the right IPS, conventionally suggestive of Cushing's disease. However, again, IPS ACTH level did not increase after CRH stimulation. Magnetic resonance imaging, while showing no evidence of an intrasellar tumour, revealed an 1.5 x 1.0 cm mass in the left sphenoid sinus which was initially interpreted as most probably being a mucosal polyp. After neurosurgical removal of the tumour, transient secondary adrenal insufficiency was present. The structure and immunostaining characteristics of the tumour demonstrated an ACTH cell adenoma of the pituitary. Ectopic ACTH-secreting pituitary adenomas may cause significant difficulties in differential diagnosis, localisation and appropriate therapy. Thus, although these tumours are rare, they should be included in the list of possible causes of ACTH-dependent Cushing's syndrome. SN - 0300-0664 UR - https://www.unboundmedicine.com/medline/citation/10619988/Cushing's_syndrome_due_to_an_ectopic_ACTH_secreting_pituitary_tumour_mimicking_occult_paraneoplastic_ectopic_ACTH_production_ L2 - https://onlinelibrary.wiley.com/resolve/openurl?genre=article&sid=nlm:pubmed&issn=0300-0664&date=1999&volume=51&issue=6&spage=809 DB - PRIME DP - Unbound Medicine ER -