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- Title
Ectopic adrenocorticotropic hormone syndrome originating in maxillary sinus papilloma with a relapse in an non-localized origin.
- Authors
PRZYBYLIK-MAZUREK, Elwira; MINASYAN, Mari; WITOSZEK, Anna; SZUMIŃSKA, Małgorzata; SOWA-STASZCZAK, Anna; JAKUBOWSKA, Magdalena; URBANIK, Andrzej; HUBALEWSKA-DYDEJCZYK, Alicja
- Abstract
Ectopic adrenocorticotropic hormone syndrome (EAS) is a very rare cause of Cushing Syndrome (CS) and is caused by non-pituitary adrenocorticotropic hormone (ACTH) hypersecretion. The presentation is much more rapid and severe than in Cushing Disease. The most common source of ACTH overproduction is lung neoplasm. Despite many diagnostic evaluations, the origin of hormonal hypersecretion may remain occult. We present 81year old female who presented with severe hypokalemia, cushingoid features, hypercortisolemia, elevated ACTH and lack of suppression on 2 and 8mg dexamethasone suppression test. Further imaging work ups showed tumor in the right maxillary sinus, which was resected. Histopathological evaluation showed oncocytic cell type Schneiderian papilloma with ACTH expression. According to the reviewed literature, beside our case, until now there's been only one other case presenting with EAS produced by paranasal sinus' polyp mucosae. She remained in remission for 5 years. Five years later Cushing syndrome relapsed, with complications like urosepsis and absence type seizures. Despite extensive diagnostic methods, the source of recurrent EAS remained unlocalized and patient stayed on steroidogenesis inhibitor. Nevertheless, further controlling imaging studies are warranted, because primarily occult origin may become detectable over some period of time.
- Subjects
ADRENOCORTICOTROPIC hormone; MAXILLARY sinus; DISEASE relapse; CUSHING'S syndrome diagnosis; PITUITARY-adrenal function tests
- Publication
Review of Medicine / Przeglad Lekarski, 2019, Vol 76, Issue 10, p520
- ISSN
0033-2240
- Publication type
Article