naa a22 4500 510773346 CHVBK 20180411083222.0 cr unu---uuuuu 180411e20130301xx s 000 0 eng 10.1007/s12022-012-9228-5 doi (NATIONALLICENCE)springer-10.1007/s12022-012-9228-5 Recurrent Acute-Onset Cushing's Syndrome 6Years after Removal of a Thymic Neuroendocrine Carcinoma: From Ectopic ACTH to CRH [Elektronische Daten] [Camilla Schalin-Jäntti, Sylvia Asa, Johanna Arola, Timo Sane] We describe a rare case of ectopic Cushing's syndrome that recurred 6years after resection of a thymic neuroendocrine carcinoma. We discuss reasons for the differing clinical presentations, management, hormone profiles, as well as immunopathology. A 41-year-old male developed acute-onset Cushing's syndrome. Clinical presentation and laboratory results were compatible with ectopic adrenocorticotropin hormone (ACTH) production. Computerized tomography (CT) showed a 3.6cm thymic tumor which was successfully resected. Plasma ACTH (P-ACTH) normalized the first postoperative day. Histopathology demonstrated a well-differentiated neuroendocrine carcinoma with diffuse positivity for ACTH and focal corticotropin-releasing hormone (CRH) reactivity in a few scattered cells. The patient was in remission for 6years. He then again presented with acute-onset Cushing's syndrome. Fluorine-labeled dihydroxyphenylalanine (18F-DOPA) PET/CT showed local uptake in the mediastinum and he underwent repeat resection. However, P-ACTH remained increased (613ng/l) and 24-h urinary cortisol was 36,720nmol, suggesting incomplete tumor removal or metastatic spread. Metyrapone treatment was initiated but then withdrawn because the patient spontaneously recovered and cortisol metabolism gradually normalized within 3weeks. Histopathology demonstrated a recurrent neuroendocrine carcinoma with the same features as the previous lesion but this time CRH was strongly positive in more numerous cells. Normalization of P-ACTH after primary surgery was compatible with ectopic ACTH production. However, the delayed fall in P-ACTH and serum cortisol is compatible with ectopic CRH production and stimulation of pituitary ACTH secretion, which gradually resolved. Although ectopic CRH production is very rare, the unusual dynamics illustrated here should raise the possibility of CRH production by a neuroendocrine tumor. Springer Science+Business Media New York, 2012 Cushing's syndrome nationallicence Neuroendocrine carcinoma nationallicence Ectopic ACTH nationallicence Ectopic CRH nationallicence Immunostaining nationallicence Schalin-Jäntti Camilla Division of Endocrinology, Department of Medicine, Helsinki University Central Hospital, P.O. Box 340, 00290, Helsinki, Finland aut Asa Sylvia Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Canada aut Arola Johanna Department of Pathology, Haartman Institute, University of Helsinki, Helsinki, Finland aut Sane Timo Division of Endocrinology, Department of Medicine, Helsinki University Central Hospital, P.O. Box 340, 00290, Helsinki, Finland aut Endocrine Pathology Springer US; http://www.springer-ny.com 24/1(2013-03-01), 25-29 1046-3976 24:1<25 2013 24 12022 https://doi.org/10.1007/s12022-012-9228-5 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s12022-012-9228-5 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Schalin-Jäntti Camilla Division of Endocrinology, Department of Medicine, Helsinki University Central Hospital, P.O. Box 340, 00290, Helsinki, Finland aut NATIONALLICENCE 700 1- Asa Sylvia Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Canada aut NATIONALLICENCE 700 1- Arola Johanna Department of Pathology, Haartman Institute, University of Helsinki, Helsinki, Finland aut NATIONALLICENCE 700 1- Sane Timo Division of Endocrinology, Department of Medicine, Helsinki University Central Hospital, P.O. Box 340, 00290, Helsinki, Finland aut NATIONALLICENCE 773 0- Endocrine Pathology Springer US; http://www.springer-ny.com 24/1(2013-03-01), 25-29 1046-3976 24:1<25 2013 24 12022 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer