Chief Cushie ~MaryO~ Posted September 25, 2008 Chief Cushie Report Share Posted September 25, 2008 http://www.eje.org/cgi/content/abstract/159/4/483 DOI: 10.1530/EJE-08-0385 European Journal of Endocrinology, Vol 159, Issue 4, 483-488 Copyright ? 2008 by European Society of Endocrinology CASE REPORT Dual bronchial carcinoids and Cushing's syndrome with a paradoxical response to dexamethasone and a false positive outcome of inferior petrosal sinus sampling Pia Burman, ?saLinda Lethagen, Krasnodar Ivancev, Leif Johansson and Anders Sundin Departments of Endocrinology , Radiology Pathology, University Hospital MAS, SE-205 02 Malm?, Sweden4 Department of Diagnostic Radiology, University Hospital, Uppsala, Sweden (Correspondence should be addressed to P Burman; Email: pia.a.burman@skane.se) Abstract Context: Establishing the cause of Cushing's syndrome (CS) can be a considerable challenge, in particular in ectopic adrenocorticotropic hormone (ACTH) syndrome, and often requires a combination of biochemical tests and imaging procedures. Subject: A 27-year-old man presented with signs of CS. P-ACTH levels were three times above the upper limit of normal (ULN) and free urinary cortisol around 2000 nmol/24 h. The work-up showed remarkable results. Results: A 2-day low-dose dexamethasone suppression test demonstrated paradoxical increases in cortisol. Sampling from the bilateral inferior petrosal sinus sampling (BIPSS) showed a central to peripheral ACTH ratio of 4.7 after corticotrophin-releasing hormone (CRH) stimulation, i.e. indicated pituitary disease, but magnetic resonance imaging of the pituitary was normal. Computed tomography (CT) scan of the lungs showed two oval-shaped masses, 1.3x1.8 and 1.3x2 cm, in the middle lobe. Both were positive at somatostatin receptor scintigraphy, compatible with tumors or inflammatory lesions. Subsequently, 11C-5-hydroxytryptophan-PET showed distinct uptake in the tumors but not elsewhere. Two carcinoids situated 3 cm apart, both staining for ACTH, were removed at surgery. Conclusion: This unique case with dual bronchial carcinoids inducing hypercortisolism illustrates the problems with identifying the source of ACTH in CS. Possibly, an abnormal regulation of ACTH production in response to dexamethasone, or steroid-induced tumor necrosis, explains the paradoxical outcome at dexamethasone suppression, and the false positive result at BIPSS reflects an unusual sensitivity of the pituitary corticotrophs to CRH in this patient. The work-up illustrates the great value of 11C-5-hydroxytryptophan-PET as a diagnostic procedure when other investigations have produced ambiguous results. Link to comment Share on other sites More sharing options...
Member of the 1000 Post Club WackyPhotographer Posted September 25, 2008 Member of the 1000 Post Club Report Share Posted September 25, 2008 Sounds to scary for me to read Link to comment Share on other sites More sharing options...
Over 2000 Posts Jo MacRaild Posted September 25, 2008 Over 2000 Posts Report Share Posted September 25, 2008 Thanks Mary, kind of blows the only adrenal disease paradoxicaly increases to dex theory right out the water..Poor guy, but glad they worked it out for him. Link to comment Share on other sites More sharing options...
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