Dual bronchial carcinoids and Cushing's syndrome with a paradoxical response to dexamethasone and a false positive outcome of inferior petrosal sinus sampling.

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Dual bronchial carcinoids and Cushing's syndrome with a paradoxical response to dexamethasone and a false positive outcome of inferior petrosal sinus sampling. / Burman, Pia; Lethagen, ÅsaLinda; Ivancev, Krasnodar; Johansson, Leif; Sundin, Anders.

I: European Journal of Endocrinology, Vol. 159, 2008, s. 483-488.

Forskningsoutput: TidskriftsbidragArtikel i vetenskaplig tidskrift

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T1 - Dual bronchial carcinoids and Cushing's syndrome with a paradoxical response to dexamethasone and a false positive outcome of inferior petrosal sinus sampling.

AU - Burman, Pia

AU - Lethagen, ÅsaLinda

AU - Ivancev, Krasnodar

AU - Johansson, Leif

AU - Sundin, Anders

N1 - The information about affiliations in this record was updated in December 2015. The record was previously connected to the following departments: Pediatrics/Urology/Gynecology/Endocrinology (013240400), Pathology, (Lund) (013030000)

PY - 2008

Y1 - 2008

N2 - Objective Establishing the cause of Cushing's syndrome can be a considerable challenge, in particular in ectopic ACTH syndrome, and often requires a combination of biochemical tests and imaging procedures. Subject A 27-year-old man presented with signs of Cushing's syndrome. P-ACTH levels were 3 times above the ULN and free urinary cortisol in the order of 1900 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 inferior petrosal sinuses (BIPSS) showed a central to peripheral ACTH ratio of 4.7 after CRH stimulation, i.e. indicated pituitary disease, but MRI of the pituitary was normal and baseline P-CRH <1.0 (ref. <5) pmol/L. CT scan of the lungs showed 2 oval-shaped masses, 1.3 x 1.8 and 1.3 x 2 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 unusual case with dual bronchial carcinoids inducing hypercortisolism illustrates the problems with identifying the source of ACTH in Cushing s syndrome. Possibly, the false positive result at BIPSS reflects an unusual sensitivity of the pituitary corticotrophs to CRH in this patient since ectopic CRH secretion and/or eucortisolism at the time of the procedure could be ruled out. The work-up illustrates the great value of 11C-5-hydroxytryptophan-PET as a diagnostic procedure when other investigations have produced ambiguous results.

AB - Objective Establishing the cause of Cushing's syndrome can be a considerable challenge, in particular in ectopic ACTH syndrome, and often requires a combination of biochemical tests and imaging procedures. Subject A 27-year-old man presented with signs of Cushing's syndrome. P-ACTH levels were 3 times above the ULN and free urinary cortisol in the order of 1900 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 inferior petrosal sinuses (BIPSS) showed a central to peripheral ACTH ratio of 4.7 after CRH stimulation, i.e. indicated pituitary disease, but MRI of the pituitary was normal and baseline P-CRH <1.0 (ref. <5) pmol/L. CT scan of the lungs showed 2 oval-shaped masses, 1.3 x 1.8 and 1.3 x 2 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 unusual case with dual bronchial carcinoids inducing hypercortisolism illustrates the problems with identifying the source of ACTH in Cushing s syndrome. Possibly, the false positive result at BIPSS reflects an unusual sensitivity of the pituitary corticotrophs to CRH in this patient since ectopic CRH secretion and/or eucortisolism at the time of the procedure could be ruled out. The work-up illustrates the great value of 11C-5-hydroxytryptophan-PET as a diagnostic procedure when other investigations have produced ambiguous results.

U2 - 10.1530/EJE-08-0385

DO - 10.1530/EJE-08-0385

M3 - Article

VL - 159

SP - 483

EP - 488

JO - European Journal of Endocrinology

T2 - European Journal of Endocrinology

JF - European Journal of Endocrinology

SN - 1479-683X

ER -