Dual bronchial carcinoids and Cushing's syndrome with a paradoxical response to dexamethasone and a false positive outcome of inferior petrosal sinus sampling.
(2008) In European Journal of Endocrinology 159. p.483-488- Abstract
- 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.... (More)
- 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. (Less)
Please use this url to cite or link to this publication:
https://lup.lub.lu.se/record/1181157
- author
- Burman, Pia ; Lethagen, ÅsaLinda LU ; Ivancev, Krasnodar ; Johansson, Leif LU and Sundin, Anders
- organization
- publishing date
- 2008
- type
- Contribution to journal
- publication status
- published
- subject
- in
- European Journal of Endocrinology
- volume
- 159
- pages
- 483 - 488
- publisher
- Society of the European Journal of Endocrinology
- external identifiers
-
- wos:000260169900018
- pmid:18625689
- scopus:54049084525
- pmid:18625689
- ISSN
- 1479-683X
- DOI
- 10.1530/EJE-08-0385
- language
- English
- LU publication?
- yes
- additional info
- 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)
- id
- a80f55d1-0cdb-4a58-8f4d-0e7eade95140 (old id 1181157)
- alternative location
- http://www.ncbi.nlm.nih.gov/pubmed/18625689?dopt=Abstract
- date added to LUP
- 2016-04-04 08:56:31
- date last changed
- 2022-01-29 07:55:51
@article{a80f55d1-0cdb-4a58-8f4d-0e7eade95140,
abstract = {{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.}},
author = {{Burman, Pia and Lethagen, ÅsaLinda and Ivancev, Krasnodar and Johansson, Leif and Sundin, Anders}},
issn = {{1479-683X}},
language = {{eng}},
pages = {{483--488}},
publisher = {{Society of the European Journal of Endocrinology}},
series = {{European Journal of Endocrinology}},
title = {{Dual bronchial carcinoids and Cushing's syndrome with a paradoxical response to dexamethasone and a false positive outcome of inferior petrosal sinus sampling.}},
url = {{http://dx.doi.org/10.1530/EJE-08-0385}},
doi = {{10.1530/EJE-08-0385}},
volume = {{159}},
year = {{2008}},
}