Pitfalls in the Diagnosis of Ectopic Adrenocorticotropic Hormone Syndrome: A Case Report
Bibliographic record
Abstract
Ectopic production of adrenocorticotropic hormone by carcinoid tumours is uncommon and covert and occult presentations often hamper its diagnosis. A 27-year-old man was admitted with multiple vertebral bodies collapse related to osteoporosis along with other clinical features consistent with Cushing’s syndrome. Both basal and dynamic laboratory evaluation suggested adrenocorticotropic-dependent Cushing’s syndrome diagnosis, specifically ectopic adrenocorticotropic syndrome. Laboratory testing also showed high serum levels of chromogranin A and urinary 5-hydroxyindoleacetic acid. Pituitary and thoracic-abdominal-pelvic imaging proved to be normal, including the octreoscan. To reduce cortisol levels, and before resorting to adrenalectomy, metyrapone was prescribed but eucortisolism was not achieved. Octreotide therapy reduced plasma adrenocorticotropic hormone and cortisoluria to half although the octreoscan had been negative. As no primitive tumour had been detected, the patient underwent bilateral adrenalectomy. Two years and a half after the diagnosis, an octreoscan detected a lung tumour, which was resected. Pathological analysis revealed an atypical carcinoid tumour and immunohistochemistry was positive for adrenocorticotropic hormone but negative for corticotropin-releasing hormone and chromogranin A. Clinical and biochemical resolution of Cushing’ syndrome followed but chromogranin A levels remained above normal. This case highlights both that, although rare, an exuberant osteoporosis can be the initial presentation of ectopic adrenocorticotropic hormone syndrome and high chromogranin A levels may have other origin than direct tumour production. It also raises the question of prescribing somatostatin agonists in patients whose octreoscan is negative as small tumours may produce false negative results. Moreover, the reduction of hypercortisolism before surgery may improve the surgical outcome and with bilateral adrenalectomy may contribute to the primary tumour growth and detection. doi: http://dx.doi.org/10.4021/jem145w
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.001 | 0.007 |
| Meta-epidemiology (narrow) | 0.003 | 0.002 |
| Meta-epidemiology (broad) | 0.002 | 0.002 |
| Bibliometrics | 0.006 | 0.003 |
| Science and technology studies | 0.004 | 0.003 |
| Scholarly communication | 0.004 | 0.005 |
| Open science | 0.002 | 0.004 |
| Research integrity | 0.011 | 0.006 |
| Insufficient payload (model declined to judge) | 0.003 | 0.002 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".