The Elusive Neuroendocrine Tumor: Finding the Ectopic ACTH Source 16 Years After the Diagnosis of Cushing Syndrome
Bibliographic record
Abstract
Ectopic adrenocorticotropin hormone (ACTH) syndrome (EAS) accounts for the minority of cases of Cushing syndrome. Up to 20% of these cases remain occult, despite multiple imaging attempts to localize the ACTH-producing tumor. Here we describe long-term follow-up of a 41-year-old woman, with ectopic Cushing syndrome initially classified as occult due to negative localization studies, who had bilateral adrenalectomy to manage hypercortisolism. After 16 years and many computed tomography (CT) scans, magnetic resonance imaging scans, Octreoscans, and 2 exploration surgeries for false positives on imaging, the source of ectopic ACTH production was localized in the pancreas utilizing molecular imaging with gallium-68 somatostatin receptor-targeted positron emission tomography (PET)/CT and fluorine-18 fluorodeoxyglucose PET/CT. She underwent a distal pancreatectomy, and pathology confirmed a 1.7-cm well-differentiated pancreatic neuroendocrine tumor with a moderately strong reactivity to ACTH stain. This case demonstrates the utility of multiple functional imaging modalities in resolving these "cold cases" of occult ectopic Cushing syndrome and the importance of a timely management of hypercortisolism with bilateral adrenalectomy.
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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 | 0.000 | 0.002 |
| Meta-epidemiology (narrow) | 0.001 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.001 |
| Bibliometrics | 0.001 | 0.001 |
| Science and technology studies | 0.001 | 0.001 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.001 | 0.001 |
| Research integrity | 0.003 | 0.002 |
| Insufficient payload (model declined to judge) | 0.001 | 0.001 |
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".