Ectopic cushing syndrome caused by intestinal neuroendocrine tumors: a systematic review.
Fernando Guerrero-Pérez, Reinaldo Sánchez-Barrera, Jordi Puig de la Bellacasa et al.
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In 30 seconds
This systematic review characterized ectopic Cushing syndrome caused by intestinal neuroendocrine tumors using published case reports and an illustrative unpublished case. It identified 24 published cases. Ectopic Cushing syndrome was the initial presentation in 87.5%, and 70.8% had an aggressive clinical phenotype. Primary tumor localization was difficult: conventional imaging detected the lesion in fewer than half of cases, while functional imaging, particularly gallium-68 somatostatin receptor PET, had higher detection rates. Surgery was performed in 87.5%, with biochemical remission in 72.7%; recurrence occurred in 14.3%.
Key findings
- Ectopic Cushing syndrome was the initial manifestation in 87.5% of the 24 published cases; 70.8% had an aggressive clinical phenotype.
- Conventional imaging detected the primary lesion in fewer than half of cases; functional imaging, particularly gallium-68 somatostatin receptor PET, had higher detection rates.
- Surgical resection was performed in 87.5% of patients, and biochemical remission was achieved in 72.7%.
- Half required medical therapy for hypercortisolism, 20.8% underwent bilateral adrenalectomy, and recurrence occurred in 14.3%.
Why it matters
These cases highlight intestinal neuroendocrine tumors as an uncommon source of ectopic Cushing syndrome that may present with severe disease and be difficult to localize. The findings underscore the need to consider functional imaging when conventional imaging is unrevealing and to address both cortisol excess and the underlying tumor. Recurrence after initial remission makes ongoing follow-up relevant.
What to keep in mind
The review is based on published case reports, with descriptive analysis; the small case count and potential for publication bias limit generalizability.