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Ectopic Cushing's Syndrome in Pediatric Age
Guido Roberto1, Maria Elena Aloini1, Irene Biondo1
1Azienda Ospedaliera Sant'Andrea, Endocrinology, Department of Clinical and Molecular Medicine, Sant'Andrea University Hospital, Sapienza University of Rome, Rome, Italy.
Endocrine, Metabolic & Immune Disorders Drug Targets
|July 23, 2024
Summary
Ectopic ACTH-secreting neuroendocrine tumors cause Cushing's syndrome. 68Ga-PET/DOTATOC imaging and ACTH monitoring effectively identified and managed a rare thymic neuroendocrine tumor case.
Area of Science:
- Endocrinology
- Oncology
- Nuclear Medicine
Background:
- Ectopic ACTH secretion causing Cushing's syndrome is rare, originating from neuroendocrine tumors.
- The incidence of endogenous Cushing's syndrome is low, with children representing a small fraction of cases.
Purpose of the Study:
- To describe a challenging case of ectopic Cushing's syndrome.
- To evaluate the utility of 68Ga-PET/DOTATOC imaging and ACTH level monitoring in diagnosis and surgical management.
Main Methods:
- Case report of a 17-year-old female with ectopic Cushing's syndrome.
- Utilized 68Ga-PET/DOTATOC and 18FDG-PET scans for lesion localization.
- Monitored adrenocorticotropic hormone (ACTH) levels throughout surgical interventions.
Main Results:
- Initial PET scans were negative; a subsequent 68Ga-PET/DOTATOC identified a thymic nodule.
- Despite thymectomy, hypercortisolism persisted, indicating incomplete resection.
- Repeated imaging and surgery successfully resolved the condition, confirmed by normalized ACTH levels.
Conclusions:
- 68Ga-PET/DOTATOC is a sensitive and specific tool for identifying ACTH-secreting neuroendocrine lesions.
- Monitoring short-acting ACTH levels intraoperatively aids in confirming complete surgical resection.
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