Ectopic ACTH production by thymic and appendiceal neuroendocrine tumors - two case reports

Objectives: Ectopic adrenocorticotropic syndrome (EAS) causes approximately 10-18% of cases of Cushing's syndrome (CS) in adults, while in children it occurs much less frequently. Case presentation: We report two cases of neuroendocrine tumors (of the thymus and the appendix) in a 12-yearold boy and a 15-year-old girl who presented with the clinical features of CS. Elevated serum cortisol, ACTH, and chromogranin levels were observed in both patients. Diagnoses were made on the basis of a mass in the thymus/appendix region visualized with chest/abdominal CT scan and radiotracer accumulation in scintigraphy in the same areas. Histopathological examinations confirmed the diagnoses of NET. Conclusion: EAS is an extremely rare endocrine disorder. However, it should be taken into consideration in the diagnostic process of every case of ACTH-dependent CS.

Automated summary

Two cases of neuroendocrine tumors in adolescents presenting with clinical features of Cushing's syndrome were diagnosed with ectopic adrenocorticotropic syndrome based on imaging and histopathological examinations. This rare endocrine disorder should be considered in the diagnostic process of ACTH-dependent Cushing's syndrome cases.