INTRODUCTION: ACTH-secreting pituitary microadenomas are associated with insidious onset in children and atypical early clinical manifestations, making them highly susceptible to misdiagnosis and underdiagnosis. They are characterized by elevated ACTH and cortisol levels with a disrupted circadian rhythm. We reported two pediatric cases that showed significant elevation of Dehydroepiandrosterone Sulfate (DHEAS) and Androstenedione (Ad) in the early stage, while ACTH and cortisol were in the normal range or mildly abnormal. CASE PRESENTATION: Case 1, a 13-year-old male, was admitted to the hospital with two years of growth retardation. The initial pituitary Magnetic Resonance Imaging (MRI) was normal. After one year, the patient developed Cushing's Syndrome (CS), and a repeat MRI suggested a pituitary microadenoma. Case 2, a 13.1-year-old female who had experienced excessive weight gain for two years and amenorrhea for 11 months. The child presented with CS initially, and the MRI revealed a pituitary microadenoma. Both of them were pathologically confirmed as ACTH-secreting pituitary microadenomas after Transsphenoidal Surgery (TSS). CONCLUSION: This report demonstrates that in cases with a significant DHEAS elevation, even with near-normal ACTH/cortisol levels and negative pituitary MRI, high clinical suspicion for ACTH- secreting microadenomas should be maintained, warranting close follow-up and further evaluation.
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