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Cushing Syndrome in a 6-Month-Old Infant due to Adrenocortical Tumor
Elizabeth B Fudge1, Daniel von Allmen, Keith E Volmar
1Division of Pediatric Endocrinology, University of North Carolina, Chapel Hill, NC 27599, USA.
Insights
Pediatric Cushing syndrome, rare in infants, can stem from adrenocortical carcinoma. Surgical removal of an infant
Area of Science:
- Pediatric Endocrinology
- Pediatric Oncology
- Surgical Oncology
Background:
- Cushing syndrome is rare in infancy.
- Adrenocortical tumors (ACT) are a common cause of pediatric Cushing syndrome.
- Early diagnosis and treatment are crucial for favorable outcomes.
Observation:
- A six-month-old infant presented with growth failure, weight gain, acne, and irritability.
- Clinical signs included obesity, hypertension, and Cushingoid features.
- Biochemical tests revealed elevated cortisol and suppressed ACTH, with an adrenal mass identified on MRI.
Findings:
- The infant had Cushing syndrome secondary to adrenocortical carcinoma.
- Surgical resection of the right adrenal mass was successful.
- Pathology confirmed capsular and vascular invasion, despite a low mitotic rate.
Implications:
- Adrenocortical neoplasms should be considered in the differential diagnosis of infant Cushing syndrome.
- Complete surgical excision is the primary treatment modality.
- Long-term surveillance is necessary to monitor for recurrence.
Abstract:
Cushing syndrome is rare in infancy and usually due to an adrenocortical tumor (ACT). We report an infant with Cushing syndrome due to adrenocortical carcinoma. The patient presented at six months of age with a three-month history of growth failure, rapid weight gain, acne, and irritability. Physical examination showed obesity, hypertension, and Cushingoid features. Biochemical evaluation showed very high serum cortisol, mildly elevated testosterone, and suppressed ACTH. Abdominal MRI revealed a heterogeneous right adrenal mass extending into the inferior vena cava. Evaluation for metastases was negative. The tumor was removed surgically en bloc. Pathologic examination demonstrated low mitotic rate, but capsular and vascular invasion. She received no adjuvant therapy. Her linear growth has improved and Cushingoid features resolved. Hormonal markers and quarterly PET scans have been negative for recurrence 24 months postoperatively. In conclusion, adrenocortical neoplasms in children are rare, but should be considered in the differential diagnosis of Cushing syndrome.
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