Using Etomidate in a Two-month-old Infant with Cushing Syndrome due to Adrenocortical Carcinoma

Abstract

Cushing syndrome (CS) is a rare disease caused by hypercortisolemia. Although surgical treatment is the first-line treatment in CS, the appropriate medication for the patient’s condition should be selected when medical treatment is needed. Etomidate is an adrenal-blocking drug used to treat CS and the most suitable for severe hypercortisolemia and adrenocortical carcinoma (ACC), due to cardiovascular stability and an anti-tumorigenic effect. However, its use and safe recommended dosage in infants with CS is unreported. Here we describe the case of a 2-month-old girl treated with etomidate for CS caused by ACC. Even though radical mass excision was performed, severe hypercortisolemia persisted, resulting from metastatic lesions in the liver, and medical treatment was considered. The etomidate doses, no bolus dose and infusion rate of 0.03 mg/kg/hour, may be an appropriate dose for severe hypercortisolemia in infants. This case will help determine future treatment strategies for similar cases in infants.

Adrenocortical Carcinoma: Updates of Clinical and Pathological Features after Renewed World Health Organisation Classification and Pathology Staging

“…The disease is more common in the first decade with peak in 1 to 4 years [ 82 ] and with the mean age at presentation at 5 [ 86 ]. Infants of less than 6-months-old with ACC have been reported [ 87 , 88 , 89 ].…”

Adrenocortical carcinoma and a sporadic MEN1 mutation in a 3-year-old girl: a case report

“…During our search on PubMed.gov, we found only five cases of childhood ACC reported in Korea. Among them, 3 cases reported treatment modalities and prognoses of ACC [ 14 , 17 , 18 ]. Another was about virilizing ACC in Turner syndrome with a TP53 gene mutation, and the last was a case of virilizing adrenocortical oncocytoma with borderline malignancy potential based on the Lin-Weiss-Bisceglia criteria [ 19 , 20 ].…”

Adrenocortical carcinoma: Pediatric aspects (Review)

“…A previous study revealed that virilization was a favorable prognostic factor (12). In addition, the control of tumor-related Cushing syndrome may be achieved not only through mitotane, but also by using other adrenal blockers that are mostly used in adults, and only exceptionally used in children to date (61). In 2020, a case of a 2-month-old female treated with etomidate (0.03 mg/hg/h) to control high cortisol levels was published, and further implementation of the mentioned drug in complex regimes is required (61).…”

“…In addition, the control of tumor-related Cushing syndrome may be achieved not only through mitotane, but also by using other adrenal blockers that are mostly used in adults, and only exceptionally used in children to date (61). In 2020, a case of a 2-month-old female treated with etomidate (0.03 mg/hg/h) to control high cortisol levels was published, and further implementation of the mentioned drug in complex regimes is required (61). Mitotane, an adrenolytic chemotherapeutic, causes adrenal insufficiency which requires glucocorticoid and mineralocorticoid substitution, increased liver enzymes, high cholesterol, gynecomastia, and primary hypothyroidism in adults and children (62).…”