Oncocytic adrenocortical tumor with uncertain malignant potential in pediatric population: A case report and review of literature

BACKGROUND: Oncocytic adrenocortical tumor (OACT) is rare, with few cases reported in the literature. No more than 20 cases in children have been reported. The clinical characteristics, diagnosis, treatment and prognosis of children with OACT are summarized based on a literature review, in order to improve the understanding of OACT in children. CASE SUMMARY: We report a case of a 17-mo-old patient who was admitted to our hospital due to symptoms of odynuria and fever, which are clinical features consistent with a functional adrenocortical tumor. The patient was diagnosed with OACT of uncertain malignant potential. Computed tomography indicated a soft tissue giant tumor in the right adrenal region, approximately 4.3 cm × 5.5 cm in size. Multiple nodular and speckled calcifications were observed in the lesion. The patient received robot-assisted laparoscopic right adrenal tumor resection. Postoperative pathological results were consistent with OACT, and immunohistochemical results showed cytokeratin(+/-), chromogranin A(+), synaptophysin(-), neuron-specific enolase(-), S100(-), Ki67 about 10%, CD34(-) and D2-40(-). After surgery, urinary tract ultrasonography was reviewed monthly, catecholamine hormone and sex hormone levels were examined every 2 mo and computed tomography was performed every 6 mo. To date, no tumor metastasis or recurrence has been identified in this patient. The levels of sex hormones and catecholamine hormones decreased to normal 1 mo after surgery. CONCLUSION: OACT is rare in the pediatric population, with few cases reported in the literature. Although most pediatric OACTs are benign, malignant cases have been reported. Surgical resection is the preferred option in most patients.