A Case of Virilizing Adrenal Cortical Tumor in Child

Adrenal cortical tumor is a rare disease both in adults and children. Most of these tumors are malignant and functional, especially in children, producing endocrine syndrome such as virilization, Cushing's syndrome, hyperaldosteronism or feminization. Recently, we experienced a case of adrenal cortical tumor in a 26 month-old boy who showed features of virilization. This case was diagnosed with typical hormonal findings and abdominal CT and confirmed by pathologic examinations. He was successfully treated with right adrenalectomy. We report a case of virilizing adrenal cortical tumor and review the literature.

Signal Transduction Related Oncogenes in Human Adrenal Cortical Tumor; Gsα Giα, CREB / 대한내분비학회지

Functioning adrenal cortical tumors are originated form a distinct zone(zonna glomerulosa, zonna fasciculata or zonna reticularis) or the transitonal zone of adrenal gland. Each zone of the gland is regulated by their specific hormons or cytokines, and their signal transduction systems are different. The oncogenes of many endocrine tumors were mutated proteins involved in signal transduction, however gip is the only reported oncogene in adrenal cortical tumors. Therefore we decided to reevaluate whether gsp might be detected as an oncogene in several different functioning adrenal tumors, and we also tested whether CREB protein is a tentative oncogene or not. In our study, gsp was not detected in 13 patients, however gip was not also detected unexpectedly. There were no mutations in the phosporylation site of CREB("P" box) in adrenal cushing syndrome. We concluded that gip was not a oncogene detected frequently in adrenal cortical tumor, and CREB protein was not considered as a tentative oncogen, because there might be no amplification of the signals due to its extreme distal component of PKA or PKC system.

Neuroendocrine Differentiation in Adrenal Cortical Tumor of Chidhood: A case report

Although neuroendocrine differentiation is a characteristic feature of tumors of the adrenal medulla, cortical tumors may also rarely be differentiated into medullary element. Recently we experienced such a case of adrenal cortical tumor having features of both cortical and medullary tumor. The patient was an 11-year-old girl who was incidentally found to have a left adrenal mass. Laboratory results showed elevated serum cortisol, aldosterone, renin, and epinephrine with high excretion of urinary metanephrine. Urine vanillyl mandelic acid and 17-ketosteroid remained within normal limits. Histologic featuresof a 6 cm round yellowish tumor were ambiguous to decide the orgin of this neoplasm. Cortical element predominated in the tumor with minor areas of pheochromocytomatous feature. Immunohistochemically, the tumor cells were positive for vimentin, neuron specific enolase, and epithelial membrane antigen. Ultrastructural examination revealed scattered membrane bound dense core granules in the tumor cells of medullary differentiation, measuring 150~500 nm in average diameter. Cortical tumor element showed corresponding ultrastructural features. These results indicate that this is a case of adrenal cortical tumor with features of neuroendocrine differentiation.

Perioperative management of adrenal cortical tumor in children / 重庆医科大学学报

Objective:To evaluate the understanding and success rate in diagnosis and treatment of adrenal cortical tumor in children.Methods:6 cases were analyzed retrospectively about diagnosis,treatment,pathomorphological changes and prognosis.Results:6 cases of adrenal cortical tumor were treated surgically,which include 4 of cases Cushings syndrome,4 cases developed HBP,in 1 case developed hyperglycemia and in another case had hypokalemia developed.1 patient died of Ⅲ AVB(atrial ventricle block,AVB) during operation.Conclusion:Early diagnosis and early surgical interventions are crucial for treatment.Perioperative management plays an important role in improving prognosis.