조직학적 악성소견을 보이는 소아 부신피질종양 - 1 증례 보고 및 악성 진단기준에 관한 고찰 -

Abstract

Adrenal cortical neoplasm, especially carcinoma, is extremely rare in pediatric patients. We describe here a rare pediatric case of adrenal cortical neoplasm. A 2-year-old girl presented with an enlarged clitoris. The other physical findings and laboratory tests were nonspecific. The magnetic resonance imaging showed a 4 cm-sized heterogeneously enhancing soft tissue mass with calcification in the left adrenal gland. The mass was removed by laparoscopic operation. Grossly, several fragments of reddish tan soft tissue were present, and they weighed 19 gm in total. Microscopically, there were capsular invasion, diffuse/solid growth pattern with focal necrosis, high cellularity, cytoplasmic eosinophilia, marked nuclear pleomorphism, high N/C ratio, prominent nucleoli, atypical mitotic figures and calcifications, which all suggested adrenal cortical neoplasm of histologic malignancy. On immunohistochemistrical staining, there were positive reactivities to pancytokeratin, cytokeratin 7/20, CEA, inhibin and p53. The Ki-67 labeling index was about 6%. All these findings were indicative of adrenal cortical neoplasm of histologic malignancy.