We report a case of a 4-year-old girl with an ovarian
steroid cell
tumor, not otherwise specified (SCT-NOS). She was admitted to the hospital with progressing
virilization and
Cushing's syndrome, which included abnormality of the perineum,
hirsutism,
hypertrichosis,
flushing of face,
hoarseness, and
weight gain. Blood testing showed a significantly increased
testosterone level and slightly increased
cortisol level. Computed tomography scan revealed an 8.0 × 5.0 × 5.0 cm
tumor of the right ovary. The patient underwent right
salpingo-oophorectomy, and pathological examination showed malignant potential. Three courses of
bleomycin,
etoposide, and
cisplatin were administered as postoperative
chemotherapy. After
tumor resection, her
testosterone decreased to undetectable levels. However, during the course of the treatment, the patient suffered from
adrenal insufficiency resulting in the need for
hydrocortisone replacement
therapy. Although SCT-NOS in childhood are typically benign, pathological findings should be carefully observed for potential
malignancy. In cases of
cortisol-producing SCT-NOS, serum levels should be monitored, and
hydrocortisone replacement
therapy should be considered before resection.