2021 Volume 57 Issue 6 Pages 959-964
Sex cord tumor with annular tubules (SCTAT) is a rare borderline malignant tumor compared with other sex cord-stromal tumors. This tumor often produces female sex hormones and is known to complicate Peutz-Jeghers syndrome (PJS). The patient was a girl aged 9 years and 6 months who was brought to our hospital presenting with chief complaints of breast enlargement and atypical genital bleeding. An approximately 11-cm serous cystic lesion was present in the left ovary and the serum estradiol (E2) level was high. The lesion temporarily shrank and the E2 level also decreased during the course of observation, but the lesion re-enlarged thereafter. Suspecting a reactive ovarian cyst, we performed partial ovarian cystectomy and the lesion was histopathologically diagnosed as SCTAT. No standard treatment for SCTAT has been established. In this patient, to preserve fertility, adnexectomy was performed only on the affected side seven months after partial ovarian cystectomy. Since the patient’s case was not complicated by PJS and it has been reported that about 20% of noncomplicated cases follow a malignant course, a strict course of observation will be necessary hereafter.
