Histological changes of non-Peutz-Jegher syndrome associated ovarian sex cord tumor with annular tubules in childhood

Ovarian sex cord stromal tumor is a relatively rare subtype of ovarian neoplasms, consisting of only 8% of all primary ovarian neoplasms, among which sex cord tumor with annular tubules (SCTAT) accounts for only 6% of sex cord stromal tumors. The majority of patients with SCTAT are women at reproductive age. Roughly one-third of the patients have Peutz-Jeghers syndrome (PJS), and in cases without PJS, about 15%-20% SCTAT tend to be clinical malignant. Herein we report 3 cases of non-PJS associated ovarian SCTAT onset in childhood, among which, two cases had recurrence and metastasis. And we summarize the pathologic manifests and report our discovery of the pathologic difference between primary and recurrent/metastatic SCTAT in childhood.