Management and prognosis of patients with ovarian sex cord tumor with annular tubules: a retrospective study.

Qiuhong Qian,Yan You,Jinghe Lang,Ming Wu,Keng Shen,Jiaxin Yang,Dongyan Cao,Jie Chen,Zhaohui Zhu

doi:10.1186/s12885-015-1277-y

Abstract

BackgroundOwing to the rarity of sex cord tumor with annular tubules (SCTAT), it is difficult to recognize SCTAT clinically and there is no standard treatment. The aim of our study was to investigate the treatment outcomes and prognosis of patients with ovarian SCTAT.MethodsA cohort of 13 patients with SCTAT diagnosed and treated in Peking Union Medical College Hospital was studied. Data on clinicopathological characteristics, treatment, and prognosis were retrospectively reviewed and analyzed.ResultsSCTAT accounted for 1.4% of ovarian sex cord stromal tumors, with an average onset age of 22.6 years. All patients presented with menstrual disturbances or isosexual precocity at disease onset. Initial surgery was unilateral salpingo-oophorectomy in 11 cases. Recurrence rate was 46.2%, and 38.5% of patients experienced multiple recurrences. The disease free interval gradually shortened with increasing numbers of recurrences. Recurrent tumors were mostly ipsilateral to the primary tumor and located in retroperitoneum. Surgery remained the main treatment for recurrent cases. Serum estradiol and progesterone levels usually elevated at disease onset, decreased dramatically after operation, and they elevated again with the development of recurrence. The median progression-free survival (PFS) was 97.8 months, and the 1-year and 5-year PFS were 92% and 67%, respectively. Five-year overall survival (OS) was 100%.ConclusionsUnilateral salpingo-oophorectomy is a feasible treatment for primary SCTAT cases with intact capsules and without PJS. Complete tumor resection is suggested for recurrent cases and long-term follow-up is strongly recommended. Despite the high risk of recurrence, SCTAT prognosis is relatively favorable.

Highlights

Owing to the rarity of sex cord tumor with annular tubules (SCTAT), it is difficult to recognize SCTAT clinically and there is no standard treatment
Clinical features Thirteen patients with SCTAT were identified from 936 sex cord-stromal tumor (SCST) cases admitted in Peking Union Medical College Hospital (PUMCH) at the same period, accounting for 1.4% of SCST cases
All patients had menstrual disturbances at disease onset, including seven patients presenting with amenorrhea, three patients presenting with prolonged menstrual bleeding, and three patients presenting with isosexual precocity at the age of 8, 6, and 5 years old, respectively

Summary

Introduction

Owing to the rarity of sex cord tumor with annular tubules (SCTAT), it is difficult to recognize SCTAT clinically and there is no standard treatment. The aim of our study was to investigate the treatment outcomes and prognosis of patients with ovarian SCTAT. Sex cord tumor with annular tubules (SCTAT) is a rare and distinctive ovarian sex cord-stromal tumor (SCST), accounting for approximately 2.3% of SCSTs [1]. The predominant component of this tumor has intermediate morphologic features between granulosa cell tumors and Sertoli cell tumors and focal differentiation into either of these tumor types may occur [2]. In 1970, Scully first described this distinctive tumor [3], and a series of case reports have since been published.

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