SCTAT has low malignant potential with late recurrence. It was first described by Scully in 1970 as a specific form of ovarian neoplasm, characterized by the coexistence of simple and complex annular tubules [1]. The predominant component of this tumor has intermediate morphologic features between granulosa cell tumors and Sertoli cell tumors with possible focal differentiation into either of these tumor types.

The classification of SCTAT is controversial. In the past, SCTAT have been placed in the category of unclassified sex cord-stromal neoplasms. In the recently published classification of tumors of female reproductive organs by WHO, SCTAT was classified as pure sex cord neoplasms [5].

Since Scully described it, numerous case reports and series have contributed to the better understanding of this unusual morphological entity. There are two forms of SCTAT, which differ in terms of association to PJS, the age of onset, initial presentation and its nature. Firstly, SCTAT related to PJS are usually benign with a genetic predisposition, in which the tumors usually showed small, bilateral, multifocal, and calcified lesions [6]. Secondly, sporadic SCTAT are not associated to PJS, in which the tumors generally present as large, unilateral, solitary, and non-calcified neoplasms. Based on the tumor characteristics and the absence of PJS features, our patient likely harbored sporadic SCTAT.

Recurrence of sporadic SCTAT ranges from 8.5% to 46.2% [7]. The latency period ranges from 3 months to 20 years. Most recurrence occurs at pelvic, para-aortic and supraclavicular lymph nodes and metastasize to retroperitoneum, parietal and visceral peritoneum, lung, liver, and kidney [10].

In our reported case, the disease spread through retroperitoneum 16 years after initial diagnosis.

The majority of SCTAT arise in reproductive age patients [2]. Rare pediatric cases have been reported [8,9].

The most frequent clinical manifestations of SCTAT relate to estrogen-progesterone secretion like: menorrhagia, postmenopausal bleeding, precocious puberty, and sterility. In the present case, the patient was in reproductive age group and though did not have any clinical features of high estrogen production; she had symptoms related to the voluminous retroperitoneal mass.

SCTAT’s have been rarely reported in literature. It remains a clinical complexity and preoperative diagnosis is difficult. Although most of the SCTAT are picked up by advanced imaging, the diagnosis is ultimately made by pathological examination.

In both syndromic and non-syndromic cases, the distinctive histologic findings of SCTAT include the presence of simple and complex annular tubules encircling central hyaline cores, and antipodal arrangement of the nucleus 5, as it was objectified with our patient. Nuclear atypia and mitotic figures are otherwise not common features. In PJS lesions, extensive calcifications may be seen. On immunochemistry, the tumor cells are typically positive for calretinin, inhibine, and WT1. On the other hand, they are typically negative for EMA and CD10 [5].

A number of sex cord-stromal tumours including granulosa cell tumour, Sertoli–Leydig cell tumour, and gonadoblastoma should be taken into account for differential diagnosis of SCTATs [8]. However, these tumors may share some of the components that are found in SCTATs.

Given its rarity, there is no standardized management algorithm worldwide, and the surgical total resection (with preservation of fertility if possible) is known to be the only way to influence positively the prognosis and save patient lives [10]. The role of adjuvant treatment is reported for disease control, but preventing recurrence is still unproven [9].

In the present case, hysterectomy with bilateral salphingo-oopherectomy was performed (stage 1) and was apparently well on 6 months follow-up period for after which she was lost to follow-up.

The prognosis is excellent because most of patients have stage I disease at presentation as our patient. But Long-term follow-up is mandatory in such type of tumor because of the high risk of recurrence [7].