AGCTs vary from tiny lesions to huge masses with a mean diameter of about 13 cm. AGCTs are bilateral in only 2 % of cases. The external surface may be smooth or bosselated. The cut surface is often solid and sometimes partly solid and partly cystic. Hemorrhage and necrosis are common [16].

The tumor cells grow in a variety of patterns, including microfollicular, macrofollicular, trabecular, insular, tubular, diffuse, moiré silk, and gyriform. The microfollicular variant, the most easily recognized, is characterized by multiple small rounded spaces formed by cystic degeneration in small aggregates of granulosa cells, containing eosinophilic PAS-positive material and often fragments of nuclear debris or pyknotic nuclei. These spaces, known as Call–Exner bodies, are found in only 30–50 % of tumors [17].

JGCTs’ appearance is similar to the adult variant.

JGCT is typically a solid cellular neoplasm, with focal follicle formation. The follicles are of variable sizes and shapes but generally don’t reach the large size of the follicles in the macrofollicular variant of adult granulosa cell tumor. Their lumens contain basophilic or eosinophilic fluid. Variable layers of granulosa cells line the follicles and occasionally surrounded by mantle of theca cells. The neoplastic granulosa cells have abundant eosinophilic and/or vacuolated cytoplasm and rounded hyperchromatic nuclei. Nuclear grooves are rare. Nuclear atypia in JGCTs varies from minimal to marked. The mitotic rate is also variable but is generally higher than adult granulosa cell tumor.

SLCTs are variable in dimension but frequently are smaller than 10 cm. They mostly are solid, firm, encapsulated, and lobulated masses, typically yellow or tan in color. They are typically unilateral.

SLCTs are derived from mesenchyme and sex cords, which regroup histologically all the embryonic phases of testicular development, from an undifferentiated cord to well-differentiated Sertoli tubes. These tumors contain variable proportions of sertolian and leydigian elements. Tumors with only a sertolian component (Sertoli tumors) belong to the benign group. Tumors containing both types are classified into three groups: (1) benign differentiated forms (androgenic, secretory in 60 % of the cases), (2) intermediate differentiation (immature Sertoli cells), and (3) poorly differentiated forms (sarcomatoid or retiform).

In the forms with poor or intermediate differentiation (primarily epithelial or mesenchymatous), it is possible to see heterogeneous elements. In the largest series of SLCTs with greater than 200 tumors, 18 % contains glands and cysts lined by well-differentiated intestinal-type or gastric-type epithelium, 16 % has microscopic foci of carcinoid tumor, and 5 % has stromal heterologous elements, including islands of cartilage and/or areas of embryonal rhabdomyosarcoma [18]. Other heterologous elements that have been associated with SLCTs include hepatocyte-like cells, retinal tissue, neuroblastoma, and mucinous adenocarcinoma [19, 20]. Interestingly, tumors that contain heterologous or the retiform type are more frequently cystic.

SCTAT is a tumor composed of sex cord (Sertoli) cells arranged in simple and complex annular tubules. SCTATs which occur in conjunction with the PJS are usually multifocal, bilateral, and almost always very small tumorlets found incidentally in ovaries. In patients without the PJS, SCTAT is usually unilateral and presents as a solitary, large solid mass up to 33 cm in diameter.

SCTAT typically exhibits well-circumscribed, rounded or oval, epithelial islands made up of ring-shaped, lumenless tubules encircling glassy, acidophilic, PAS-positive, basement membrane-like material.

Surgery represents the most important therapeutic tool for patients with suspected SCSTs at early stage [32].

The staging system for SCSTs is generally adopted from that for epithelial ovarian cancer as originally defined by the International Federation of Gynecology and Obstetrics staging system (FIGO staging). However, the prognostic significance of certain features within the FIGO classification (such as positive cytology or ovarian surface involvement) has not been well defined for patients with SCSTs [33].

Surgical approach can be carried out through an open route that allows adequate visualization of the upper abdomen or, in selected cases, by laparoscopy or robotics. Minimally invasive surgery could be considered feasible and safe either for primary surgery or restaging procedures in selected patients [34].

The staging procedure includes infracolic omentectomy, biopsy of the diaphragmatic peritoneum, paracolic gutters, pelvic peritoneum, and peritoneal washings [33].

Many reports indicate that more than 90 % of these neoplasms are unilateral and confined to the ovary. Thus, a fertility-sparing surgery with unilateral salpingo-oophorectomy and staging seems to be reasonable in patients wishing to preserve their fertility, in the absence of extra-ovarian spread. For young women who can be offered conservative therapy, uterine curettage should be performed before surgery, because of the frequent association of GCT with endometrial hyperplasia (55 %) or endometrial adenocarcinoma (4–20 %).

Removal of the other ovary and total abdominal hysterectomy are recommended in postmenopausal women, and it is advisable at the conclusion of childbearing, though this issue is still controversial. Some authors reported a worse survival for patients undergoing fertility-sparing surgery, but this was related mostly to a higher stage of disease in the group analyzed [35].

Zanagnolo and Zhang published the two largest series about conservative treatment.

In the series of 63 cases published by Zanagnolo et al., a conservative surgical treatment was performed in 23 % of early stage tumors; none of them recurred, and five of 11 patients became pregnant after treatment [36].

Zhang et al., in a series of 110 patients, showed no statistical difference on survival between the conservative vs demolitive treatment (94.8 % and 94.9 %, p = 0.38) [37].

SLCTs are frequently low-grade malignancies, although occasionally a poorly differentiated variety may behave more aggressively. Unilateral salpingo-oophorectomy with preservation of the contralateral ovary and the uterus is now considered the adequate surgical treatment for patients in childbearing age.

There is no consensus about the role of systematic lymphadenectomy in SCSTs because of the very low incidence of retroperitoneal metastases in early stages [38, 39].

Thrall et al. confirmed previous reports and strengthened the principle that routine lymphadenectomy is unnecessary in the primary surgical management of SCSTs; they reviewed 47/87 patients, who had some nodal tissue examined as part of the initial or restaging procedure, and all examined nodes were negative [39–41].

In a more recent study of Shim et al., 578 patients were analyzed, and lymph node metastases were not detected in the 86 patients who underwent lymph node removal. This study confirms that the incidence of lymph node metastases in patients with clinical stage I and II SCSTs is low [34].

Node dissection should be carried out only in those cases with evidence of nodal abnormality.

In advanced stage SCSTs, surgery is necessary to establish a definitive pathological diagnosis, to perform staging, and to achieve optimal debulking. In patients with advanced stage disease or with bilateral ovarian involvement, abdominal hysterectomy and bilateral salpingo-oophorectomy should be performed with a careful surgical staging/cytoreduction. This includes a thorough exploration of abdominal cavity, washing for cytological analysis, multiple peritoneal biopsies, omentectomy, and pelvic and para-aortic lymph node sampling/dissection. Although no scientific evidence exists on the role of cytoreduction in these tumors, an effort should be made to remove all metastatic disease.

The majority of SCSTs are diagnosed at early stage (60–95 %). Given the indolent nature and the overall good prognosis, there are no data to support any kind of postoperative adjuvant treatment for patients with stage I who underwent an adequate staging procedure. Some authors suggest adjuvant therapy for stage IC with high mitotic index with preoperative tumor rupture [5, 35]. For SLCTs, postoperative adjuvant chemotherapy should be considered for those patients with stage I poorly differentiated tumors or with heterologous elements. Platinum-based chemotherapy is the treatment of choice. The most commonly used regimen is the BEP combination (bleomycin, etoposide, cisplatin) for three courses [42–44].

Alternative chemotherapy options include etoposide plus cisplatin, cyclophosphamide, doxorubicin and cisplatin, paclitaxel and carboplatin, or platinum agent alone.

In metastatic and recurrent GCTs, debulking surgery continues to be the most effective treatment.

The rarity of GCTs has made it impossible to conduct a well-designed randomized study assessing the value of postoperative therapy after debulking surgery.

Postoperative treatment should be considered in case of residual tumor after surgery [45–49].

Different approaches such as surgery followed by either chemotherapy or hormonal therapy and radiation have been associated with prolonged disease-free survival in some series [17, 35, 36, 42].