Surgery and chemotherapy for prepubertal and postpubertal females (purported stage I and stages II–IV)

A revised risk stratification was developed by the Malignant Germ Cell Tumor International Consortium (see Figure 4).[8]

Chemotherapy regimens with cisplatin (PEb) or carboplatin (JEb) have been used successfully in children.[9,13,14,20] BEP is a common regimen in young women with ovarian GCTs.[37,38] BEP differs from PEb with the addition of weekly bleomycin. This approach results in a high rate of cure and the preservation of menstrual function and fertility in most patients with nondysgerminomas.[32,35] For more information about the dosing schedules for BEP, PEb, and JEb, see Table 8.

In prepubertal females with purported stage I ovarian tumors (when strict surgical staging guidelines are not followed) surgery followed by chemotherapy (four cycles of PEb) is an appropriate treatment choice and results in EFS and OS rates of 95%.[13,14]

In postpubertal females with purported stage I ovarian tumors, chemotherapy after resection remains the standard treatment. In postpubertal females, the strategy of observation after surgery has not been established and is under investigation in the AGCT1531 (NCT03067181) trial.

In prepubertal and postpubertal females with stages II, III, or IV ovarian tumors, surgery and chemotherapy are considered standard treatments. Surgery and chemotherapy with four to six cycles of standard PEb is used to treat younger (prepubertal) girls,[13,14] and BEP is used to treat postpubertal girls.[37,38] Patients with normalization of tumor markers undergo imaging after four cycles of PEb, and any residual tumor is resected. Patients with residual viable tumor after surgery are considered refractory.

Alternatively, surgery and chemotherapy with four to six cycles of JEb is a treatment option (as demonstrated in one study in which all patients were younger than 15 years).[9]