The clinical practice guidelines on ovarian cancer were released by the European Society for Medical Oncology on April 25, 2018.
Diagnostic workup should include pelvic ultrasound, abdominopelvic CT scan and chest x-ray, and PET scan in selected cases.
In young adult patients, hCG, α-FP, LDH and inhibin B levels, full blood count, and liver and renal function tests should be carried out.
In case of suspected gonadoblastomas, a preoperative karyotype should be obtained on all premenarche girls.
Histologic second opinion by an expert pathologist should always be considered. Diagnosis can be made on conventional histologic material.
Neoplasms of pure ovarian stroma: in morphologically ambiguous cases, an immunopanel of inhibin alpha, calretinin, and FOXL2, plus mutational analysis for FOXL2 (402C-G), is useful to confirm adult granulosa cell tumors (AGCTs).
A surgical approach can be carried out through open route or, in selected cases, by laparoscopy and robotics approaches, thereby avoiding tumor rupture during surgery.
A careful examination of the abdominal cavity is required.
The staging procedure includes infracolic omentectomy, biopsy of the diaphragmatic peritoneum, paracolic gutters, pelvic peritoneum, and peritoneal washings (macroscopic stage I disease).
Unilateral salpingo-oophorectomy with preservation of the contralateral ovary and the uterus is considered an adequate surgical treatment for patients with GCTs. This should be considered even in advanced disease because of the sensitivity of the tumor to ChT (chemotherapy). No systematic ovarian biopsy is necessary when the contralateral ovary is macroscopically normal.
Given the very high chemosensitivity of GCTs, potential nodal metastasis should be cured by adjuvant ChT in these patients.
Nodal dissection should be carried out only if there is evidence of nodal abnormality.
For sex cord stromal tumors (SCSTs), retroperitoneal evaluation is not mandatory.
An endometrial curettage must be carried out to rule out concomitant uterine cancers in patients with granulosa cell tumors.
Conservative surgery is also an acceptable approach in young patients with stage I SCSTs.
In postmenopausal women and in patients with advanced-stage disease or with bilateral ovarian involvement, abdominal hysterectomy and bilateral salpingo-oophorectomy should be carried out with careful surgical staging for SCST.
Stage IA pure dysgerminoma should be treated with surgery only.
Patients with stage IA grade 1 immature teratoma do not require further adjuvant ChT after adequate surgical staging.
Adjuvant ChT in stage IA-IC G2-G3 immature teratoma, stage IA-IB YST with negative postoperative tumor markers, and IB-IC dysgerminomas is recommended, but active surveillance is an option.
5-day BEP (bleomycin, etoposide, cisplatin) is the most used regimen.
The close surveillance schedule involves regular clinical review with clinical examination, radiologic imaging including abdomen-pelvic intravaginal sonography at regular intervals, and the monitoring of tumor markers to detect relapse over a period of 10 years, with a gradual increase of the interval between clinical appointments.
Fertility-sparing surgery should be considered also in advanced stages. The aim of surgery is to remove as much gross tumor as possible; however, the procedure should be moderated to avoid delays in postoperative ChT and long-term morbidity.
In postmenopausal women with advanced-stage disease or with bilateral ovarian involvement, abdominal hysterectomy and bilateral salpingo-oophorectomy could be carried out with careful surgical staging.
Platinum-based regimens are the treatment of choice, with the BEP regimen being the most widely used--generally, 3 cycles of 5-day BEP regimen in completely resected disease, and 4 cycles (bleomycin should be omitted to reduce the risk of lung toxicity after the third cycle) for patients with macroscopic residual disease.
In patients previously treated with platinum, with platinum-sensitive relapse (progression >4–6 weeks after completion of ChT), combinations with platinum should be considered.
Patients resistant to a cisplatin-based combination may receive VAC or paclitaxel/gemcitabine or gemcitabine/oxaliplatin as salvage therapy.
HDCT for recurrent ovarian GCTs may result in durable and prolonged remissions.
Any resectable residual disease should be removed, particularly for patients with normal serum marker and for patients with immature teratoma in order to avoid the growing teratoma syndrome.
Stage IA granulosa cell tumor disease has an excellent prognosis after surgery alone and does not require adjuvant therapy.
Adjuvant therapy should be considered for juvenile granulosa tumor stage IC patients or for AGCT stage IC2-IC3 patients. In these cases, platinum-based ChT is the treatment of choice.
For Sertoli-Leydig cell tumors (SLCTs), postoperative adjuvant ChT should be considered for patients with stage I poorly differentiated or heterologous elements (mesenchymal type).
BEP is the most commonly used regimen. Alternative ChT options include paclitaxel and carboplatin, EP, CAP, or platinum agent alone.
Debulking surgery remains the most effective treatment of advanced or recurrent granulosa cell tumor.
Platinum-based ChT is currently used for patients with advanced-stage SCSTs or recurrent disease.
BEP regimen for 3 cycles or 6 cycles of carboplatin/paclitaxel is recommended for postoperative ChT and patients with recurrent SCSTs.
Patients with steroid cell tumors that are pleomorphic, large, at an advanced stage, or with an increased mitotic count should be treated with additional postoperative platinum-based ChT, either BEP (if not previously used) or a taxane–platinum combination.
Response to GnRH agonists, tamoxifen, progestin, and AIs has been reported and could be an interesting option specifically for adult GCT.
For patients with persistent SLCTs, adjuvant ChT should be considered.
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Cite this: Ovarian Cancer Clinical Practice Guidelines (2018) - Medscape - May 11, 2018.