Guideline sources The following summarized guidelines for the evaluation and management of non-epithelial ovarian cancer are prepared by our editorial team based on guidelines from the European Society of Medical Oncology (ESMO 2018) and the U.S. Preventive Services Task Force (USPSTF 2018). 1 2 Calculator Calculator Calculator Eastern Cooperative Oncology G FIGO staging for ovarian cancer Karnofsky Guidelines 1. Screening and diagnosis https://web.pathway.md/diseases/recllj1OAFsheVhj8 1/5 6/30/23, 1:17 AM Non-epithelial ovarian cancer Pathway Indications for screening: avoid screening for ovarian cancer in asymptomatic females. D 2. Diagnostic investigations Diagnostic imaging: obtain pelvic ultrasound, abdominopelvic CT, CXR, and positron emission tomography in the diagnostic work-up of selected patients with non-epithelial ovarian cancer (germ cell tumor). B Laboratory testing: Obtain the following tests in young adult patients with non-epithelial ovarian cancer: hCG AFP LDH inhibin B CBC LFTs renal function tests. Show 2 more 3. Diagnostic procedures Biopsy and pathology: perform infracolicomentectomy, a biopsy of the diaphragmatic peritoneum, paracolic gutters, pelvic peritoneum, and peritoneal washings (macroscopic stage I disease) for staging. Show 3 more Retroperitoneal lymph node assessment: do not obtain a routine retroperitoneal evaluation in patients with sex cord-stromal tumor. D Endometrial curettage: perform endometrial curettage to rule out concomitant uterine cancers in patients with granulosa cell tumors. B 4. Medical management General principles: Attempt treating patients with small cell carcinoma of the ovary, hypercalcemic type in a more homogeneous way through national and international networks. B Offer a multimodal treatment approach including chemotherapy B , radical surgery B , high- dose chemotherapy B and radiotherapy in patients with all stages of small cell carcinoma of the ovary, hypercalcemic type. B Adjuvant chemotherapy (indications): offer adjuvant chemotherapy in patients with germ cell tumor with potential nodal metastasis. B https://web.pathway.md/diseases/recllj1OAFsheVhj8 2/5 6/30/23, 1:17 AM Non-epithelial ovarian cancer Pathway Show 8 more Adjuvant chemotherapy (regimens): administer platinum-based regimens as the treatment of choice, with bleomycin/etoposide/cisplatin regimen being the most widely used. Administer bleomycin/etoposide/cisplatin for 5 days in patients with early-stage germ cell tumor. B Show 9 more Hormone replacement therapy: Consider offering HRT in patients with germ cell tumors. B Do not use HRT in patients with sex cord-stromal tumors. D 5. Therapeutic procedures Pelvic radiotherapy: consider offering postoperative pelvic radiotherapy, either concurrently or sequentially to high-dose chemotherapy and ASCT. in patients with early or advanced small cell carcinoma of the ovary, hypercalcemic type. C 6. Surgical interventions General principles: use an open surgical approach or, in selected cases, laparoscopic and robotic approaches, thereby avoiding tumor rupture during surgery. Show 6 more Conservative fertility-sparing surgery: consider performing unilateral salpingo-oophorectomy with preservation of the contralateral ovary and the uterus in patients with germ cell tumor, even in advanced disease because of the sensitivity of the tumor to chemotherapy. C Show 2 more Abdominal hysterectomy and bilateral salpingo-oophorectomy: Perform abdominal hysterectomy and bilateral salpingo-oophorectomy with careful surgical staging for sex cord-stromal tumors in postmenopausal patients and in patients with advanced- stage disease or with bilateral ovarian involvement. B Consider performing abdominal hysterectomy and bilateral salpingo-oophorectomy with careful surgical staging in postmenopausal patients with advanced-stage disease or with bilateral ovarian involvement. B 7. Specific circumstances Patients wishing to preserve fertility: consider offering oocyte cryopreservation in patients scheduled to receive chemotherapy. C Show 2 more 8. Follow-up and surveillance https://web.pathway.md/diseases/recllj1OAFsheVhj8 3/5 6/30/23, 1:17 AM Non-epithelial ovarian cancer Pathway Assessment of treatment response: recognize that serum tumor markers (hCG, AFP, LDH, cancer antigen 125, and inhibin B) can correlate with tumor response during chemotherapy. B Show 2 more Follow-up: obtain regular clinical review with clinical examination, radiological imaging, including abdominopelvic intravaginal ultrasound at regular intervals and monitoring of tumor markers to detect relapse over a period of 10 years, with a gradual increase of the interval between clinical appointments for the close surveillance of patients with early-stage germ cell tumor. B Show 3 more Clinical findings Symptoms Past medical history Abdominal pain Adnexal torsion Amenorrhea Abdominal exam Bloating Constipation Abdominal distension Deep voice Ascites Hirsutism Integument exam Irregular periods Pelvic pain Virilization Urinary frequency Urinary retention Imaging findings Vaginal bleeding Findings of small bowel obstruction Vital signs Pleural effusion Hypertension Gynecologic exam Adnexal mass Uterine bleeding Lab findings serum CA 19-9 serum cancer antigen 125 References 1. Ray-Coquard I, Morice P, Lorusso D et al. Non-epithelial ovarian cancer: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2018 Oct 1;29 Suppl 4):iv1-iv18. Open https://web.pathway.md/diseases/recllj1OAFsheVhj8 4/5 6/30/23, 1:17 AM Non-epithelial ovarian cancer Pathway 2. US Preventive Services Task Force, Grossman DC, Curry SJ et al. Screening USPSTF for Ovarian Cancer: US Preventive Services Task Force Recommendation Statement. JAMA. 2018 Feb 13;319 6 588 594. Open https://web.pathway.md/diseases/recllj1OAFsheVhj8 5/5