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Non-epithelial ovarian cancer

Key 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.Screening and diagnosis

Indications for screening: avoid screening for ovarian cancer in asymptomatic females.
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2.Diagnostic investigations

Diagnostic imaging: obtain pelvic ultrasound, abdominopelvic CT, CXR, and PET in the diagnostic work-up of selected patients with non-epithelial ovarian cancer (germ cell tumor).

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  • Laboratory testing

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.
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  • Retroperitoneal lymph node assessment

  • Endometrial curettage

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.
Offer a multimodal treatment approach including chemotherapy
, radical surgery
, high-dose chemotherapy
and radiotherapy in patients with all stages of small cell carcinoma of the ovary, hypercalcemic type.

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  • Adjuvant chemotherapy (indications)

  • Adjuvant chemotherapy (regimens)

  • Hormone replacement therapy

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.

6.Surgical interventions

General principles: use an open surgical approach or, in selected cases, laparoscopic and robotic approaches, thereby avoiding tumor rupture during surgery.
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  • Conservative fertility-sparing surgery

  • Abdominal hysterectomy and bilateral salpingo-oophorectomy

7.Specific circumstances

Patients wishing to preserve fertility: consider offering oocyte cryopreservation in patients scheduled to receive chemotherapy.
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8.Follow-up and surveillance

Assessment of treatment response: recognize that serum tumor markers (hCG, AFP, LDH, CA-125, and inhibin B) can correlate with tumor response during chemotherapy.
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  • Follow-up