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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).
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Guidelines

1.Screening and diagnosis

Indications for screening: avoid screening for ovarian cancer in asymptomatic females.
D
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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).
B

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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.
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Offer a multimodal treatment approach including chemotherapy
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, radical surgery
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, high-dose chemotherapy
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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.
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.
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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.
C
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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.
B
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  • Follow-up