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Non-epithelial ovarian cancer

Guidelines

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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Screening and diagnosis

Indications for screening: as per USPSTF 2018 guidelines, avoid screening for ovarian cancer in asymptomatic females.
D
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Diagnostic investigations

Diagnostic imaging: as per ESMO 2018 guidelines, 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

Diagnostic procedures

Biopsy and pathology: as per ESMO 2018 guidelines, 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

Medical management

General principles
As per ESMO 2018 guidelines:
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

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  • Adjuvant chemotherapy (indications)

  • Adjuvant chemotherapy (regimens)

  • Hormone replacement therapy

Therapeutic procedures

Pelvic radiotherapy: as per ESMO 2018 guidelines, 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

Surgical interventions

General principles: as per ESMO 2018 guidelines, 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

Specific circumstances

Patients wishing to preserve fertility: as per ESMO 2018 guidelines, consider offering oocyte cryopreservation in patients scheduled to receive chemotherapy.
C
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Follow-up and surveillance

Assessment of treatment response: as per ESMO 2018 guidelines, recognize that serum tumor markers (hCG, α-fetoprotein, LDH, CA-125, and inhibin B) can correlate with tumor response during chemotherapy.
B
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  • Follow-up