Borderline, germ cell and sex cord-stromal ovarian tumours: fertility-sparing surgery, BEP chemotherapy, tumour markers and FIGO staging

Also known as Borderline ovarian tumour · Ovarian germ cell tumour · Dysgerminoma · Yolk sac tumour · Immature teratoma · Granulosa cell tumour · Sertoli-Leydig cell tumour · Sex cord-stromal tumour

Exam-exhaustive FRANZCOG fellowship topic on the non-epithelial ovarian tumours: borderline ovarian tumours (serous and mucinous, the peritoneal implants distinction, fertility-sparing surgery and the recurrence-versus-malignant-transformation data), malignant ovarian germ cell tumours (dysgerminoma, yolk sac, immature teratoma — the BEP chemotherapy regimen with doses and the tumour markers), and the sex cord-stromal tumours (adult granulosa cell tumour with inhibin B and the late-recurrence pattern, Sertoli-Leydig with virilisation). FIGO ovarian staging applies throughout. Anchored to the FIGO 2021 ovarian cancer update (Berek), the ESMO 2018 non-epithelial ovarian cancer guideline (Ray-Coquard), the CNGOF borderline guidelines (Bourdel), the Taniskidi 2026 borderline systematic review, and the landmark GOG stromal trials. RANZCOG-primary, globally tagged.

high10 referencesUpdated 27 July 202612 min readVerification in progress

Practise this topic

8 MCQs

One-line fellowship answer

The non-epithelial ovarian tumours are three families that share a young population, a fertility-sparing surgical philosophy, and the FIGO ovarian staging. Borderline tumours (serous, mucinous) are proliferating epithelial lesions without destructive invasion; treat with fertility-sparing surgery, watch for recurrence (cystectomy roughly doubles it) and rare malignant transformation. Malignant germ cell tumours (dysgerminoma, yolk sac, immature teratoma) are cured by conservative surgery plus the BEP regimen (etoposide 100 mg/m²/day and cisplatin 20 mg/m²/day for 5 days, bleomycin 30 IU days 1, 8, 15, every 3 weeks for 3 cycles), tracked by AFP, hCG and LDH. Sex cord-stromal tumours (granulosa, Sertoli-Leydig) are indolent but recur late; granulosa is followed lifelong with inhibin B, Sertoli-Leydig presents with virilisation.^[1]^[2]

A 19-year-old woman presents to emergency with acute left iliac fossa pain and a 12 cm solid-cystic adnexal mass. Her serum alpha-fetoprotein is markedly elevated. This is not "a big ovarian cyst for the general surgeons" — it is a malignant ovarian germ cell tumour until proven otherwise, and the operation that cures her is conservative surgery that preserves her uterus and the other ovary, followed by BEP chemotherapy. The three families of non-epithelial ovarian tumour share one principle: young women, fertility-sparing surgery, and curable disease. This topic is where you stop reflexively reaching for a hysterectomy and bilateral salpingo-oophorectomy.^[1]^[2]

Overview and definition

The non-epithelial ovarian tumours are grouped because they are not high-grade serous carcinomas and they behave, and are managed, differently. They fall into three families:^[1]^[2]

Borderline ovarian tumours (BOT) — proliferating epithelial tumours with atypical proliferation but no destructive stromal invasion. They comprise about 10 to 15 percent of epithelial ovarian tumours and present at a younger mean age (approximately 40 to 50 years) than invasive carcinoma.
Malignant ovarian germ cell tumours (MOGCT) — tumours of primordial germ cell origin (dysgerminoma, yolk sac, immature teratoma and others). About 5 percent of malignant ovarian tumours; mean age in the early 20s; largely curable.
Sex cord-stromal tumours (SCST) — tumours of the ovarian sex cord and stromal cells (granulosa, Sertoli-Leydig, fibroma-thecoma). About 5 to 7 percent of malignant ovarian tumours; the adult granulosa cell tumour is the most common.^[1]^[2]

All three are staged with the FIGO ovarian system, applied at laparotomy/laparoscopy.^[1]

Why this matters — the burden in numbers

over 95%Germ cell tumour 5-year survival (Stage I)with fertility-sparing surgery and BEP where indicated

16 to 23%Granulosa cell tumour recurrencetypically late — decades later, peritoneal

about 1% overallBorderline malignant transformationbut rises to about a fifth after recurrence

31% vs 13%BOT recurrence after cystectomy vs oophorectomycystectomy roughly doubles recurrence

^[1] ^[4] ^[9]

References10Show

[1]Berek JS, Renz M, Kehoe S, Kumar L, Friedlander M Cancer of the ovary, fallopian tube, and peritoneum: 2021 update. Int J Gynaecol Obstet, 2021.PMID 34669199
[2]Ray-Coquard I, Morice P, Lorusso D, Prat J, Oaknin A, Pautier P, et al. Non-epithelial ovarian cancer: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol, 2018.PMID 29697741
[3]Bourdel N, Huchon C, Abdel Wahab C, Azaïs H, Bendifallah S, Bolze PA, et al. Borderline ovarian tumors: French guidelines from the CNGOF. Part 2. Surgical management, follow-up, hormone replacement therapy, fertility management and preservation. J Gynecol Obstet Hum Reprod, 2021.PMID 33144266
[4]Taniskidi A, Giannas E, Lekka S, Taniskidi A, Lecoyte A, Brincat M, et al. Recurrence and Malignant Transformation After Borderline Ovarian Tumors: A Systematic Review and Meta-analysis. Anticancer Res, 2026.PMID 42049343
[5]Boyraz G, Durmus Y, Cicin I, Kuru O, Bostanci E, Comert GK, et al. Prognostic factors and oncological outcomes of ovarian yolk sac tumors: a retrospective multicentric analysis of 99 cases. Arch Gynecol Obstet, 2019.PMID 30982145
[6]Homesley HD, Bundy BN, Hurteau JA, Roth LM Bleomycin, etoposide, and cisplatin combination therapy of ovarian granulosa cell tumors and other stromal malignancies: A Gynecologic Oncology Group study. Gynecol Oncol, 1999.PMID 10021290
[7]Brown J, Brady WE, Schink J, Van Le L, Leitao M, Yamada SD, et al. Efficacy and safety of bevacizumab in recurrent sex cord-stromal ovarian tumors: results of a phase 2 trial of the Gynecologic Oncology Group. Cancer, 2014.PMID 24166194
[8]Brown J, Sood AK, Deavers MT, Milojevic L, Gershenson DM Patterns of metastasis in sex cord-stromal tumors of the ovary: can routine staging lymphadenectomy be omitted? Gynecol Oncol, 2009.PMID 19162310
[9]Yasukawa M, Matsuo K, Matsuzaki S, Dainty LA, Sugarbaker PH Management of recurrent granulosa cell tumor of the ovary: Contemporary literature review and a proposal of hyperthermic intraperitoneal chemotherapy as novel therapeutic option. J Obstet Gynaecol Res, 2021.PMID 33103312
[10]Javadi S, Ganeshan DM, Jensen CT, Iyer RB, Bhosale PR Comprehensive review of imaging features of sex cord-stromal tumors of the ovary. Abdom Radiol (NY), 2021.PMID 33725145

Borderline, germ cell and sex cord-stromal ovarian tumours: fertility-sparing surgery, BEP chemotherapy, tumour markers and FIGO staging

high10 referencesUpdated 27 July 202612 min readVerification in progress

Practise this topic

8 MCQs

Overview and definition

The non-epithelial ovarian tumours are grouped because they are not high-grade serous carcinomas and they behave, and are managed, differently. They fall into three families:^[1]^[2]

Borderline ovarian tumours (BOT) — proliferating epithelial tumours with atypical proliferation but no destructive stromal invasion. They comprise about 10 to 15 percent of epithelial ovarian tumours and present at a younger mean age (approximately 40 to 50 years) than invasive carcinoma.

Malignant ovarian germ cell tumours (MOGCT) — tumours of primordial germ cell origin (dysgerminoma, yolk sac, immature teratoma and others). About 5 percent of malignant ovarian tumours; mean age in the early 20s; largely curable.

Sex cord-stromal tumours (SCST) — tumours of the ovarian sex cord and stromal cells (granulosa, Sertoli-Leydig, fibroma-thecoma). About 5 to 7 percent of malignant ovarian tumours; the adult granulosa cell tumour is the most common.^[1]^[2]