[Durgical](https://durgical.com/en/) › [DurgiPedia](https://durgical.com/en/durgipedia/) › [Conditions](https://durgical.com/en/conditions/) › Ovarian germ-cell and sex cord–stromal tumours

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# Ovarian germ-cell and sex cord–stromal tumours

Ovarian tumours that start from egg cells or hormone-making cells. They hit younger women, may make hormones, and most are curable.

Updated 2026-11-01

## Structures involved

Tap one to see what it is and open it in 3D.

- [Ovaries](https://durgical.com/en/anatomy/ovary/)
- [Suspensory ligament of ovary](https://durgical.com/en/anatomy/suspensory-ligament-of-ovary/)
- [Ovarian ligament](https://durgical.com/en/anatomy/proper-ovarian-ligament/)
- [Endometrium](https://durgical.com/en/anatomy/endometrium/)
- [Rectouterine pouch](https://durgical.com/en/anatomy/rectouterine-pouch/)

## Why it happens

- [Naming tumours: -oma, carcinoma, sarcoma, and the misleading names](https://durgical.com/en/general-pathology/naming-tumours-oma-carcinoma-sarcoma-and-the-misleading-names/)
- [Tumour markers: serum and tissue, and what each one is for](https://durgical.com/en/general-pathology/tumour-markers-serum-and-tissue-and-what-each-one-is-for/)
- [Ovary: follicles, ovulation and corpus luteum](https://durgical.com/en/histology/ovary-follicles-ovulation-and-corpus-luteum/)

## What it is

Non-epithelial ovarian neoplasms: germ-cell tumours arising from primordial germ cells (mature cystic teratoma, dysgerminoma, yolk sac tumour, immature teratoma, choriocarcinoma) and sex cord–stromal tumours arising from the cells that surround the oocyte and ovarian stroma (granulosa cell tumour, thecoma, fibroma, Sertoli-Leydig cell tumour).

## Who gets it

Germ-cell tumours make up about a fifth to a quarter of ovarian tumours but only a small fraction of ovarian cancers; they mainly affect girls and women under 30. Mature cystic teratoma (dermoid) is the most common ovarian neoplasm in women of reproductive age. Sex cord–stromal tumours are rarer; adult granulosa cell tumour typically appears around menopause.

## Causes

- Germ-cell tumours: neoplastic transformation of primordial germ cells
- Sex cord–stromal tumours: granulosa, theca, Sertoli, Leydig cells or fibroblasts
- FOXL2 mutation in adult granulosa cell tumour; DICER1 in some Sertoli-Leydig tumours
- Gonadal dysgenesis with Y chromosome material (gonadoblastoma and dysgerminoma)

## Risk factors

- Young age (germ-cell tumours)
- Gonadal dysgenesis with a Y chromosome (Swyer syndrome)
- Peutz-Jeghers syndrome (sex cord tumour with annular tubules)
- DICER1 syndrome

## Pathophysiology

Germ-cell tumours reproduce stages of embryonic development: dysgerminoma resembles primitive germ cells (the ovarian counterpart of seminoma); teratomas differentiate into tissues of all three germ layers (hair, teeth, [skin](https://durgical.com/en/anatomy/skin/), thyroid); yolk sac tumour secretes alpha-fetoprotein; choriocarcinoma secretes hCG. Sex cord–stromal tumours keep their hormonal role: granulosa cell tumours and thecomas produce oestrogen (endometrial hyperplasia, precocious puberty, postmenopausal bleeding) and inhibin; Sertoli-Leydig tumours produce androgens (virilisation). Large tumours act as a lead point for ovarian torsion.

## Classification

- Benign germ-cell: mature cystic teratoma (dermoid); struma ovarii (thyroid tissue)
- Malignant germ-cell: dysgerminoma (LDH), yolk sac tumour (AFP, Schiller-Duval bodies), immature teratoma, choriocarcinoma (hCG)
- Oestrogen-producing stromal: granulosa cell tumour (inhibin, Call-Exner bodies), thecoma
- Androgen-producing stromal: Sertoli-Leydig cell tumour
- Fibroma (Meigs syndrome: fibroma, ascites, pleural effusion)

## Symptoms and signs

- Pelvic or abdominal mass, often large and unilateral
- Abdominal pain, sometimes acute from torsion or rupture
- Abdominal distension
- Precocious puberty or postmenopausal bleeding (oestrogen-producing tumours)
- Hirsutism, deepening voice, clitoromegaly, amenorrhoea (androgen-producing tumours)
- Hyperthyroid symptoms (struma ovarii, rare)

## Red flags

- Sudden severe pelvic pain with vomiting (torsion)
- Rapidly growing solid adnexal mass in a girl or young woman
- Rapid-onset virilisation
- Postmenopausal bleeding with an adnexal mass
- Ascites with pleural effusion

## Diagnosis

**Clinical**
Adnexal mass in a young woman, or hormonal signs (precocious puberty, bleeding, virilisation) combined with a mass.

**Tests**
Tumour markers guide the diagnosis: hCG (choriocarcinoma, and pregnancy test), AFP (yolk sac tumour), LDH (dysgerminoma), inhibin (granulosa cell tumour), testosterone (Sertoli-Leydig); CA-125 for comparison. Karyotype in a girl with primary amenorrhoea and dysgerminoma. Final diagnosis is histological after surgery.

**Imaging**
Transvaginal or pelvic ultrasound first; a dermoid shows fat, calcification and hair echoes. CT or MRI for staging of malignant tumours. Endometrial evaluation in oestrogen-producing tumours.

## Differential diagnosis

- Functional ovarian cyst
- Epithelial ovarian cancer
- Endometrioma
- Ectopic pregnancy and tubo-ovarian abscess
- Polycystic ovary syndrome or adrenal tumour (as cause of virilisation)

## Goal of treatment

Remove the tumour, stage it, preserve fertility whenever possible in young women, and achieve cure with chemotherapy when needed.

## Treatment

**Surgical**
Benign dermoid: ovarian cystectomy preserving the [ovary](https://durgical.com/en/anatomy/ovary/), usually by laparoscopy.

**Surgical**
Malignant germ-cell or stromal tumours: fertility-sparing unilateral salpingo-oophorectomy with surgical staging in young women; hysterectomy and bilateral removal when fertility is not desired.

**Medical**
Platinum-based combination chemotherapy for most malignant germ-cell tumours beyond the earliest stage; dysgerminoma is also very radiosensitive.

**Follow-up**
Follow-up with the tumour marker that was raised (AFP, hCG, LDH, inhibin) and imaging to detect recurrence; granulosa cell tumours can recur many years later.

## Complications

- Ovarian torsion and rupture (chemical peritonitis with dermoids)
- Endometrial hyperplasia and cancer (oestrogen-secreting tumours)
- Virilisation
- Malignant transformation of a dermoid (rare, squamous)
- Anti-NMDA receptor encephalitis associated with teratoma
- Late recurrence (granulosa cell tumour)

## Prognosis

Dermoid cysts are benign. Malignant germ-cell tumours are highly chemosensitive and most patients are cured, often with preserved fertility. Granulosa cell tumours usually have good outcomes but need long follow-up because of late recurrence.

## Test yourself

Think of the answer, then tap the question to see it.

## Most common ovarian neoplasm in women of reproductive age, with fat, teeth and hair?

Mature cystic teratoma (dermoid cyst).

## Which marker is raised in yolk sac tumour, and which histological feature is classic?

Alpha-fetoprotein; Schiller-Duval bodies.

## A 60-year-old with postmenopausal bleeding, thickened endometrium and an adnexal mass; inhibin is raised. Diagnosis?

Granulosa cell tumour (oestrogen-secreting); Call-Exner bodies on histology.

## What is Meigs syndrome?

Ovarian fibroma with ascites and right pleural effusion, resolving after tumour removal.

## Which ovarian germ-cell tumour is the counterpart of testicular seminoma and has raised LDH?

Dysgerminoma.

[Add to my cards (5)](https://durgical.app/estudiar/?add=/en/conditions/ovarian-germ-cell-and-sex-cord-stromal-tumours/)

## Related conditions

- [Ovarian cancer](https://durgical.com/en/conditions/ovarian-cancer/)
- [Ovarian cyst](https://durgical.com/en/conditions/ovarian-cyst/)
- [Ovarian torsion](https://durgical.com/en/conditions/ovarian-torsion/)
- [Endometrial hyperplasia](https://durgical.com/en/conditions/endometrial-hyperplasia/)
- [Endometrial cancer](https://durgical.com/en/conditions/endometrial-cancer/)
- [Testicular cancer](https://durgical.com/en/conditions/testicular-cancer/)
- [Gestational trophoblastic neoplasia (choriocarcinoma)](https://durgical.com/en/conditions/gestational-trophoblastic-neoplasia/)
- [Polycystic ovary syndrome](https://durgical.com/en/conditions/polycystic-ovary-syndrome/)
- [Abnormal uterine bleeding](https://durgical.com/en/conditions/abnormal-uterine-bleeding/)
- [Thyroid storm](https://durgical.com/en/conditions/thyroid-storm/)

Educational information, not medical advice. For any symptom, see a health professional.

Sources: [Ovarian Germ Cell Tumors Treatment (PDQ®)–Patient Version](https://www.cancer.gov/types/ovarian/patient/ovarian-germ-cell-treatment-pdq) (National Cancer Institute, 2024).

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Source: https://durgical.com/en/conditions/ovarian-germ-cell-and-sex-cord-stromal-tumours/
License: CC BY 4.0 (https://creativecommons.org/licenses/by/4.0/). Cite as: DurgiPedia (durgical.com), https://durgical.com/en/conditions/ovarian-germ-cell-and-sex-cord-stromal-tumours/