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Other sex cord–stromal tumours

  • Lesion

Solid ovarian tumours of the supporting cells: fibromas can bring ascites and pleural fluid, and Sertoli–Leydig tumours can make a woman's body more masculine.

Updated

What it is

Besides granulosa cell tumours, the sex cord–stromal family includes fibromas, thecomas, Sertoli–Leydig cell tumours and steroid cell tumours. Fibromas are benign, hormonally inactive tumours of ovarian stromal fibroblasts, sometimes accompanied by ascites and a right pleural effusion (Meigs syndrome). Thecomas are made of lipid-laden cells that secrete estrogen. Sertoli–Leydig cell tumours of young women secrete androgens and are linked to DICER1 mutation. Gonadoblastoma arises in dysgenetic gonads with a Y chromosome.

Structure

  • Fibroma: interlacing bundles of bland spindle cells with collagen; can be large.
  • Thecoma: sheets of round to oval cells with pale vacuolated cytoplasm; yellow cut surface from lipid.
  • Sertoli–Leydig: solid or tubular growth of Sertoli-like cells, with clusters of Leydig cells with eosinophilic cytoplasm; poorly differentiated types are more aggressive.
  • Gonadoblastoma: nests of large germ cells and small sex cord cells around hyaline deposits, often calcified.

Step by step

  1. Ovarian stromal and sex cord cells can follow female (granulosa, theca) or male (Sertoli, Leydig) lines.
  2. A tumour keeps the hormone program of its cell type: theca cells make estrogen, Leydig cells make androgen.
  3. Hormones act on target tissues: endometrium and breast with estrogen, hair follicles, voice and clitoris with androgen.
  4. Fibromas make no hormones but can leak fluid into the peritoneum, which crosses the diaphragm into the pleura (Meigs).

Sex cord–stromal tumours and their hormones

TumourTypical ageHormoneClue
Adult granulosa cell tumourPeri- and postmenopausalEstrogen, inhibinCall–Exner bodies, FOXL2
Juvenile granulosa cell tumourChildren and young womenEstrogenPrecocious puberty
ThecomaPostmenopausalEstrogenYellow, lipid-rich cells
FibromaMiddle-aged and olderNoneMeigs syndrome, Gorlin syndrome
Sertoli–Leydig cell tumourYoung womenAndrogenVirilisation, DICER1
Steroid cell tumourAdultsAndrogen (sometimes estrogen or cortisol)Reinke crystals in Leydig type

Markers

  • Inhibin and calretinin
  • SF1
  • DICER1 testing

Clinical notes

  • New hirsutism, deepening voice, clitoral enlargement and amenorrhea in a young woman with an adnexal mass suggest a Sertoli–Leydig cell tumour.
  • Ascites with a pleural effusion and a solid ovarian mass can mimic ovarian cancer; with a fibroma it disappears after removal.
  • Patients with DICER1-associated tumours and their families are offered genetic counselling.

Test yourself

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

What is Meigs syndrome?

An ovarian fibroma with ascites and a pleural effusion, usually right-sided, which resolve after the tumour is removed.

Which ovarian tumour causes virilisation in a young woman and is linked to DICER1?

The Sertoli–Leydig cell tumour.

In which patients does gonadoblastoma arise?

In dysgenetic gonads that contain a Y chromosome, where it can progress to dysgerminoma.

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Educational content on the mechanisms of disease; not a diagnostic or treatment guide and no substitute for a health professional's advice. No doses are given.

Sources: DICER1 syndrome: MedlinePlus Genetics (MedlinePlus, US National Library of Medicine, 2026); Ovarian Cyst (StatPearls (NCBI Bookshelf), 2026).

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