Sex cord-stromal tumors are a heterogeneous group comprising 5-8% of ovarian neoplasms. They originate from sex cord cells (granulosa, Sertoli) and stromal cells (theca, Leydig, fibroblast) of the ovary. Hormone production is characteristic: estrogen (granulosa-theca tumors → endometrial thickening/bleeding), androgen (Sertoli-Leydig tumors → virilization/hirsutism). On imaging, they appear as solid or solid-cystic heterogeneous masses. Most have low-intermediate malignant potential and prognosis is good when diagnosed at early stage.
Age Range
30-60
Peak Age
50
Gender
Female predominant
Prevalence
Rare
Sex cord-stromal tumors originate from gonadal stromal and sex cord components of the ovary. Granulosa cells normally produce estrogen — granulosa cell tumors secrete excess estrogen causing endometrial proliferation, creating risk for endometrial hyperplasia or carcinoma. On imaging, solid component reflects high cellularity and fibrous stroma — moderate T2 signal on MRI, prominent enhancement. Cystic/hemorrhagic component reflects tendency for intratumoral hemorrhage — granulosa tumors are prone to bleeding (destruction of Call-Exner bodies). Fibroma/thecoma subgroup contains dense collagen and fibroblasts — typically low T2 signal (fibrous tissue). Sertoli-Leydig tumors produce androgen causing virilization. Tumor size and subtype determine imaging characteristics: small solid tumors are homogeneous, large tumors appear heterogeneous with cystic degeneration/hemorrhage.
Presence of solid or solid-cystic ovarian mass with endometrial thickening (>5 mm postmenopausal, >14 mm premenopausal) is the strongest clue for estrogen-producing sex cord-stromal tumor. Granulosa cell tumor (70-75%), thecoma (60%), and fibrothecoma can produce estrogen. Endometrial biopsy is recommended due to risk of endometrial hyperplasia (25-50%) or concurrent endometrial carcinoma (5-10%). This combination is rare in ovarian epithelial tumors and germ cell tumors — near-pathognomonic clue in differential diagnosis.
Unilateral, predominantly solid or solid-cystic heterogeneous ovarian mass. Solid component hypoechoic-isoechoic. Cystic areas variable in size and scattered — may create 'sponge' or 'Swiss cheese' pattern. Hemorrhagic areas may appear hyperechoic.
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A unilateral solid-cystic heterogeneous ovarian mass is seen, and sex cord-stromal tumor (granulosa cell tumor/fibroma) should be considered in the differential diagnosis.
Intermediate-to-low signal intensity in solid component on T2-weighted images — reflecting fibrous stroma and dense cellularity. In fibroma/thecoma subtypes, T2 signal may be very low (below muscle). Cystic/hemorrhagic areas show variable high signal.
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Low-intermediate signal in the solid component is seen on T2-weighted sequences, suggesting fibrous stromal composition; consistent with sex cord-stromal tumor.
Heterogeneously enhancing solid mass in portal venous phase. Solid areas show moderate-avid enhancement. Hemorrhagic areas are high density (50-70 HU on non-contrast CT). Cystic areas are low density.
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A heterogeneously enhancing mass of ovarian origin is seen on contrast-enhanced CT with accompanying hemorrhagic component.
Intratumoral hemorrhagic areas show high signal on T1-weighted images (methemoglobin). Solid component isointense to muscle. Hemorrhage tendency is high in granulosa cell tumors.
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Focal high-signal areas are seen within the mass on T1-weighted sequences, consistent with subacute hemorrhage.
Mild-moderate diffusion restriction in solid component on DWI. Less prominent compared to high-grade malignant tumors. Diffusion restriction is minimal in fibroma/thecoma subtypes.
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Mild diffusion restriction is seen in the solid component on DWI, consistent with low-intermediate grade neoplasm.
Criteria
Most common sex cord-stromal tumor (70%). More common in postmenopausal women. Call-Exner bodies are histologically diagnostic. Elevated inhibin B is tumor marker.
Distinct Features
Solid-cystic, heterogeneous, sponge/Swiss cheese pattern, hemorrhage common. Estrogen production → endometrial thickening. Late recurrence risk (30%, 5-30 years later). Multilocular cystic or solid-cystic on US.
Criteria
Solid, homogeneous, dense fibrous tissue. Non-functional (no hormone production). Very low T2 signal (below muscle). Meigs syndrome may accompany (pleural effusion + ascites).
Distinct Features
Very low T2 signal (lowest among pelvic tumors). Late and minimal enhancement. Calcification may be present. Benign. In Meigs syndrome, ascites + pleural effusion resolves with surgery.
Criteria
Rare (<0.5%). Young women (20-30 years). Androgen production → virilization. Spectrum from well-differentiated to poorly differentiated.
Distinct Features
Small solid mass or heterogeneous solid-cystic. Homogeneous enhancement. Virilization signs (hirsutism, acne, amenorrhea, clitoromegaly, voice deepening) strongly support diagnosis. Retiform or heterologous elements in poorly differentiated type.
Criteria
Originates from theca cells. In postmenopausal women. Estrogen production. Solid, well-defined. Benign.
Distinct Features
Similar to fibroma but more homogeneous and may enhance more. May contain fat (lipid-rich thecoma — low density on CT, fat signal on MRI). Endometrial thickening commonly accompanies.
Distinguishing Feature
Fibroma is entirely solid, homogeneous, very low T2 signal, minimal enhancement, non-functional. Granulosa cell tumor is solid-cystic, heterogeneous, intermediate T2 signal, prominent enhancement, estrogen production + endometrial thickening. Hemorrhage is rare in fibroma, common in granulosa tumor.
Distinguishing Feature
Serous carcinoma has prominent papillary projections, predominant cystic component, intermediate-high T2 signal, marked diffusion restriction, very high CA-125. Sex cord-stromal tumor is solid-predominant, intermediate-low T2 signal, less diffusion restriction, estrogen/androgen production. Elevated inhibin B is specific for sex cord-stromal tumor.
Distinguishing Feature
Metastasis is bilateral (60-80%), known primary malignancy history, peritoneal implants/ascites accompany. Sex cord-stromal tumor is unilateral (95%+), hormone production (estrogen/androgen), endometrial thickening, elevated inhibin B distinguishes.
Urgency
urgentManagement
surgicalBiopsy
Not NeededFollow-up
annualTreatment of sex cord-stromal tumors is surgical — fertility-sparing (unilateral salpingo-oophorectomy) in young women, bilateral salpingo-oophorectomy + hysterectomy in postmenopausal women. Endometrial biopsy is mandatory in estrogen-producing tumors (concurrent endometrial carcinoma 5-10%). Long-term follow-up is required for granulosa cell tumors due to late recurrence risk (30%, 5-30 years) — inhibin B and estradiol levels monitored as tumor markers. Platinum-based chemotherapy (BEP regimen) may be given for advanced-stage granulosa cell tumor. Fibroma is benign and oophorectomy is curative. In Meigs syndrome, ascites and pleural effusion resolve with surgery. Virilization signs in Sertoli-Leydig tumors reverse with surgery.
Sertoli-Leydig cell tumors have low-intermediate malignant potential. Virilization symptoms usually regress after tumor removal. Prognosis depends on stage and differentiation grade. Fertility-sparing surgery can be considered in young patients.