STEPTRACKERREPRODUCTIVE + ONCOLOGY | PATHOLOGY + ENDOCRINE | HIGH-YIELD REVIEW

Ovarian Germ Cell & Sex Cord–Stromal Tumors

Tumors of young women and girls named by the tissue they imitate — and identified by the hormone or marker they make.

FOCUS ON
Mature teratoma (dermoid): most common ovarian tumor in young women; hair, teeth, sebum; torsion. Markers: dysgerminoma → LDH; yolk sac → AFP (Schiller-Duval bodies); choriocarcinoma → hCG. Hormones: granulosa → estrogen + inhibin (Call-Exner bodies); Sertoli-Leydig → androgens. Fibroma + ascites + pleural effusion = Meigs.
TUMOR
ORIGIN · BEHAVIOR
FEATURES
MARKER · CLUE
Mature cystic teratoma (dermoid)
most common germ cell tumor
Benign; all three germ layers; arises from a germ cell after meiosis I
Cyst with hair, sebum, teeth, skin; calcifications; can cause torsion
Struma ovarii → hyperthyroidism · rare SCC in older women
Teratoma — special effects
Tissues within the tumor function
Anti-NMDA receptor encephalitis (neural tissue): psychiatric symptoms, seizures, dyskinesias
Remove teratoma → improvement
Immature teratoma
Malignant; immature (fetal) tissue, mostly neuroectoderm
Adolescents, young women; solid
Grade by amount of immature neural tissue
Dysgerminoma
Malignant; ovarian counterpart of seminoma
Sheets of uniform large cells with clear cytoplasm ("fried egg"); lymphocytes in fibrous septa
↑ LDH (± hCG) · OCT4, c-KIT ⁺ · radiosensitive · Turner/gonadal dysgenesis
Yolk sac (endodermal sinus) tumor
Malignant; extraembryonic yolk sac differentiation
Girls and young women; aggressive; Schiller-Duval bodies (glomerulus-like)
↑ AFP
Ovarian choriocarcinoma
Malignant trophoblast without villi (non-gestational)
Syncytio- + cytotrophoblasts; hemorrhagic; precocious puberty, theca lutein cysts
↑↑ hCG · spreads by blood
Granulosa cell tumor
most common malignant sex cord–stromal
Makes estrogen; FOXL2 mutation (adult type)
Postmenopausal bleeding, endometrial hyperplasia/carcinoma · girls: precocious puberty · Call-Exner bodies, coffee-bean nuclei
↑ inhibin
Thecoma
Benign; lipid-laden theca cells
Postmenopausal; may produce estrogen
Yellow solid mass
Fibroma
Benign; bundles of spindle fibroblasts
Meigs syndrome: fibroma + ascites + (right) pleural effusion — resolves after removal
Mimics ovarian cancer · Gorlin syndrome
Sertoli-Leydig cell tumor
Makes androgens; DICER1
Young women: virilization (hirsutism, deep voice, clitoromegaly), amenorrhea; Reinke crystals in Leydig cells
↑ testosterone
Krukenberg tumor
Metastatic signet ring adenocarcinoma (stomach, also breast, colon)
Bilateral solid ovarian masses
Mucin-filled signet ring cells

Mechanism — germ cell tumor family tree

Primordial germ cell
→
UndifferentiatedDysgerminoma
TotipotentEmbryonal carcinoma
Embryonic tissue
→
TeratomaMature / immature
Extraembryonic
→
Yolk sac (AFP)
Choriocarcinoma (hCG)

Each tumor makes what its model tissue makes — yolk sac → AFP, trophoblast → hCG, granulosa → estrogen and inhibin.

Marker and hormone sheet

TUMOR
PRODUCT
EFFECT
Dysgerminoma
LDH, sometimes hCG
—
Yolk sac
AFP
—
Choriocarcinoma
hCG
Theca lutein cysts, hyperthyroid-like
Granulosa
Estrogen, inhibin
Bleeding, hyperplasia, precocious puberty
Sertoli-Leydig
Testosterone
Virilization
Struma ovarii
Thyroid hormone
Hyperthyroidism

How it's tested

COMMON CONFUSIONSchiller-Duval bodies (yolk sac, AFP) vs Call-Exner bodies (granulosa, estrogen and inhibin). The "S" tumor makes AFP; the "C-E" tumor makes estrogen.
THE TRAPMeigs syndrome looks like metastatic ovarian cancer (mass + ascites + effusion), but it's a benign fibroma and everything resolves after removal.
YOUR APPROACHAge (girl, young woman, postmenopausal) → hormonal effect (estrogen, androgen, thyroid) → marker (AFP, hCG, LDH, inhibin) → histologic body.

If you see… think…

Young woman with an ovarian cyst containing hair and teeth → mature cystic teratoma
Dermoid cyst + hyperthyroidism → struma ovarii
Young woman with psychosis and seizures + ovarian mass → anti-NMDA receptor encephalitis (teratoma)
Sheets of fried-egg cells + ↑ LDH → dysgerminoma
Girl with ovarian mass, ↑ AFP, glomerulus-like structures → yolk sac tumor
Ovarian mass with very high hCG and no villi → choriocarcinoma
Postmenopausal bleeding + ovarian mass + ↑ inhibin → granulosa cell tumor
Ovarian mass + ascites + right pleural effusion, all resolve after surgery → Meigs (fibroma)
Young woman with deepening voice and ovarian mass → Sertoli-Leydig cell tumor
Bilateral ovarian masses with signet ring cells → Krukenberg tumor