Juvenile granulose cell tumours

Clinical features

  • Account for half of paediatric/adolescent sex cord stromal cell tumours
  • Children and young adults
  • 80% before age of 20 years
  • Sexual precocity and irregular uterine bleeding (due to oestrogen production)
  • Association with enchondromatosis (Ollier’s disease), Mafucci syndrome, infantile dimorphism and ambiguous genitalia
  • 2% are bilateral
  • Abdominal distension, pain or ascites

 

Fig 101 - Juvenile granulosa cell tumor- Loose clusters of homogeneous neoplastic cells (H&E)
Fig 102 - Juvenile granulosa cell tumor- Neoplastic granulosa cells have homogeneous round centrally placed nuclei with fine chromatin. Cytoplasm is pale and vacuolated with indistinct borders (H&E)
Fig 103 - Juvenile granulosa cell tumor- Cluster of granulosa cells (grooves are barely seen) (Giemsa)

 

 

  • Cellular smears
  • Granulose cells
    • In loose clusters or single
    • Homogeneous round centrally placed nuclei
    • Nuclei with fine chromatin
    • Conspicuous nucleoli
    • Paucity of nuclear grooves
    • Atypia may be present
    • Pale and vacuolated cytoplasm
    • Indistinct cell borders
  • Theca cells
    • Elongated cells
    • Hyperchromatic nuclei
  • Macrophages

 

Immunocytochemistry

  • Inhibin: positive (granulose cells)
  • Calretinin: positive
  • EMA: negative
  • Vimentin: positive
  • Smooth muscle actin : positive in theca cells

 

Electron microscopy

  • Prominent smooth endoplasmic reticulum
  • Numerous mitochondria
  • Large lipid droplets in cytoplasm

 

Genetic studies

  • Trisomy 12 (common to other stromal cell tumours)

 

Differential diagnosis

  • Adult granulose tumour
    • Nuclear grooves
    • Less luteinized cytoplasm
  • Yolk sac tumour/ embryonal carcinoma
    • Granulose cell atypia is more pronounced than in yolk-sac or embryonal carcinoma
    • Inhibin: negative
    • Alpha-fetoprotein: positive
  • Fibroma/ thecoma
    • Rare in children
    • Spindle cells intermingled with luteinized cells

 

Main points

  • Most tumours are benign
  • Propensity for late recurrence
  • Prognostic factors: stage, size, rupture, nuclear atypia and mitotic activity
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