Sex cord stromal tumors
Granulosa cell tumor-juvenile

Topic Completed: 1 December 2012

Minor changes: 4 May 2021

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PubMed search: Ovarian granulosa cell tumor juvenile

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Cite this page: Ehdaivand S. Granulosa cell tumor-juvenile. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/ovarytumorgctjuv.html. Accessed May 10th, 2021.
Definition / general
  • 5% of ovarian tumors in children
  • Mean age 13 years, range 0 - 67 years, 80% occur before age 20 and 97% before age 30
  • Most prepubertal patients present with sexual precocity due to excessive estrogen production; rarely produce androgens (J Endocrinol Invest 2006;29:653); older patients have non-specific abdominal swelling and pain (Am J Surg Pathol 1984;8:575)
  • Rarely associated with enchondromatosis (Ollier disease), Mafucci syndrome, abnormal karyotype / ambiguous genitalia (Am J Surg Pathol 1985;9:737)
Case reports
Gross description
  • Usually unilateral, up to 12 cm
  • Multiloculated, cystic and solid tumor with yellow-white solid areas
  • May have hemorrhage and necrosis
Gross images

Contributed by Hanni Gulwani, M.B.B.S.

Mostly solid
tumor with focal
cystic change

AFIP images

Juvenile granulosa cell tumor

Microscopic (histologic) description
  • Diffuse or macrofollicular patterns with microcysts containing eosinophilic secretions, tumor cells either have scant cytoplasm or are luteinized (Arch Pathol Lab Med 1989;113:40)
  • Round / oval hyperchromatic nuclei with small nucleoli, irregular nuclear contours
  • No / rare nuclear grooves; high mitotic rate (mean 7/10 HPF)
  • May have hobnail-type cells, pseudopapillary pattern (Am J Surg Pathol 2008;32:581)
Microscopic (histologic) images

Contributed by Hanni Gulwani, M.B.B.S.

Primordial follicles in normal ovary

Poorly formed follicles with mucoid secretions

Well demarcated
tumor with

Compact spindle cell areas with hemangiopericytoma like pattern

Loose edematous areas with abortive follicles

Spindle cells are SMA+


High MIB-1 expression

ER+ and PR+

Case #59

Various images

AFIP images

Solid cellular neoplasm with focal follicle formation

Solid nodules

Papillary pattern

Lumen of follicles stained by mucicarmine

Theca cells

Mitotic figures

Hobnail nuclei

Severe nuclear atypia

Positive stains
  • Inhibin, calretinin
Negative stains
  • HER2
Molecular / cytogenetics description
  • Consistent trisomy 12 (as with adult granulosa cell tumors)
  • More aneuploidy in juvenile than adult granulosa cell tumors
Differential diagnosis
  • Adult granulosa cell tumor: more regularly shaped follicles with basement membrane material, prominent nuclear grooves and no hyperchromasia
  • Clear cell carcinoma: older patients, diffuse hobnail cells, no follicles and no juvenile granulosa type cells
  • Thecoma: older patients, no follicles, no atypia and no mitotic figures
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