Ovary

Sex cord stromal tumors

Granulosa cell tumor-juvenile



Topic Completed: 6 July 2021

Minor changes: 6 July 2021

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PubMed search: Ovarian granulosa cell tumor juvenile [TI] pathology full text [SB]

Jutta Huvila, M.D., Ph.D.
C. Blake Gilks, M.D.
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Cite this page: Huvila J, Gilks CB. Granulosa cell tumor-juvenile. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/ovarytumorgctjuv.html. Accessed October 21st, 2021.
Definition / general
  • Sex cord stromal tumor composed of primitive appearing granulosa cells with follicular and solid growth patterns
Essential features
  • Sex cord stromal tumor with primitive granulosa cell differentiation
  • Solid and follicular growth
  • Almost always occurs in patients younger than 30 years
  • Lacks the FOXL2 somatic mutation seen in adult granulosa cell tumor
  • Usually stage IA and associated with a favorable prognosis
ICD coding
  • ICD-O: 8622/1 - granulosa cell tumor, juvenile
  • ICD-10:
    • C56.9 - malignant neoplasm of unspecified ovary
    • D39.1 - neoplasm of uncertain behavior of ovary
  • ICD-11: 2F76 & XH2KH2 - granulosa cell tumor, juvenile
Epidemiology
Sites
Pathophysiology
  • Unknown
Etiology
  • Unknown
Clinical features
Diagnosis
  • Diagnosis is made at the time of removal of an adnexal mass
  • Possibility of juvenile granulosa cell tumor may be suspected if there are estrogenic manifestations in a prepubertal patient but histopathological examination is required for diagnosis
Radiology description
  • Typically appears on imaging as a large, unilateral, multicystic mass with occasional septae; most contain both solid and cystic components
  • Can have a sponge-like appearance on imaging (Radiographics 2014;34:2039)
Prognostic factors
  • Of low malignant potential, with a very favorable prognosis for patients with stage IA tumors (which account for a large majority of cases) but a guarded prognosis if there is spread beyond the ovary
Case reports
Treatment
Gross description
  • Usually unilateral with a smooth surface
  • Mean size 12.5 cm (Am J Surg Pathol 1984;8:575)
  • Multiloculated, cystic and solid tumor with yellow-white solid areas
  • May have hemorrhage and necrosis
Gross images

AFIP Images
Solid and slightly lobulated surface

Solid and slightly lobulated

Large locules with smooth linings

Large locules with smooth linings

Solid and cystic

Solid and cystic

Frozen section description
  • Smooth surfaced ovarian mass in a young patient
  • Presence of follicular differentiation is helpful but definitive diagnosis may have to be deferred to permanent sections
Microscopic (histologic) description
  • Diffuse or nodular appearance at low power
  • Macrofollicle and microfollicle formation containing eosinophilic secretions
  • Tumor cells are often luteinized
  • Round / oval hyperchromatic nuclei with small nucleoli, irregular nuclear contours
  • No / rare nuclear grooves; high mitotic rate (mean 11/10 high power fields) (Arch Pathol Lab Med 1989;113:40)
  • Bizarre / atypical nuclei are rarely present
  • May have pseudopapillary architecture (Am J Surg Pathol 2008;32:581)
Microscopic (histologic) images

Contributed by Jutta Huvila, M.D., Ph.D. and AFIP
Lobulated growth pattern

Lobulated growth pattern

Follicular differentiation

Follicular differentiation

Atypical cells lining follicle

Atypical cells lining follicle

Solid growth

Solid growth

Solid cellular neoplasm with focal follicle formation Solid cellular neoplasm with focal follicle formation

Solid cellular neoplasm with focal follicle formation


Solid nodules

Solid nodules

Mitotic figures

Mitotic figures

Hobnail nuclei

Hobnail nuclei

Bizarre nuclei

Bizarre nuclei

Virtual slides

Images hosted on other servers:
Lobulated architecture with follicle-like spaces

Lobulated architecture with follicle-like spaces

Molecular / cytogenetics description
Sample pathology report
  • Right ovary, oophorectomy:
    • Juvenile granulosa cell tumor, negative for ovarian surface involvement or extraovarian spread
Differential diagnosis
Board review style question #1

A 2 year old girl presents with a unilateral ovarian tumor. Histology shows follicle-like spaces lined by cells with moderate amounts of cytoplasm and showing nuclear atypia and prominent nucleoli. What is the diagnosis?

  1. Juvenile granulosa cell tumor
  2. Sertoli-Leydig cell tumor
  3. Teratoma
  4. Yolk sac tumor
Board review style answer #1
A. Juvenile granulosa cell tumor

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Reference: Granulosa cell tumor - juvenile
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