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adult-type granulosa cell tumor

Monday 13 February 2006

Definition: Ovarian sex cord stromal tumor showing differentiation towards follicular granulosa cells that can occur in adults (adult-type granulosa cell tumor) and in younger patients (juvenile granulosa cell tumor).


- Adult-type Granulosa Cell Tumor

- Call-Exner bodies in Adult Granulosa Cell Tumor; nuclear grooves, inhibin+ and EMA neg.

Clinical synopsis

- Usually childbearing age
- 75% have hyperestrinism, which may result in:

  • isosexual precocious puberty in children
  • metrorrhagia in adults, including postmenopausal
    - Some cases clinically hormonally inactive
    - A few androgenic
    - Elevated serum inhibin and follicle regulatory proteins


- Differentiation toward follicular granulosa cells
- Debatable whether arises from:

  • granulosa cells in preexisting follicles
  • specialized ovarian stroma


- Usually encapsulated
- Usually smooth, lobulated outline
- Cut surface:

  • predominantly solid
  • or cystic
    • possible entirely cystic gross appearance.
    • cysts filled with straw-colored or mucoid fluid
    • sometimes so prominent as to simulate appearance of a cystadenoma
    • a disproportionate number of androgenic tumors are large and cystic, either unilocular or multilocular
  • admixture of solid and cystic areas

- Color

  • usually gray
  • may be yellow in areas of luteinization


- Extremely variable, even within same neoplasm
- Patterns of growth:

- Typical appearance of nuclei (WebPathology 1 and 2)

  • Folds or grooves in nuclei (important diagnostic feature)
    • result in ‘coffee-bean’ appearance
    • coffee-bean nuclei in adult type of ovarian granulosa cell tumor

- May be:

  • a theca cell component
  • focal luteinization of either granulosa (WebPathology) or theca cell component
    • particularly prominent if associated with pregnancy, together with edema and disorderly arrangement
  • hepatocytic differentiation

- Stromal hemorrhage (WebPathology)
- Possible fusiform cell stroma (WebPathology)


Neoplastic granulosa cells have:
- abundant intermediate filaments
- specialized cell junctions, some with appearance of typical desmosomes

Steroid production

- by both theca and granulosa cells with predominance of:


- vimentin
- desmoplakin (desmosomal plaque protein)
- inhibin
- follicle regulatory proteins
- CD99 (O13; MIC2)
- A103 (melan-A)
- cytokeratin

  • 33–50% of cases
  • typical dot-like distribution
  • mainly CK8 and CK18 types
    - smooth muscle actin (SMA) (nearly all cases)
    - desmin (less common)
    - S-100 protein (≈50% of cases)
    - Negative for EMA

Differential diagnosis

Suspect granulosa cell origin of a metastatic tumor if a combination of microcystic and trabecular formations, especially if also Call–Exner bodies and grooved nuclei

- thecoma
- endometrial stromal tumors
- endometrial carcinomas
- sex cord tumor with annular tubules
- gonadoblastoma


- Adult Granulosa Cell Tumor at WebPathology
- PathConsult

See also

- testicular tumors
- ovarian tumors

  • ovarian granulosa cell tumor
    • juvenile-type granulosa cell tumor
    • ovarian granulosa-stromal cell tumors
    • fibroma-thecoma tumor groups


- see granulosa cell tumor