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Juvenile granulosa cell tumor: Wikis

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Granulosa cell tumour
Classification and external resources

Low magnification micrograph of a granulosa cell tumour showing the typical central location in the ovary; there is no involvement of the ovarian surface (seen upper-left of image). H&E stain.
ICD-10 C56.
ICD-9 183
ICD-O: 8620
eMedicine med/928
MeSH D006106

Granulosa cell tumours (or granulosa-theca cell tumours) are tumours that produce granulosa cells. These tumours are part of the sex cord-gonadal stromal tumour group of tumours. Although granulosa cells normally occur only in the ovary, granulosa cell tumours occur in both ovaries and testicles (see Ovarian cancer and Testicular cancer). Most of these tumours are benign. The peak age at which they occur is 50-55 years, but they may occur at any age.

Juvenile granulosa cell tumour is a similar but distinct rare tumour. It too occurs in both the ovary and testis. In the testis it is extremely rare, and has not been reported to be malignant.[1] Although this tumour usually occurs in children (hence its name), it has been reported in adults.[2]

Contents

Clinical presentation

Estrogens are produced by functioning tumours, and the clinical presentation depends on the patient's age and sex.

  • Male

Histology

Intermediate magnification micrograph of a granulosa cell tumour showing polygonal-shaped cells in gland-like formations. H&E stain.

The most characteristic gross appearance is a smooth surfaced solid and cystic lesion with the cysts filled with blood. There have been cases where the tumour presented as a single cyst-like space, but with no bleeding into the space. Hemoperitoneum is an infrequent but classical presentation. A large variety of histological presentations exists, but they have two key features:

  1. Call-Exner bodies (granulosa cells arranged haphazardly around a space containing eosinophilic fluid); and
  2. Pale uniform nuclei, often with grooves

Gene Defect

Using next generation DNA sequencing, it was discovered that 97% of granulosa cell tumours contain an identical mutation in the FOXL2 gene [1]. This is a somatic mutation meaning it is not usually transmitted to descendants. It is believed that this mutation may be the cause of granulosa cell tumours.

References

External links

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