Granulosa cell tumour

Granulosa cell tumour
Micrograph of a juvenile granulosa cell tumour with hyaline globules. H&E stain.
Classification and external resources
Specialty oncology
ICD-10 C56
ICD-9-CM 236.2 183 236.2
ICD-O 8620
eMedicine med/928
MeSH D006106

Granulosa cell tumours (or granulosa-theca cell tumours) are tumours that arise from granulosa cells. These tumours are part of the sex cord-gonadal stromal tumour or non-epithelial 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). These tumours should be considered malignant and treated in the same way as other malignant tumours of ovary. The ovarian disease has two forms, juvenile and adult, both characterized by indolent growth,[1] and therefore has high recovery rates.[2] [3] The staging system for these tumours is the same as for epithelial tumours and most present as stage I.[4] 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.[5] Although this tumour usually occurs in children (hence its name), it has been reported in adults.[6]

Clinical presentation

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

Gene defect

Using next generation DNA sequencing, it was discovered that 97% of granulosa cell tumours contain an identical mutation in the FOXL2 gene . 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.

Tumor marker

Inhibin, a hormone, has been used as tumor marker for granulosa cell tumor.

Gross appearance

Tumors vary in size, from tiny spots to large masses, with an average of 10 cm in diameter. Tumors are oval and soft in consistency. On cut-section, histology reveals reticular, trabecular areas with interstitial haemorrhage and Call-Exner bodies-small cyst like spaces interspersed within a graafian follicle.

Granulosa cell clusters in other species

In the ovaries of aging squirrel monkeys (Saimiri sciureus), clusters of granulosa cells occur that resemble granulosa cell tumours in humans.[7] These appear to be a normal change with age in this species.

See also

References

  1. ↑ "Prognostic factors in adult granulosa".
  2. ↑ "Juvenile granulosa cell tumor of the ovary. A clinic pathological analysis of 125 cases. Beth Israel Deaconess Medical Center, Boston".
  3. ↑ "Program in Gynecologic Medical Oncology, Beth Israel Deaconess Medical Center, Boston".
  4. ↑ Gynaecology. 3rd Ed. 2003. Churchill Livingstone. PP. 690-691.
  5. ↑ Dudani R, Giordano L, Sultania P, Jha K, Florens A, Joseph T (April 2008). "Juvenile granulosa cell tumor of testis: case report and review of literature". Am J Perinatol. 25 (4): 229–31. doi:10.1055/s-2008-1066878. PMID 18548396.
  6. ↑ Lin KH, Lin SE, Lee LM (July 2008). "Juvenile granulosa cell tumor of adult testis: a case report". Urology. 72 (1): 230.e11–3. doi:10.1016/j.urology.2007.11.126. PMID 18313118.
  7. ↑ Walker ML, Anderson DC, Herndon JG, Walker LC (2009). "Ovarian aging in squirrel monkeys (Saimiri sciureus)". Reproduction. 138 (4): 793–799. doi:10.1530/REP-08-0449. PMID 19656956.
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