Granulosa cell tumour
Encyclopedia
Granulosa cell tumours are tumour
Tumor
A tumor or tumour is commonly used as a synonym for a neoplasm that appears enlarged in size. Tumor is not synonymous with cancer...

s that arise from granulosa cell
Granulosa cell
A granulosa cell or follicular cell is a somatic cell of the sex cord that is closely associated with the developing female gamete in the ovary of mammals.-Anatomy and function:...

s. These tumours are part of the sex cord-gonadal stromal tumouror non-epithelial group of tumours. Although granulosa cells normally occur only in the ovary
Ovary
The ovary is an ovum-producing reproductive organ, often found in pairs as part of the vertebrate female reproductive system. Ovaries in anatomically female individuals are analogous to testes in anatomically male individuals, in that they are both gonads and endocrine glands.-Human anatomy:Ovaries...

, granulosa cell tumours occur in both ovaries and testicles (see Ovarian cancer
Ovarian cancer
Ovarian cancer is a cancerous growth arising from the ovary. Symptoms are frequently very subtle early on and may include: bloating, pelvic pain, difficulty eating and frequent urination, and are easily confused with other illnesses....

 and Testicular cancer
Testicular cancer
Testicular cancer is cancer that develops in the testicles, a part of the male reproductive system.In the United States, between 7,500 and 8,000 diagnoses of testicular cancer are made each year. In the UK, approximately 2,000 men are diagnosed each year. Over his lifetime, a man's risk of...

). These tumours should be considered malignant and treated in the same way as other malignant tumours of ovary. The staging system for these tumours is the same as for epithelial tumours and most present as stage I. 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. Although this tumour usually occurs in children (hence its name), it has been reported in adults.

Clinical presentation

Estrogen
Estrogen
Estrogens , oestrogens , or œstrogens, are a group of compounds named for their importance in the estrous cycle of humans and other animals. They are the primary female sex hormones. Natural estrogens are steroid hormones, while some synthetic ones are non-steroidal...

s are produced by functioning tumours, and the clinical presentation depends on the patient's age and sex.
  • Female
    • If the patient is postmenopausal, she usually presents with abnormal uterine bleeding.
    • If the patient is of reproductive age, she would present with menometrorrhagia
      Menometrorrhagia
      Menometrorrhagia is a condition in which prolonged or excessive uterine bleeding occurs irregularly and more frequently than normal.-Causes:It can occur due to any of several causes, including hormonal imbalance, endometriosis, uterine fibroids, or cancer. It can lead to anemia in long-standing...

      . However, in some cases she may stop ovulating altogether.
    • If the patient has not undergone puberty
      Puberty
      Puberty is the process of physical changes by which a child's body matures into an adult body capable of reproduction, as initiated by hormonal signals from the brain to the gonads; the ovaries in a girl, the testes in a boy...

      , early onset of puberty may be seen.

  • Male

Gene defect

Using next generation DNA sequencing
DNA sequencing
DNA sequencing includes several methods and technologies that are used for determining the order of the nucleotide bases—adenine, guanine, cytosine, and thymine—in a molecule of DNA....

, it was discovered that 97% of granulosa cell tumours contain an identical mutation in the FOXL2 gene http://content.nejm.org/cgi/content/full/NEJMoa0902542. 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.

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. These appear to be a normal change with age in this species.

External links

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