sex cord tumour

Sex cord-stromal Tumour

Sex cord-stromal tumour is a group of tumours of sex cord-derived tissues of the ovary and testis. It accounts for 8% of ovarian cancers and a minority of testicular cancers.
This group consists of:
Granulosa cell tumour
Thecoma
Sertoli-Leydig cell tumour
Fibroma
These tumours tend to be functional, producing estrogens or androgens. They may have dramatic clinical presentations. Almost all are unilateral.

Granulosa cell tumour

Granulosa cell tumo(u)rs (or granulosa-theca cell tumo(u)rs) are tumors of the granulosa cell. They are part of the sex cord-stromal tumour group of ovarian neoplasms.
The peak age at which they occur is 50-55 years, but they may occur at any age.

Granulosa cell tumour

Clinical presentation
Estrogens are produced by functioning tumours, and the clinical presentation depends on the patient's age.
If the patient is postmenopausal, she usually presents with abnormal uterine bleeding.
If the patient is of reproductive age, she would present with menometrorrhagia. However, in some cases she may stop ovulating altogether.
If the patient has not undergone puberty, HYPERLINK "http://en.wikipedia.org/w/index.php?title=Isosexual-pseudo-precocity&action=edit&redlink=1" isosexual-pseudo-precocity may be seen
Histology
The most characteristic gross appearance is a smooth surfaced solid and cystic lesion with the cysts filled with blood. Hemoperitoneum is an infrequent but classical presentation. A large variety of histological presentations exists, but they have two key features:
Call-Exner bodies ( HYPERLINK "http://en.wikipedia.org/wiki/Granulosa_cell" granulosa cells arranged haphazardly around a space containing eosinophilic fluid); and
Pale uniform nuclei, often with grooves
There have been cases where the tumor presented as a single, cyst-like, space, with no internal bleeding.
Sertoli-Leydig cell tumour
Sertoli-Leydig cell tumour, also known as arrhenoblastoma or androblastoma, is a member of the sex cord-stromal tumour group of ovarian and testicular cancers. The tumour is rare, comprising less than 1% of ovarian tumours. While the tumour can occur at any age, it occurs most often in young adults.


Classification
The tumour is subdivided into many different subtypes. The most typical is composed of tubules lined by Sertoli cells and interstitial clusters of Leydig cells.
Presentation
Due to excess testosterone secreted by the tumour, one-third of female patients present with a recent history of progressive masculinization. Masculinization is preceded by anovulation, HYPERLINK "http://en.wikipedia.org/wiki/Oligomenorrhea" oligomenorrhea, HYPERLINK "http://en.wikipedia.org/wiki/Amenorrhea" amenorrhea and defeminization. Additional signs include acne and hirsutism, voice deepening, HYPERLINK "http://en.wikipedia.org/wiki/Clitoromegaly" clitoromegaly, temporal hair recession, and an increase in musculature. Serum testosterone level is high.
Diagnosis
A recent study has shown that CD56 can be a marker for tumors of this class.
Treatment
Treatment consists of surgical resection alone with a unilateral salpingo-oophorectomy. The prognosis is generally good as the tumour tends to grow slowly and usually is benign: 25% are malignant.