ovary

Fifth stage

Gynecology
Lec-

9/5/2017

Sexcord-stromal tumour

Sex cord-stromal tumouris agroup oftumoursofsex cord-derivedtissues of theovaryandtestis. Itaccounts for 8% ofovarian cancersanda minority oftesticular cancers.

This group consists of:

Granulosa cell tumour
Thecoma
Sertoli-Leydigcell tumour
Fibroma
These tumours tend to befunctional, producingestrogensorandrogens. They may have dramatic clinical presentations. Almost all are unilateral.

Granulosacell tumour

Granulosa cell tumo(u)rs (orgranulosa-thecacell tumo(u)rs) aretumorsofthegranulosa cell. They are part of thesex cord-stromaltumourgroup ofovarian neoplasms.
The peak age at which they occur is 50-55years, but they may occur at any age.


Clinical presentation
Estrogensareproduced byfunctioningtumours, and the clinical presentation depends on the patient's age.
If the patient ispostmenopausal, sheusually presents withabnormal uterinebleeding.
If the patient is ofreproductiveage, she would present withmenometrorrhagia. However, in some cases she may stop ovulating altogether.
If the patient has not undergonepuberty, isosexual-pseudo-precocitymaybe seen.

Histology

The most characteristic gross appearanceis a smooth surfaced solid and cystic lesion with the cysts filled withblood. Hemoperitoneum is an infrequent but classical presentation. A large variety ofhistologicalpresentations exists, but they have two key features:
Call-Exner bodies (granulosa cellsarranged haphazardly around a space containingeosinophilicfluid); and
Pale uniform nuclei, often with grooves
There have been cases where the tumorpresented as a single, cyst-like, space, with no internal bleeding.

Sertoli-Leydigcell tumour

Sertoli-Leydig cell tumour, also known asarrhenoblastomaorandroblastoma, isa member of thesex cord-stromaltumourgroup ofovarianandtesticular cancers. Thetumour is rare, comprising less than 1% of ovarian tumours. While the tumourcan occur at any age, it occurs most often in young adults.

Classification

The tumour is subdivided into manydifferent subtypes. The most typical is composed oftubuleslined bySertoli cellsandinterstitial clusters ofLeydig cells.

Presentation

Due to excesstestosteronesecreted by the tumour, one-third offemalepatients present with a recent history of progressivemasculinization. Masculinization is preceded byanovulation, oligomenorrhea, amenorrheaanddefeminization. Additional signs includeacneandhirsutism, voice deepening, clitoromegaly, temporal hair recession, and an increase in musculature. Serum testosteronelevel is high.


Diagnosis
A recent study has shown thatCD56canbe a marker for tumors of this class.

Treatment

Treatment consists of surgical resectionalone with a unilateral salpingo-oophorectomy. Theprognosisisgenerally good as the tumour tends to grow slowly and usually isbenign: 25% aremalignant.