Sex cord–gonadal stromal tumour

s by incidence and risk of ovarian cancer, with sex cord-stromal tumours at right. Tumour types in order of prevalence • Granulosa cell tumour. This tumour produces granulosa cells, which normally are found in the ovary. It is malignant in 20% of women diagnosed with it. It tends to present in women in the 50-55yo age group with post menopausal vaginal bleeding. Uncommonly, a similar but possibly distinct tumour, juvenile granulosa cell tumour, presents in pre-pubertal girls with precocious puberty. In both groups, the vaginal bleeding is due to oestrogen secreted by the tumour. In older women, treatment is total abdominal hysterectomy and removal of both ovaries. but it has been reported with juvenile-type granulosa cells. It has been reported to occur in the ovary usually, rarely in the testis. Due to its rarity, the malignant potential of this tumour is unclear; there is one case report of late metastasis. • Sex cord tumour with annular tubules, abbreviated SCTAT. These are rare tumours that may be sporadic or associated with Peutz–Jeghers syndrome. ==Diagnosis==

Definitive diagnosis of these tumours is based on the histology of tissue obtained in a biopsy or surgical resection. In a retrospective study of 72 cases in children and adolescents, the histology was important to prognosis. A number of molecules have been proposed as markers for this group of tumours. CD56 may be useful for distinguishing sex cord–stromal tumours from some other types of tumours, although it does not distinguish them from neuroendocrine tumours. Calretinin has also been suggested as a marker. For diagnosis of granulosa cell tumour, inhibin is under investigation. Granulosa cell tumours and Sertoli-Leydig cell tumours have specific genetic mutations that are characteristic and can help support the diagnosis. Image:Granulosa cell tumour1.jpg|Low magnification micrograph of a granulosa cell tumour. H&E stain. Image:Thecoma high mag.jpg|High magnification micrograph of a thecoma. H&E stain. Image:Thecoma low mag.jpg|Low magnification micrograph of a thecoma. H&E stain. Image:Leydig cell tumour1.jpg|Low magnification micrograph of a Leydig cell tumour. H&E stain. Image:Leydig cell tumour2.jpg|Intermediate magnification micrograph of a Leydig cell tumour. H&E stain. Image:Leydig cell tumour3.jpg|High magnification micrograph of a Leydig cell tumour. H&E stain. Image: Sertoli cell tumour low mag.jpg|Low magnification micrograph of a Sertoli cell tumour. H&E stain. Image: Sertoli cell tumour high mag.jpg|High magnification micrograph of a Sertoli cell tumour. H&E stain. ==Prognosis==

A retrospective study of 83 women with sex cord–stromal tumours (73 with granulosa cell tumour and 10 with Sertoli-Leydig cell tumour), all diagnosed between 1975 and 2003, reported that survival was higher with age under 50, smaller tumour size, and absence of residual disease. The study found no effect of chemotherapy. A retrospective study of 67 children and adolescents reported some benefit of cisplatin-based chemotherapy. ==Research==

A prospective study of ovarian sex cord–stromal tumours in children and adolescents began enrolling participants in 2005. The International Ovarian and Testicular Stromal Tumor Registry is studying these rare tumours and collecting data on them to further research. Targeted treatments are being evaluated for these tumours as well. ==See also==