Ovarian sex cord-stromal tumours: “newly recognised entities”.

ABSTRACT

A sex cord-stromal tumour is one that is composed of granulosa cells, theca cells, Sertoli cells, Leydig cells and fibroblasts of stromal origin, singly or in various combinations. A pseudopapillary pattern has been described recently in granulosa cell tumours. This was seen in both juvenile and adult types. Pseudopapillae develop as a secondary or degenerative phenomenon lacking true stromal cores. The distinction of a granulosa cell tumour from a surface epithelial carcinoma can be aided by the use of Epithelial Membrane Antigen (EMA) which is positive in carcinomas and Inhibin and Calretinin is positive in granulosa cell tumours. A negative inhibin on immunohistochemistry does not exclude a diagnosis of granulosa cell tumour.The study by Irving et al (2006) emphasizes the distinction of a group of mitotically active, cytologically bland, cellular fibromatous tumours from fibrosarcomas. These tumours show a mean of 6.7 Mitotic figure (MF)/10 HPF with a range of 4 to !9 MF/ I 0 HPF. Luteinized thecomas occur in younger women than typical the comas, the mean age being 46 years. A distinct variant of luteinized the coma is one that is associated with sclerosing peritonitis. Microcystic stromal tumour is a newly recognized entity. Twelve types of this non-functioning, neoplasm have been described, all occurring in adults. Sertoli cell tumours are rare in their pure form. They often present with oestrogenic manifestations in young women. Occasional cases are seen in the Peutz-Jeghers syndrome.