Feminizing ovarian gynandroblastoma.
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A total of 30 sex cord-stromal tumors including 9 adult type and 5 juvenile type granulosa cell tumors (GCTs), 4 Sertoli-Leydig cell tumors (SLTs), 1 gynandroblastoma, 5 thecomas, 2 fibromas and 3 sclerosing stromal tumors were immunohistochemically evaluated by means of cytokeratins of different molecular weight, vimentin and laminin with regard to the histogenesis of these tumors and to the embryogenesis of the sex cord and stroma of developing gonads. For comparison, 7 embryonic gonads, 9 fetal and 9 adult ovaries, 14 fetal and 5 postnatal testes, and 1 gonadoblastoma were also examined. The coelomic epithelium of all gonads were positive for both cytokeratins (CAM 5.2 and AE1) and vimentin. In fetal ovaries, the granulosa cells of primordial follicles express low molecular weight cytokeratins only and those cells of more maturing follicles did not express any cytokeratin or vimentin. In adult ovaries, the granulosa cells of primordial follicles coexpressed low molecular weight cytokeratins and vimentin, but those cells of more maturing follicles expressed vimentin only. In fetal testes before 20 weeks gestational age, the Sertoli and Leydig cells did not express any cytokeratins and vimentin. After that time, both cells expressed vimentin only throughout life. The rete ovarii and rete testis from fetal to adult life coexpressed both low molecular weight cytokeratins and vimentin. The rete ovarii in all ages and rete testis in prenatal and childhood ages were surrounded by the laminin-positive basement membrane, however, the rete testis in adult were not. In neoplasia, the GCTs, thecomas, fibromas, and sclerosing stromal tumors expressed vimentin only.(ABSTRACT TRUNCATED AT 250 WORDS)
AIMS: Inhibin is a heterodimeric protein hormone which appears to be a sensitive serological and immunohistochemical marker of ovarian granulosa cell tumours. The purposes of this study were to examine inhibin immunoreactivity in a wide range of gonadal stromal neoplasms and to assess its value in the differential diagnosis of problematic ovarian tumours. METHODS AND RESULTS: Inhibin immunostaining was identified in eight cases of stromal hyperthecosis, 24 adult-type granulosa cell tumours, two juvenile granulosa cell tumours, nine Sertoli cell or Sertoli-Leydig cell tumours, one gynandroblastoma, three gonadoblastomas, two cases of sex cord tumour with annular tubules, two steroid cell tumours and two sclerosing stromal tumours. Inhibin was also present in 4/6 fibrothecomas and 2/3 unclassified gonadal stromal tumours. There was no staining of neoplastic cells in any of the problematic ovarian tumours, in many of which a diagnosis of gonadal stromal tumour had been initially considered. However, inhibin was detected in reactive stromal cells in many cases. CONCLUSIONS: Inhibin is a sensitive immunohistochemical marker of a wide range of gonadal stromal tumours and is of value in the differential diagnosis of ovarian neoplasia.
In a study of 194 masculinizing ovarian tumors from the Ovarian Tumor Registry, the criteria for reclassifying these tumors on a histologic basis are discussed. It is proposed that the terms "gynandroblastoma" and "arrhenoblastoma" be discarded in favor of "gonadal stromal," and that the general classification of "lipoid tumor" can be avoided if careful search is made for specific histologic structures. Finally, it is suggested that the malignancy rate for gonadal stromal tumors, Sertoli-Leydig tumors, hilus cell tumors and adrenal rest tumors is closer to 5% rather than 25-30%.
Ovarian tumors are the most frequent genital neoplasms found during childhood and adolescence. The authors of this chapter cover feminizing ovarian tumors-including juvenile granulosa cell tumors, nongestational ovarian choriocarcinoma, and others-and masculinizing ovarian tumors such as Sertoli-Leydig cell tumors, gynandroblastoma, and steroid cell tumors.