title: Ovarian Sertoli-Leydig cell tumor: a SRY gene-independent pathway of pseudomale gonadal differentiation.
abstract: Sertoli-Leydig cell tumors (SLCT) are rare sex-cord stromal tumors of the ovary composed of undifferentiated gonadal stromal cells, Leydig cells (LC), and Sertoli cells (SC), with the latter forming structures resembling fetal testicular tubules. The histogenetic basis of morphological male differentiation patterns in females is controversial. Here, we report a SLCT with intermediate differentiation in a 23-year-old woman investigated by light microscopy, immunohistochemistry for intermediate filaments, and sex steroid hormone receptors (SSHR), as well as by polymerase chain reaction (PCR) for the presence of the sex-determining region Y gene (SRY). Our investigation shows that the SCs of SLCT express progesterone and androgen receptors as well as cytokeratins and vimentin. By PCR, SLCT-derived genomic DNA lacked the SRY gene, indicating that the SLCT results from a SRY gene-independent pathway of pseudomale gonadal differentiation. The expression of progesterone receptors (PRs) in the SCs of the SLCT is in contrast to their absence in testicular SCs, but in line with their presence in ovarian granulosa and surface epithelial cells. Thus, our results provide strong evidence for a close histogenetic relationship between the SLCT and the female gonocyte-supporting cell, the granulosa cell (GC).
authors.affiliation: Department of Pathology, University of Innsbruck, Medical School, Austria.
authors.forename: A
authors.initials: A
authors.lastname: Hittmair
authors.forename: B G
authors.initials: BG
authors.lastname: Zelger
authors.forename: P
authors.initials: P
authors.lastname: Obrist
authors.forename: S
authors.initials: S
authors.lastname: Dirnhofer
mesh_terms: D000328:Adult; D017931:DNA Primers / Q000737:chemistry; D004268:DNA-Binding Proteins / Q000032:analysis; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D007382:Intermediate Filaments / Q000276:immunology; D007633:Keratins / Q000032:analysis; D009687:Nuclear Proteins; D010051:Ovarian Neoplasms / Q000737:chemistry / Q000235:genetics / Q000473:pathology*; D016133:Polymerase Chain Reaction; D011980:Receptors, Progesterone / Q000097:blood; D018310:Sertoli-Leydig Cell Tumor / Q000737:chemistry / Q000235:genetics / Q000473:pathology*; D019849:Sex Determination Processes; D012733:Sex Differentiation / Q000235:genetics; D051876:Sex-Determining Region Y Protein; D014157:Transcription Factors; D014746:Vimentin / Q000032:analysis
chemical_list: D017931:DNA Primers; D004268:DNA-Binding Proteins; D009687:Nuclear Proteins; D011980:Receptors, Progesterone; C497745:SRY protein, human; D051876:Sex-Determining Region Y Protein; D014157:Transcription Factors; D014746:Vimentin; D007633:Keratins