FOXL2 and SOX9 distinguish the lineage of the sex cord-stromal cells in gonadoblastomas

Rebecca Buell-Gutbrod, Marina Ivanovic, Anthony Montag, Ernst Lengyel, Oluwole Fadare, Katja Gwin

Research output: Contribution to journalArticle

15 Citations (Scopus)

Abstract

Gonadoblastomas are mixed germ cell sex cord-stromal tumors that arise in dysgenetic gonads and are composed of immature germ cells and sex cord-stromal cells of indeterminate differentiation. FOXL2 is one of the first genes expressed in female gonad development, and it is required for proper granulosa cell differentiation during folliculogenesis. SOX9, a downstream target of SRY, the gene in the Y chromosomal sex-determining region, is required for testicular development and for the formation and maintenance of (pre-)Sertoli cells. This study characterized the sex cord-stromal cells of gonadoblastoma by evaluating the expression of these counteracting transcription factors. Archival paraffin-embedded material of 7 gonadoblastomas, 5 of which were overgrown by dysgerminoma, was examined by immunohistochemistry for expression and localization of FOXL2 and SOX9. The sex cord-stromal cells revealed strong nuclear staining for FOXL2 and were negative for SOX9 expression. Germ cells in the gonadoblastoma and dysgerminoma components showed no FOXL2 and SOX9 expression. Areas of transition between gonadoblastoma and dysgerminoma revealed nests with a gradual reduction of FOXL2 expression. Our results support the hypothesis that the sex cord-stromal cell component of gonadoblastomas is of granulosa cell origin. In addition, FOXL2 appears to be a useful marker for the evaluation of overgrowth by dysgerminomas and for the identification of the transition zone of "dysgerminoma in situ." As FOXL2 and SOX9 are differentially expressed, they also should be useful for distinguishing gonadoblastomas from intratubular germ cell neoplasias and can help to differentiate those with a Sertoli cell component from gonadoblastoma with a granulosa cell component.

Original languageEnglish (US)
Pages (from-to)391-395
Number of pages5
JournalPediatric and Developmental Pathology
Volume14
Issue number5
DOIs
StatePublished - 2011

Fingerprint

Gonadoblastoma
Stromal Cells
Dysgerminoma
Granulosa Cells
Cellular Structures
Germ Cells
Sertoli Cells
Gonads
Cell Differentiation
Sex Cord-Gonadal Stromal Tumors
Germ Cell and Embryonal Neoplasms
Paraffin
Genes
Transcription Factors
Immunohistochemistry
Maintenance
Staining and Labeling

Keywords

  • Dysgerminoma
  • FOXL2
  • Gonadoblastoma
  • Intratubular germ cell neoplasia
  • Mixed germ cell/sex cord-stromal cell tumors
  • SOX9

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Pathology and Forensic Medicine

Cite this

FOXL2 and SOX9 distinguish the lineage of the sex cord-stromal cells in gonadoblastomas. / Buell-Gutbrod, Rebecca; Ivanovic, Marina; Montag, Anthony; Lengyel, Ernst; Fadare, Oluwole; Gwin, Katja.

In: Pediatric and Developmental Pathology, Vol. 14, No. 5, 2011, p. 391-395.

Research output: Contribution to journalArticle

Buell-Gutbrod, Rebecca ; Ivanovic, Marina ; Montag, Anthony ; Lengyel, Ernst ; Fadare, Oluwole ; Gwin, Katja. / FOXL2 and SOX9 distinguish the lineage of the sex cord-stromal cells in gonadoblastomas. In: Pediatric and Developmental Pathology. 2011 ; Vol. 14, No. 5. pp. 391-395.
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