GATA-4 and FOG-2 Expression in Pediatric Ovarian Sex Cord-Stromal Tumors (SCST): An Italian Multi-Institutional Cooperative Study
C Virgone, G Cecchetto, G Bisogno, P Dall'Igna, V D'Onofrio, R Boldrini, P Collini, R Alaggio. University of Padova, Padova, Italy; Ospedale Pausilipon, Napoli, Italy; Ospedale B. Gesù, Roma, Italy; Istituto Nazionale Tumori, Milano, Italy
Background: GATA-4 is a transcription factor regulating cell differentiation and proliferation: in the ovary it controls the expression of α-inhibin and anti-Müllerian Hormone (MIH). FOG-2 activates or inhibits the transcriptional activity of GATA-4, counteracting the trans-activation of the MIH gene by GATA-4 in fetal ovary. A prognostic role of GATA-4 in adult ovarian Granulosa Cell Tumor has been reported. Pediatric SCST are less than 8-10% of all ovarian tumors, with the most frequent histotypes represented by Juvenile Granulosa Cell Tumors (JGCT) and Sertoli-Leydig Cell tumors (SLCT). We explored the potential pathogenetic and prognostic role of FOG-2 and GATA-4 in pediatric SCST.
Design: Histological slides from 15 SCST entered into TREP-study (an Italian multi-institutional study for rare tumors) were reviewed, and immunostains for GATA-4, FOG-2 and α-inhibin were performed. Clinical information were retrieved from TREP files.
Results: Clinico-pathologic features are summarized in the table. Mean age was 112 months (range 7-224); 1 tumor was bilateral, 12 were stage I, 2 stage II, 1 was metastatic at diagnosis. Mean follow-up was 26 months.
|6 JGCT||5/6||1/5||3/6||6 I CR|
|6 SLCT||4*/6||2/4||5/6||4 I CR, 1 II CR(after metachronous ovarian tumor), 1DOD|
|FT/SST||2/3||1/1||0/3||3 I CR|