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  • Nicoletta ColomboNicoletta Colombo

    Sex-Cord stromal tumorsSex-Cord stromal tumors

  • Sex cord-stromal tumors account Sex cord-stromal tumors account for approximately 5% of all for approximately 5% of all ovarian cancers and for the ovarian cancers and for the

    majority of functioning tumors majority of functioning tumors with clinical manifestationswith clinical manifestations

  • Classification of sex cord Classification of sex cord stromal tumorsstromal tumors

    A) Granulosa-Stromal Cell Tumors A) Granulosa-Stromal Cell Tumors 1 - Granulosa cell tumors1 - Granulosa cell tumors 2 - Tumors in the thecoma-fibroma group2 - Tumors in the thecoma-fibroma group

    B) Sertoli-Leydig cell tumors B) Sertoli-Leydig cell tumors 1 - Well differentiated1 - Well differentiated

    2 - Of intermediated differentiation2 - Of intermediated differentiation 3 - Poorly differentiated3 - Poorly differentiated 4 - With heterologous elements4 - With heterologous elements

    C) Gynadroblastoma C) Gynadroblastoma D) Sex Cord with anular tubules D) Sex Cord with anular tubules E) UnclassifiedE) Unclassified

  • EpidemiologyEpidemiology • 1.5% of all ovarian tumors1.5% of all ovarian tumors • 3-5% of all ovarian cancer 3-5% of all ovarian cancer • Any ageAny age • Peri and early post-menopausal (median age: 50-54)Peri and early post-menopausal (median age: 50-54) • 5% premenarchal5% premenarchal • 0.99 per 100,000 in USA0.99 per 100,000 in USA • 0.4-1.7 per 100,000 in developed countries0.4-1.7 per 100,000 in developed countries • 25% endometrial hyperplasia25% endometrial hyperplasia • 5-10% endometrial cancer5-10% endometrial cancer

    GRANULOSA CELL TUMORS

  • GCT: Adult and Juvenile typesGCT: Adult and Juvenile types

    Adult 95% Juvenile 5%Adult 95% Juvenile 5% < 1% premenarchal< 1% premenarchal 50% premenarchal50% premenarchal Usual after 30 yrsUsual after 30 yrs Rare after 30 yrsRare after 30 yrs

    90% Stage I90% Stage I

    from Young & Scully, 1984from Young & Scully, 1984

  • GRANULOSA CELL TUMORSGRANULOSA CELL TUMORS

    Endocrine manifestations:Endocrine manifestations: • ChildhoodChildhood: isosexual precocious pseudopuberty: isosexual precocious pseudopuberty

    • Reproductive ageReproductive age: menstrual irregularities/ : menstrual irregularities/ secondary amenorrhea, infertility, rarely virilizationsecondary amenorrhea, infertility, rarely virilization

    • Post-menopausalPost-menopausal: abnormal vaginal bleeding: abnormal vaginal bleeding  Endometrial hyperplasia 25%Endometrial hyperplasia 25%  Endometrial adenocarcinoma 5-10%Endometrial adenocarcinoma 5-10%

  • GCT TREATMENTGCT TREATMENT

    Surgery as Primary TreatmentSurgery as Primary Treatment::

     TAH/BSO + Surgical Staging TAH/BSO + Surgical Staging  Role of Fertility-Sparing SurgeryRole of Fertility-Sparing Surgery

    Adjuvant Treatment (?)Adjuvant Treatment (?)::

     Chemotherapy (CT)Chemotherapy (CT)  Radiotherapy (RT)Radiotherapy (RT)  Hormonal Therapy (HT)Hormonal Therapy (HT)

  • Staging and Restaging • Complete surgical staging in 1/5 Complete surgical staging in 1/5

    women with GCT women with GCT • No nodal metastases identified in No nodal metastases identified in

    those surgically stagedthose surgically staged • 15% of first recurrences appear to 15% of first recurrences appear to

    involve the retroperitoneuminvolve the retroperitoneum

    Abu Rustum, Gynecol Oncol, March 2006Abu Rustum, Gynecol Oncol, March 2006

  • GCT- IEO cases GCT- IEO cases RESTAGINGRESTAGING

    STAGE at 1ST SurgerySTAGE at 1ST Surgery After Restaging After Restaging

    IA (15 pts)IA (15 pts) 14 IA (93.3%)14 IA (93.3%) 1 IIB (6.7%)1 IIB (6.7%)

    IC (9 pts)IC (9 pts)

    7 IC (77.8%)7 IC (77.8%)

    1IIIB (11.1%)1IIIB (11.1%) 1IIIC (11.1%)1IIIC (11.1%)

  • Fertility-sparing surgery

    • 134 young patients ( 134 young patients (

  • GCTGCT

    Who should you Who should you treat after surgery?treat after surgery?

  • Postoperative TherapyPostoperative Therapy

    • Due to the rarity of the disease it is still unknown whether the Due to the rarity of the disease it is still unknown whether the use of postoperative treatment in patients with high risk GCT use of postoperative treatment in patients with high risk GCT can actually confer a survival advantagecan actually confer a survival advantage

    • Postoperative treatment decisions are based on:Postoperative treatment decisions are based on: – Estimated risk of relapseEstimated risk of relapse – Observation that adjuvant treatment can Observation that adjuvant treatment can improve improve

    DFS DFS – Availability of agents with known activity in GCTAvailability of agents with known activity in GCT

  • Recurrence Rate in GCTRecurrence Rate in GCT AUTHORSAUTHORS N°casesN°cases N°rec. N°rec. %%

    SchwartzSchwartz 3737 66 16.216.2

    StenwigStenwig 118118 2424 21.221.2

    EvansEvans 118118 2222 18.618.6

    Kim 34 3 8.6 Kim 34 3 8.6

    TotalTotal 307307 5555 17.917.9

  • Survival after RecurrenceSurvival after Recurrence

    AUTHORSAUTHORS IntervalInterval SurvivalSurvival

    SchwartzSchwartz 1-9 yrs1-9 yrs 19%19% PanckratzPanckratz 13%13% StenwigStenwig 1-22 yrs1-22 yrs 13%13% EvansEvans 1-23 yrs1-23 yrs 27%27%

  • Clinical Prognostic Factors in GCTClinical Prognostic Factors in GCT

    • StageStage • AgeAge • Tumor SizeTumor Size • BilateralityBilaterality • RuptureRupture

  • Survival Rates by StageSurvival Rates by Stage

    FIGO stage FIGO stage 5-yrs5-yrs 10-yrs10-yrs Survival (%)Survival (%) Survival (%)Survival (%)

    II 90-10090-100 84-9584-95 IIII 55-7555-75 50-6550-65 III-IVIII-IV 22-5022-50 17-5717-57

  • Disease-Specific Survival by StageDisease-Specific Survival by Stage

    Zhang M et al. Gynecol Oncol 2007Zhang M et al. Gynecol Oncol 2007

  • Age and Prognosis in GCTAge and Prognosis in GCT

    AuthorAuthor SurvivalSurvival < 40 yrs< 40 yrs >40 yrs>40 yrs Fox H. et al.Fox H. et al. FavorableFavorable Stenwig et al.Stenwig et al. FavorableFavorable

  • Age and Prognosis in GCTAge and Prognosis in GCT

    Zhang M et al. Gynecol Oncol 2007Zhang M et al. Gynecol Oncol 2007

  • Event-Free Survival by AgeEvent-Free Survival by Age

    Schneider et al, JCO vol 21, 2003Schneider et al, JCO vol 21, 2003

  • Tumor Size and Prognosis in GCTTumor Size and Prognosis in GCT

    AuthorAuthor SurvivalSurvival 15cm FoxFox 100%100% 64%64% 61%61% StenwigStenwig 73%73% 63%63% 34%34% BjorkholmBjorkholm 100%100% 92%92% Zhang 91% 89%Zhang 91% 89%

    Difference not significant after correcting for stageDifference not significant after correcting for stage

  • Clinical Features in GCT: BilateralityClinical Features in GCT: Bilaterality AUTHORSAUTHORS

    MansellMansell NorrisNorris NovakNovak

    FoxFox SchwartzSchwartz PankratzPankratz StenwigStenwig EvansEvans

    N°casesN°cases 8080 9797

    307307 9292 3737 6161

    118118 1111

    Bilater.Bilater. 44 22 99 77 11 55 66 33

    %% 5.05.0 2.12.1 2.92.9 7.67.6 2.72.7 8.28.2 5.15.1 2.52.5

    TOTAL TOTAL 910 37 4.1910 37 4.1

  • Bilaterality and Prognosis in GCTBilaterality and Prognosis in GCT

    AUTHORSAUTHORS DOD/BILATDOD/BILAT..

    DiddleDiddle 38/5738/57 NorrisNorris 1/21/2 FoxFox 6/76/7 StenwigStenwig 5/65/6 TotalTotal 50/7250/72

  • Rupture and Prognosis in GCTRupture and Prognosis in GCT

    AUTHORSAUTHORS DOD/RuptureDOD/Rupture

    DinnersteinDinnerstein NONO SchwartzSchwartz NONO BjorkholmBjorkholm YESYES 86% vs 60%86% vs 60%

  • Tumor ruptureTumor rupture

    Schneider et al, JCO vol 21, 2003Schneider et al, JCO vol 21, 2003

  • Preop rupture: 3/12 received CTPreop rupture: 3/12 received CT Intraop rupture: 4/9 received CTIntraop rupture: 4/9 received CT

    Tumor ruptureTumor rupture

    Schneider et al, JCO vol 22,n10, 2004Schneider et al, JCO vol 22,n10, 2004

  • Disease-Specific Survival by GradeDisease-Specific Survival by Grade

    Zhang M et al. Gynecol Oncol 2007Zhang M et al. Gynecol Oncol 2007

  • Clinical prognostis factors Clinical prognostis factors Multivariate analysisMultivariate analysis

    376 patients376 patients Factors Hazard 95% CIFactors Hazard 95% CI p-valuep-value ratio ratio

    Olde