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RARE GYNECOLOGICAL CANCERS Isabelle Ray-Coquard MD, PhD Centre Léon Bérard Lyon, France Stresa, March 2011
Rare Gynecological cancer: ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],There are six potential sites for gynecologic cancer, each with a distinctive anatomy and histology. The cancer can be traced to a derivative cell.  Ovary epithelial neoplasms include the common varieties of adenocarcinomas and also give rise to  a rich variety of germ cell neoplasms.  The uterine wall undergoes dramatic proliferative change monthly in its glandular epithelium during the menstrual cycle, gives rise to adenocarcinomas.  The cervix has a simple columnar epithelium and changes to a stratified squamous epithelium, which gives rise to squamous cell cancers.  The fallopian tube is lined by ciliated columnar epithelium and tends to give rise mainly to serous adenocarcinomas.  Syncytiotrophoblast gives rise to choriocarcinoma, gestational trophoblast tumors.
Rare ovarian cancers  ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Rare endometrial cancers  ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Rare Cervix cancers  ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Rare vulva & vagina cancers  (10% of all gyneco cancers) ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Gestational trophoblastic diseases:  0.57 to 1/100 000 pregnancies; <1% ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Incidence & prevalence by sites
Survival rate by sites
Incidence & survival rates by subgroups
Specificity of rare gynecologic cancer ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Definition of rare gynecologic cancer ? ,[object Object],[object Object],[object Object],[object Object]
1) Example of uterine sarcoma…. Ducimetière F, et al PLoSOne 2011 3 to 5% of all invasive uterine cancers 3% of all STS or visceral sarcoma
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Uterine Sarcoma: Epidemiology
Uterine sarcoma Treatment Algorithm 1st line 3rd line 2nd line *if doxorubicin or ifosfamide not recommended Localised stage Hysterectomy with oophorectomy +/- RT Cure  (40–50%) Metastases  (50–70%) Surgery  (15% ) Doxorubicin Ifosfamide Yondelis Yondelis* Ifosfamide Doxorubicin Yondelis Yondelis Combination ? ? OS 10–12 months PFS 5–8 months ?
Endometrial stromal sarcoma biology & IHC ,[object Object],[object Object],[object Object]
Endometrial stromal sarcoma Treatment Algorithm F Amant, Lancet 2009
Endometrial stromal sarcoma  Hormonal therapy options F Amant, Lancet 10 & Fayette et al, ASCO 06
2) Rare ovarian cancers ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
PRINCIPLE OF INITIAL SURGERY ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],GERM CELL TUMOURS
GERM CELL TUMOURS ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
SEX CORD AND STROMA TUMOURS ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
SEX CORD AND STROMA TUMOURS  ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Relapse risk for GCT ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Survival after relapse ,[object Object],[object Object],[object Object],[object Object],[object Object]
Clinical Prognostic Factors in SCT ,[object Object],[object Object],[object Object],[object Object],[object Object]
Preop rupture:  3/12 pts received CT Intraop rupture: 4/9 pts received CT TUMOR RUPTURE Schneider et al, JCO vol 22, n10, 2004
HISTOLOGIC PROGNOSTIC FACTORS IN GCT ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Controversial no yes?
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Fertility sparing surgery ?
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Chemotherapy in advanced /recurrent sex-cord stromal tumors
Vorinostat  in Stage IV GGCT  Histone Deacetylase (HDAC) Inhibitor  Response  after 11 months of treatment. Prior treatment included  BEP, doxorubicin, tamoxifen, carboplatin, leuprolide, topotecan, paclitaxel, …. Rubin et al, Clin Cancer Res December 1, 2006
Summary for rare ovarian cancers ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],2002: Ovarian rare malignant tumours  national website observatory Private access Public access
RATIONAL for a dedicated Website ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
METHODS ,[object Object],[object Object],[object Object],[object Object]
Clinical study inclusion
2008 RESULTS ,[object Object],Observed inlusions Observed inclusions Expected inclusions
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],37% non agreement Centralized review diagnosis (n=67) Ray-Coquard et al, ESMO 2008
CONCLUSIONS   ,[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object]
2009,  2 nd  step  : National rare Gynecologic cancer network ,[object Object],[object Object],[object Object]
Ministère de la Santé  et des sports  Call for proposal 2010 INCa – DHOS Approval for national referent cancer center dedicated to rare cancers ,[object Object],[object Object],[object Object],[object Object]
Objectives for gynecological projet - care  ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Objectives - research ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
New strategies & new targets Today, more than 250 tumors samples and clinical data for GCT & SCT are available & dedicated to determine new targets ….
List of regional experts centers  CHU Hôpital Civil Institut Bergonié Centre Jean Perrin Centre Georges François Leclerc Centre Eugène Marquis Clinique Armoricaine de Radiologie CHU La Source Institut Jean Godinot CHU Hôpital Jean Minjoz AP-HP Hôpitaux de Paris Institut Gustave Roussy CRLC Val d'Aurelle CHU Dupuytren Centre Alexis Vautrin Centre Claudius Regaud Centre Henri Becquerel Centre François Baclesse Centre Catherine de Sienne CHU de Poitiers Institut Paoli Calmettes Groupe Hospitalier Sud Réunion CHU Hôpital Michalon CHU La Croix Rousse Centre Léon Bérard
Approval  and Financial support by INCA  2010
Example of dedicated CPG’s for mucinous ovarian cancer
 
3) Management & improvements for gestational trophoblastic disease
French Trophoblastic Disease Reference Center Lyon Sud University Hospital ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],Prof. François GOLFIER, MD, PhD – French Trophoblastic Disease Reference Center
Registration of patients to the  trophoblastic disease reference center francois.golfier@chu-lyon.fr, touria.hajri@chu-lyon.fr [email_address] Internet (mole-chorio.com) Prof. François GOLFIER, MD, PhD – French Trophoblastic Disease Reference Center
Conclusions ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
 
Specificity of rare gynecological cancers ,[object Object],[object Object],[object Object],[object Object],[object Object]
Sex cords ovarian cancers ,[object Object],[object Object],[object Object],[object Object],[object Object]

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Rare Solid Cancers: An Introduction - Slide 8 - I. Ray-Coquard - Rare female genital cancers

  • 1. RARE GYNECOLOGICAL CANCERS Isabelle Ray-Coquard MD, PhD Centre Léon Bérard Lyon, France Stresa, March 2011
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  • 10. Incidence & survival rates by subgroups
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  • 13. 1) Example of uterine sarcoma…. Ducimetière F, et al PLoSOne 2011 3 to 5% of all invasive uterine cancers 3% of all STS or visceral sarcoma
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  • 15. Uterine sarcoma Treatment Algorithm 1st line 3rd line 2nd line *if doxorubicin or ifosfamide not recommended Localised stage Hysterectomy with oophorectomy +/- RT Cure (40–50%) Metastases (50–70%) Surgery (15% ) Doxorubicin Ifosfamide Yondelis Yondelis* Ifosfamide Doxorubicin Yondelis Yondelis Combination ? ? OS 10–12 months PFS 5–8 months ?
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  • 17. Endometrial stromal sarcoma Treatment Algorithm F Amant, Lancet 2009
  • 18. Endometrial stromal sarcoma Hormonal therapy options F Amant, Lancet 10 & Fayette et al, ASCO 06
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  • 28. Preop rupture: 3/12 pts received CT Intraop rupture: 4/9 pts received CT TUMOR RUPTURE Schneider et al, JCO vol 22, n10, 2004
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  • 32. Vorinostat in Stage IV GGCT Histone Deacetylase (HDAC) Inhibitor Response after 11 months of treatment. Prior treatment included BEP, doxorubicin, tamoxifen, carboplatin, leuprolide, topotecan, paclitaxel, …. Rubin et al, Clin Cancer Res December 1, 2006
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  • 46. New strategies & new targets Today, more than 250 tumors samples and clinical data for GCT & SCT are available & dedicated to determine new targets ….
  • 47. List of regional experts centers CHU Hôpital Civil Institut Bergonié Centre Jean Perrin Centre Georges François Leclerc Centre Eugène Marquis Clinique Armoricaine de Radiologie CHU La Source Institut Jean Godinot CHU Hôpital Jean Minjoz AP-HP Hôpitaux de Paris Institut Gustave Roussy CRLC Val d'Aurelle CHU Dupuytren Centre Alexis Vautrin Centre Claudius Regaud Centre Henri Becquerel Centre François Baclesse Centre Catherine de Sienne CHU de Poitiers Institut Paoli Calmettes Groupe Hospitalier Sud Réunion CHU Hôpital Michalon CHU La Croix Rousse Centre Léon Bérard
  • 48. Approval and Financial support by INCA 2010
  • 49. Example of dedicated CPG’s for mucinous ovarian cancer
  • 50.  
  • 51. 3) Management & improvements for gestational trophoblastic disease
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  • 53. Registration of patients to the trophoblastic disease reference center francois.golfier@chu-lyon.fr, touria.hajri@chu-lyon.fr [email_address] Internet (mole-chorio.com) Prof. François GOLFIER, MD, PhD – French Trophoblastic Disease Reference Center
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Editor's Notes

  1. 8% de tumerus ovariennes derivent de cordons sexuels et du mésenchyme de la gonade embryonnaire
  2. Several known oncogenes have been investigated as candidates for a role in the pathogenesis of GCT King found no association of c-myc, .. Immunochemistery and prognosis in a panel of 40 GCTs Kalfa et al., found that the extinction of FOXL2 in 26 pts with JGCT was prognosis of relapse vs no for pts with normal expression
  3. Re Page d’accueil …. Annonce du site à tous les membres de TMRO avec login et ancien mot de passe, changeable après à la connexion. Transfert des données des patientes incluses dans TMRO dans cette nouvelle base, avec nouvelle politique … à définir.
  4. Right answer are 1 2
  5. Right answers are 1 3 5