Gonadal sex cord stromal tumors

Transcription

1 TREP Meeting Genova June 2009 Gonadal sex cord stromal tumors The Italian experience

2 TREP project (January 2000-April 2009) 32 Gonadal Sex Cord Stromal Tumors 21 Ovary (age 7-176m) 11 Testis (age 1-171m)

3 Ovarian SCST: staging and treatment STAGE (CCG/POG) Stage I Stage II Stage III Stage IV Hidden disease FEATURES Disease limited to the ovary (or both) and completely excised; negative peritoneal washing. No clinic, surgical or histologic evidence of disease extending beyond the ovary and tumoral markers' and/or hormons' levels in range after surgery. Microscopic residuals, spillage or nodes affected by disease (<2 cm); negative peritoneal washing. Tumoral markers positive or negative. Macroscopic residuals or initial biopsy only; local invasion (omentum, bowel, bladder); positve peritoneal washing; nodes affected (>2cm). Tumoral markers positive or negative Distant metastases. Negative or positive markers Stage I but tumoral markers persistently out of normal range after a complete surgery. TREATMENT Wait and see PEB x 3 cycles PEB x 4 cycles, then surgery if possible PEB x 4 cycles, then surgery (metastasis included) if possible PEB x 4 cycles, then surgery (metastasis included) if possible

4 Testicular SCST: staging and treatment STAGE (CCG/POG) Stage I Stage II Stage III FEATURES Disease limited to the testis and completely excised via inguinotomy. Tumoral markers' and/or hormons' levels in range after surgery. Negative histological examination after hemyscrotectomy (performed because of transscrotal approach at first surg.) Microscopic residuals, nodes affected by disease (<2 cm) or transcrotal orchiectomy with spillage. Tumoral markers positive or negative. Positive histological examination after hemyscrotectomy Nodes affected (>2cm). Tumoral markers positive or negative TREATMENT Wait and see PEB x 3 cycles PEB x 4 cycles, then surgery if possible Stage IV Hidden disease Distant metastasis. Negative or positive markers Stage I but tumoral markers persistently out of normal range after a complete surgery. PEB x 4 cycles, then surgery (metastasis included) if possible PEB x 4 cycles, then surgery (metastasis included) if possible

5 TREP results: 21 ovary - SCST Pts Age Endocrine Symptoms Side Surgery CT Stage and Outcome (FU) JGCT m (7-167) 8 (precocious puberty) 8 left 4 right 4 ovariectomy 8 adnectomy 2 10 st. I 11 CR 2 st. II (33 mo; 3-86) SLCT m (40-176) 0 4 left 1 bil 6 adnectomy 2 1 st. I 2nd CR (metacronu relapse) 3 st. II 2 CR, 1 DOD* 1 st. IV CR* (14; 3-32) Sclerosing stromal t ,133 m 0 1 right 1 bil 1 ovariectomy 1 adnectomy (+ contralat. Enucleation) 0 1 st. I 2 CR 1 st. II (12, unk.) Thecoma 2 59 and 175 mo 0 1 left 1 right 1 ovariectomy 1 adnectomy 0 2 st. I 2 CR (12 and 3 mo) *CT was delivered in these cases

6 TREP results: 11 testis - SCST Pts Mean age (range) Endocrine Symptoms Side Surgery Stage and Outcome (FU) JGCT mo (82-139) 1 (precocious puberty) 2 right 2 left 3 orchifunicolectomy 1 enucleation 3 st. I 4 CR 1 st. II (33; 12-52) (trans-scrotal approach) LCT 4 8 mo (2-23) 0 3 right 1 left 1 orchifunicolectomy 3 enucleation 4 st. I 4 CR (47; 12-77) SCT 1 3 mo 0 1 left Orchifunicolectomy 1 st. I 1 CR (52) SCST incompl. diff. 2 1 mo; 171 mo 0 1 left Orchifunicolectomy 2 st. I 2 CR (92;6)

7 Ovary: results (follow-up 24 months range 3-86) 21 cases: first CR 19 second CR 1 (after contralateral ovarian relapse- after 24 m) DOD 1 (PD with metastases after 32 mo) Outcome by Histology: - JGCT: 12/12 first CR - SLCT: 3/5 first CR 1 second CR 1 DOD - Others: 4/4 first CR

8 11 cases Testis: results (follow-up 47 months - range12-92) first CR 11 Outcome by Histology: - JGCT: 4/4 first CR (3 enucl., 1 with a scrotal approach) - LCT: 4/4 (1 enucl.) - SCT: 1/1 - Others: 2/2

9 Remarks General good compliance to guidelines Ovary (21 cases): - Endocrine symptoms only in JGCT - 2/21 bilateral (1 synchronous, 1 metachronous) - 6/21 St.II: 3 local invasion, 1 spillage, 1 microres, 1 pre-op rupture - Events in 2/5 SLCT Testis (11 cases): - Excellent prognosis - Efficacy of enucleation (also through scrotal approach: 1 case)

10 Forms : differences TREP: gonadal non GCT German study: ovarian SLCT Same forms for all ovarian and testicular SCST; also epithelial t are considered Lansky/Karnofsky score before and after treament; body measurements and Tanner score POG staging system, adopted also for Italian MGCT Study detailed diagnostic work-up form notes available in every section (diagnostic work-up, treatment, follow-up, events) FIGO staging system Inhibin is requested Histological form specific for ovarian SLCT

11 Prossimo futuro In corso analisi clinica (Bernini, Virgone, Cecchetto) In corso rivalutazione istologica/biologica (Boldrini, Alaggio)

12 Missing Data Paziente Centro Diagnosi Dati mancanti Ippoliti Fabiola 20if260 OP Bambin Gesù SLCT CT: quale e quanti cicli?; FU Marcini Denise 12md414 OP Bambin Gesù JGCT Tutto (solo registrazione) Mattei Claudia 12mc415 OP Bambin Gesù GCT adult type Tutto (solo registrazione) Durante Chiara 01dc050 INT Milano SLCT Esami strumentali ed ematologici; FU Delithanassis Elena 15de151 Trieste Sclerosante stromale FU Galizzi Linda 37gl003 Bergamo JGCT FU Visani Letizia 32vl478 OP Gaslini SLCT CT: eseguita?; FU Trappolini Noemi OP Bambin Gesù Non noto Non registrata

13 Gonadal epithelial tumors Histotype Pts Mean Age Symptoms Side Surgery CT Stage and Outcome (FU) Mucinous Cistoadenoma (22-183) Abdominal pain (4) Palpable mass (1) Ascites (1) Occasional (1) 4 sx 2 dx 1 bil* 4 adnectomy 1 ovarectomy 1 enucleation 1 only biopsy (bil)* no 6 st. I RC 1 st. III AWD (32; 5-64) Serous Cistoadenoma and 195 Abdominal pain (1) Palpable mass (1) 1 dx 1 sx 1 adnectomy 1 enucleation (then adnectomy as PRE) no 1 st. I RC 1 st. III RC (15 and 64) Gonadoblastoma 1 5 Occasional diagnosis Sx 1 adnectomy no 1 st. I RC *Proteus syndrome: metachronous Wilms tumor (st. IV)