SEX CORD-STROMAL TUMORS

1. SEX CORD-STROMAL TUMORS Dr.Aytekin Altıntaş ADANA

2. Granulosa cell tumor Adult Juvenile Thecoma-fibroma Thecoma Fibroma,sarkoma Sclerosing stromal tumor SEX CORD-STROMAL TUMORSCLASSIFICATION 1-GRANULOSA-STROMAL 2-SERTOLI-STROMAL 3-STEROID CELL • Sertoli cell • Leydig cell • Sertoli- Leydig • Stromal luteoma • Leydig cell • Hilus cell • Leydig cell • Other 4-SEX CORD TUMOR WITH ANULAR TUBULES 6-GYNANDROBLASTOMA 5-UNCLASSIFIED

3. SEX CORD-STROMAL TUMORS CLASSIFICATION • GRANULOSA-THECA CELL • JUVENILE • ADULT • ANDROBLASTOMA • OTHERS

4. SEX CORD-STROMAL TUMORS • SCTSs are 7% of all malignant ovarian neoplasm • The vast majority of these tumors are of low malignant potential or benign. • Long term prognosis is good. • Excessive estrogen production influences end organ responses. • Endometrial and breast cancer must be remembered.

5. In all ovarian malignancies In malignant sex cord-stromal tumors GRANULOSA CELL TUMORS %70

6. SYMPTOMS Abnormal vaginal bleeding Abdominal distension Abdominal pain Rarely,virilizing effect The adult type is 95% of all GCT GRANULOSA CELL TUMORSADULT TYPE

7. GRANULOSA CELL TUMORSADULT TYPE • Eighty percent of patients with GCT will present with stage I. • Prognostically similar to epithelial borderline neoplasms of the ovary. • Mean interval to recurrences is 8,9 years.

8. ADULT GRANULOSA CELL TUMORSSURVIVAL • Overall 5-year survival rates are nearly 90%. • In patients with extraovarian spread at the time of diagnosis, 5-year survival is 33-53%.

9. GRANULOSA CELL TUMORSADULT TYPE • PROGNOSTIC FACTORS • Tumor size • Rupture • Histologic subtype • Nuclear atypia • Mitotic activity • Ploidy status • p53 expression

10. TUMOR MARKERS Serum estrogens Inhibin Follicle-regulatory protein Mullerian inhibitory substance Elevated Inhibin Menstrual cycle Pregnancy Other tumors GRANULOSA CELL TUMORS

11. GRANULOSA CELL TUMORSJUVENILE TYPE • Ninety percent of GCTs diagnosed in prepubertal girls are juvenile type. • Clinical behavior is different from adult type

12. GRANULOSA CELL TUMORSJUVENILE TYPEPREPUBERTAL SYMPTOMS • Breast enlargement • Pubic and axillary hair • Advanced somatic development • Vaginal bleeding • Increasing abdominal girth • Abdominal pain • Spontaneous rupture • Rarely virilization syndrome

13. JUVENILE GRANULOSA CELL TUMORSSTAGE AT DIAGNOSIS %

14. SEX CORD-STROMAL TUMORS • TUMORS IN THECOMA-FIBROMA GROUP • Thecoma • Fibroma, Fibrosarkoma • Sclerosing stromal tumor

15. Less than 0.02% of all ovarian tumors. The avarage patient age is 25 years. Symptoms Defeminization Virilization Abdominal mass Abdominal pain SEX CORD-STROMAL TUMORSSERTOLI- LEYDIG CELL TUMORS

16. SEX CORD-STROMAL TUMORSSERTOLI- LEYDIG CELL TUMORS • Stage is the most important prognostic factor. • Despite an average size of 16 cm,only 2-3% of SLCTs have extraovarian disease-Low malign potential..... • Testesteron, inhibin, AFP-Tumor marker?

17. SCTs constitute only 0.1% of all avarian neoplasms. Old name:Lipid-cell tumors. Stromal luteoma and Leydig Cell tumor are always benign. Stromal luteoma Leydig cell tumor Hilus cell Leydig cell Steroid cell tumors not otherwised specified. (SCTNOS) SEX CORD-STROMAL TUMORSSTEROID CELL TUMORS • SCT-NOS • Avarage age... 47 • Produce Hormones, • Testesterone • Estrogen • Corticosteroids • Sometimes, nonpalpable • In adults,25-43% are malignant • SCTNOS are not malignant less • than 20 years of age.

18. SEX CORD-STROMAL TUMORSTREATMENT • Definitive management • Surgical stage • Desire of future childbearing • Histologic subtype • Patients age

19. SEX CORD-STROMAL TUMORSTREATMENT • Midline incision • Peritoneal washings • Abdominal exploration,inspection,palpation • Multiple biopsies • Omentectomy • Retroperitoneal sampling/dissection

20. Most of the SCSTs are bening. Surgical therapy such as USO or BSO with TAH is sufficient therapy. Thecoma Fibroma Gynandroblastoma Stromal luteoma Leydig cell Sclerosing stromal Sertoli cell SEX CORD-STROMAL TUMORSTREATMENT

21. SEX CORD-STROMAL TUMORSTREATMENT CYTOREDUCTİVE SURGERY

22. SEX CORD-STROMAL TUMORSTREATMENT • Postoperative management must be individualized. • Adjuvant chemotherapy • Radiotherapy • Hormonal therapy • Expectant therapy ?

23. Bleomycin Etoposide CisPlatinum SEX CORD-STROMAL TUMORSTREATMENT Platinum based chemotherapy Taxol CisPlatinum Bleomycin Vinblastine CisPlatinum

24. Days Bleomycin 20 units/m2 1 Etoposide 75 mg/m2 1-5 CisPlatinum 20 mg/m2 1-5 Every 3 weeks, 4 courses GRANULOSA CELL TUMORSTREATMENT Homesly,1999. A GOG study)

25. Eight years, 75 patients, 18 of them ineligible. BEP regimen Thirty-eight SLL Fourteen (%37) negative second look. Myelotoxicity in %61 of patients Two deaths were attributed to bleomycin Three-year survival is more than %75 GRANULOSA CELL TUMORSTREATMENT Homesly,1999. A GOG study)

26. Between 1948-1988 Fourteen of 34 patients were included. Complete response in 6 patients (43%). Three of 6 (50%) had recurred ADVANCED GRANULOSA CELL TUMORSADJUVANT RADIOTHERAPY CONCLUSION Radiotherapy can induce a clinical response with OCCASIONAL long-term remission... Wolf,1999

27. JUVENILE GRANULOSA CELL TUMORSTREATMENT Case reports demonstrated that... .....JGCTs were highly resistant to chemotherapy ..... • Two cases , Stage III C. • USO,Omentectomy,PPLND, apendectomy. • Carboplatin 400mg/m2 and Etoposide 120 mg/ . • They were alive without disease during 39 and 12 months • of follow -up . • Powell, Gynecologic Oncology,1997

28. SEX CORD-STROMAL TUMORSÇUMF OVARIAN CARCINOMA ,256 CASES

29. SEX CORD-STROMAL TUMORSÇUMFSTAGEs

30. SEX CORD-STROMAL TUMORSSURVIVALÇÜMF • Stage 1 and 2 16/16 %100 • Stage 3c 1/5 (4of 9?) %20 • Ex.. 24 ,52,54(myocardiopathy), 58 mounths. • Stage 4 1/5 %20 • Ex .. 2 Post op , 13, 49 months

31. SEX CORD-STROMAL TUMORS