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

3. SEX CORD-STROMAL TUMORS CLASSIFICATION Granulosa cell tumor –Adult –Juvenile Thecoma-fibroma –Thecoma –Fibroma,sarkoma –Sclerosing stromal tumor Sertoli cell Leydig cell Sertoli- Leydig Stromal luteoma Leydig cell Hilus cell Leydig cell Other 1-GRANULOSA-STROMAL 2-SERTOLI-STROMAL3-STEROID CELL 4-SEX CORD TUMOR WITH ANULAR TUBULES 5-UNCLASSIFIED 6-GYNANDROBLASTOMA

4. SEX CORD-STROMAL TUMORS CLASSIFICATION GRANULOSA-THECA CELL –JUVENILE –ADULT ANDROBLASTOMA OTHERS

5. 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.

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

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

8. 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. GRANULOSA CELL TUMORS ADULT TYPE

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

10. PROGNOSTIC FACTORS –Tumor size –Rupture –Histologic subtype –Nuclear atypia –Mitotic activity –Ploidy status –p53 expression GRANULOSA CELL TUMORS ADULT TYPE

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

12. Ninety percent of GCTs diagnosed in prepubertal girls are juvenile type. Clinical behavior is different from adult type GRANULOSA CELL TUMORS JUVENILE TYPE

13. Breast enlargement Pubic and axillary hair Advanced somatic development Vaginal bleeding Increasing abdominal girth Abdominal pain –Spontaneous rupture Rarely virilization syndrome GRANULOSA CELL TUMORS JUVENILE TYPE PREPUBERTAL SYMPTOMS

14. JUVENILE GRANULOSA CELL TUMORS STAGE AT DIAGNOSIS %

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

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

17. 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? SEX CORD-STROMAL TUMORS SERTOLI- LEYDIG CELL TUMORS

18. SEX CORD-STROMAL TUMORS STEROID CELL TUMORS 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) 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.

19. SEX CORD-STROMAL TUMORS TREATMENT Definitive management –Surgical stage –Desire of future childbearing –Histologic subtype –Patients age

20. Midline incision Peritoneal washings Abdominal exploration,inspection,palpation Multiple biopsies Omentectomy Retroperitoneal sampling/dissection SEX CORD-STROMAL TUMORS TREATMENT

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

22. CYTOREDUCTİVE SURGERY SEX CORD-STROMAL TUMORS TREATMENT

23. Postoperative management must be individualized. –Adjuvant chemotherapy –Radiotherapy –Hormonal therapy –Expectant therapy ?

24. Bleomycin Etoposide CisPlatinum SEX CORD-STROMAL TUMORS TREATMENT Platinum based chemotherapy Bleomycin Vinblastine CisPlatinum Taxol CisPlatinum

25. Days Bleomycin 20 units/m 21 Etoposide75 mg/m 21-5 CisPlatinum20 mg/m 21-5 Every 3 weeks, 4 courses GRANULOSA CELL TUMORS TREATMENT Homesly,1999. A GOG study)

26. 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 TUMORS TREATMENT Homesly,1999. A GOG study)

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

28. Case reports demonstrated that........JGCTs were highly resistant to chemotherapy..... JUVENILE GRANULOSA CELL TUMORS TREATMENT 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

29. SEX CORD-STROMAL TUMORS ÇUMF OVARIAN CARCINOMA,256 CASES

30. SEX CORD-STROMAL TUMORS ÇUMF STAGEs

31. SEX CORD-STROMAL TUMORS SURVIVAL ÇÜMF –Stage 1 and 216/16%100 –Stage 3c1/5 (4of 9?)%20 »Ex.. 24,52,54(myocardiopathy), 58 mounths. –Stage 41/5 %20 » Ex.. 2 Post op, 13, 49 months

32. SEX CORD-STROMAL TUMORS