Title: SEX CORD-STROMAL TUMORS
1SEX CORD-STROMAL TUMORS
ADANA
2SEX CORD-STROMAL TUMORSCLASSIFICATION
1-GRANULOSA-STROMAL
2-SERTOLI-STROMAL
3-STEROID CELL
- 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
4-SEX CORD TUMOR WITH ANULAR TUBULES
6-GYNANDROBLASTOMA
5-UNCLASSIFIED
3SEX CORD-STROMAL TUMORS CLASSIFICATION
- GRANULOSA-THECA CELL
- JUVENILE
- ADULT
- ANDROBLASTOMA
- OTHERS
4SEX 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.
5GRANULOSA CELL TUMORS
- In all ovarian malignancies
- In malignant sex cord-stromal tumors
70
6GRANULOSA CELL TUMORSADULT TYPE
- SYMPTOMS
- Abnormal vaginal bleeding
- Abdominal distension
- Abdominal pain
- Rarely,virilizing effect
- The adult type is 95 of all GCT
7GRANULOSA 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.
8ADULT 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.
9GRANULOSA CELL TUMORSADULT TYPE
- PROGNOSTIC FACTORS
- Tumor size
- Rupture
- Histologic subtype
- Nuclear atypia
- Mitotic activity
- Ploidy status
- p53 expression
10GRANULOSA CELL TUMORS
- TUMOR MARKERS
- Serum estrogens
- Inhibin
- Follicle-regulatory protein
- Mullerian inhibitory substance
- Elevated Inhibin
- Menstrual cycle
- Pregnancy
- Other tumors
11GRANULOSA CELL TUMORSJUVENILE TYPE
- Ninety percent of GCTs diagnosed in prepubertal
girls are juvenile type. - Clinical behavior is different from adult type
12GRANULOSA 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
13JUVENILE GRANULOSA CELL TUMORSSTAGE AT DIAGNOSIS
14SEX CORD-STROMAL TUMORS
- TUMORS IN THECOMA-FIBROMA GROUP
- Thecoma
- Fibroma, Fibrosarkoma
- Sclerosing stromal tumor
15SEX CORD-STROMAL TUMORSSERTOLI- LEYDIG CELL
TUMORS
- Symptoms
- Defeminization
- Virilization
- Abdominal mass
- Abdominal pain
- Less than 0.02 of all ovarian tumors.
- The avarage patient age is 25 years.
16SEX 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?
17SEX 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.
- SCTs constitute only 0.1 of all avarian
neoplasms. - Old nameLipid-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)
18SEX CORD-STROMAL TUMORSTREATMENT
- Definitive management
- Surgical stage
- Desire of future childbearing
- Histologic subtype
- Patients age
19SEX CORD-STROMAL TUMORSTREATMENT
- Midline incision
- Peritoneal washings
- Abdominal exploration,inspection,palpation
- Multiple biopsies
- Omentectomy
- Retroperitoneal sampling/dissection
20SEX CORD-STROMAL TUMORSTREATMENT
- 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
21SEX CORD-STROMAL TUMORSTREATMENT
22SEX CORD-STROMAL TUMORSTREATMENT
- Postoperative management must be individualized.
- Adjuvant chemotherapy
- Radiotherapy
- Hormonal therapy
- Expectant therapy
?
23SEX CORD-STROMAL TUMORSTREATMENT
Platinum based chemotherapy
Taxol CisPlatinum
- Bleomycin
- Etoposide
- CisPlatinum
Bleomycin Vinblastine CisPlatinum
24GRANULOSA CELL TUMORSTREATMENT
- Days
- Bleomycin 20 units/m2 1
- Etoposide 75 mg/m2 1-5
- CisPlatinum 20 mg/m2 1-5
- Every 3 weeks, 4 courses
Homesly,1999. A GOG study)
25GRANULOSA CELL TUMORSTREATMENT
- 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
Homesly,1999. A GOG study)
26ADVANCED GRANULOSA CELL TUMORSADJUVANT
RADIOTHERAPY
- Between 1948-1988
- Fourteen of 34 patients were included.
- Complete response in 6 patients (43).
- Three of 6 (50) had recurred
CONCLUSION Radiotherapy can induce a clinical
response with OCCASIONAL long-term remission...
Wolf,1999
27JUVENILE 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
28SEX CORD-STROMAL TUMORSÇUMF OVARIAN CARCINOMA
,256 CASES
29SEX CORD-STROMAL TUMORSÇUMFSTAGEs
30SEX 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
31SEX CORD-STROMAL TUMORS