SEX CORD-STROMAL TUMORS
Dr.Aytekin AltıntaşADANA
SEX CORD-STROMAL TUMORSCLASSIFICATION
• 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-STROMAL 3-STEROID CELL
4-SEX CORD TUMOR WITH ANULAR TUBULES
5-UNCLASSIFIED 6-GYNANDROBLASTOMA
SEX CORD-STROMAL TUMORS CLASSIFICATION
• GRANULOSA-THECA CELL– JUVENILE– ADULT
• ANDROBLASTOMA
• OTHERS
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.
GRANULOSA CELL TUMORS
• In all ovarian malignancies
• In malignant sex cord-stromal tumors
%70
%30
GCT OTHER
%70
GRANULOSA CELL TUMORSADULT TYPE
SYMPTOMS • Abnormal vaginal
bleeding• Abdominal distension• Abdominal pain• Rarely,virilizing effect
%95
%5
ADULT JUVENILE
• The adult type is 95% of all GCT
In ÇUMF;31 CASES
%97
%3
ADULT JUVENILE
• 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 TUMORSADULT TYPE
LITERATURE
%80
%20
Stage I OTHER
ÇUMF
%35
%65
• 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 TUMORSSURVIVAL
ÇUMF
0
20
4060
80
100
120
0 1.yıl 2 3 4 5
Overall With extraovarian disease
• PROGNOSTIC FACTORS– Tumor size– Rupture– Histologic subtype– Nuclear atypia– Mitotic activity– Ploidy status– p53 expression
GRANULOSA CELL TUMORSADULT TYPE
• TUMOR MARKERS
– Serum estrogens
– Inhibin
– Follicle-regulatory protein
– Mullerian inhibitory substance
GRANULOSA CELL TUMORS
• Elevated Inhibin– Menstrual cycle
– Pregnancy
– Other tumors
• Ninety percent of GCTs diagnosed in prepubertal girls are juvenile type.
• Clinical behavior is different from adult type
GRANULOSA CELL TUMORSJUVENILE TYPE
• Breast enlargement
• Pubic and axillary hair
• Advanced somatic development
• Vaginal bleeding
• Increasing abdominal girth
• Abdominal pain– Spontaneous rupture
• Rarely virilization syndrome
GRANULOSA CELL TUMORSJUVENILE TYPE
PREPUBERTAL SYMPTOMS
JUVENILE GRANULOSA CELL TUMORSSTAGE AT DIAGNOSIS
0
10
20
30
40
50
60
70
80
90
Stage 1a Stage 1B Stage 1C Stage 2
%
SEX CORD-STROMAL TUMORS
• TUMORS IN THECOMA-FIBROMA GROUP
– Thecoma
– Fibroma, Fibrosarkoma
– Sclerosing stromal tumor
SEX CORD-STROMAL TUMORSSERTOLI- 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
• 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 TUMORSSERTOLI- LEYDIG CELL TUMORS
%97
%3
Stage 1 Diğer
%20
%80
Malign Benign
SEX CORD-STROMAL TUMORSSTEROID 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.
SEX CORD-STROMAL TUMORSTREATMENT
• Definitive management– Surgical stage– Desire of future childbearing– Histologic subtype– Patients age
• Midline incision
• Peritoneal washings
• Abdominal exploration,inspection,palpation
• Multiple biopsies
• Omentectomy
• Retroperitoneal sampling/dissection
SEX CORD-STROMAL TUMORSTREATMENT
• Most of the SCSTs are bening. Surgical therapy such as USO or BSO with TAH is sufficient therapy.
SEX CORD-STROMAL TUMORSTREATMENT
• Thecoma• Fibroma• Gynandroblastoma• Stromal luteoma• Leydig cell• Sclerosing stromal• Sertoli cell
CYTOREDUCTİVE SURGERY
SEX CORD-STROMAL TUMORSTREATMENT
SEX CORD-STROMAL TUMORSTREATMENT
• Postoperative management must be individualized.– Adjuvant chemotherapy– Radiotherapy– Hormonal therapy– Expectant therapy
?
Bleomycin
Etoposide
CisPlatinum
SEX CORD-STROMAL TUMORSTREATMENT
Platinum based chemotherapy
Bleomycin
Vinblastine
CisPlatinum
Taxol
CisPlatinum
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)
• 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)
• 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
Wolf,1999
CONCLUSIONRadiotherapy can induce a clinical response with
OCCASIONAL long-term remission...
Case reports demonstrated that...
.....JGCTs were highly resistant to chemotherapy .....
JUVENILE GRANULOSA CELL TUMORSTREATMENT
•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
SEX CORD-STROMAL TUMORSÇUMF
OVARIAN CARCINOMA ,256 CASES
%89
%11
Over ca SCSTs
SEX CORD-STROMAL TUMORSÇUMF
STAGEs
11
21
29
5 302468
1012
1a 1c 2b 2c 3c 4 ?
3-B Sütun 1
SEX CORD-STROMAL TUMORSSURVIVAL
ÇÜMF
– 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
SEX CORD-STROMAL TUMORS
OPERATION,OUTSIDE OF THE ÇUMF
1716
YES NO
RESIDUEL DISEASE, DETERMINED IN ÇUMF
6
2
YES NO