Transcript SEX CORD-STROMAL TUMORS - Prof.Dr.Aytekin Altıntaş

SEX CORD-STROMAL
TUMORS
Dr.Aytekin Altıntaş
ADANA
SEX CORD-STROMAL TUMORS
CLASSIFICATION
1-GRANULOSA-STROMAL
• Granulosa cell
tumor
– Adult
– Juvenile
• Thecoma-fibroma
2-SERTOLI-STROMAL
• Sertoli cell
• Leydig cell
• Sertoli- Leydig
3-STEROID CELL
• Stromal luteoma
• Leydig cell
• Hilus cell
• Leydig cell
• Other
– Thecoma
– Fibroma,sarkoma
– Sclerosing
stromal tumor
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 cordstromal tumors
%70
%30
%70
GCT
OTHER
GRANULOSA CELL TUMORS
ADULT TYPE
• The adult type is 95%
of all GCT
%5
SYMPTOMS
In ÇUMF;31 CASES
• Abnormal vaginal
bleeding
%3
• Abdominal
distension
• Abdominal pain
• Rarely,virilizing effect
%97
%95
ADULT
ADULT
JUVENILE
JUVENILE
GRANULOSA CELL TUMORS
ADULT 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.
LITERATURE
%20
%80
Stage I
OTHER
ÇUMF
%35
%65
ADULT GRANULOSA CELL TUMORS
SURVIVAL
• Overall 5-year
survival rates are
nearly 90%.
• In patients with
extraovarian spread
at the time of
diagnosis, 5-year
survival is 33-53%.
ÇUMF
120
100
80
60
40
20
0
0
1.yıl
Overall
2
3
4
With extraovarian disease
5
GRANULOSA CELL TUMORS
ADULT TYPE
• PROGNOSTIC FACTORS
–
–
–
–
–
–
–
Tumor size
Rupture
Histologic subtype
Nuclear atypia
Mitotic activity
Ploidy status
p53 expression
GRANULOSA CELL TUMORS
• TUMOR MARKERS
– Serum estrogens
– Inhibin
– Follicle-regulatory
protein
– Mullerian inhibitory
substance
• Elevated Inhibin
– Menstrual cycle
– Pregnancy
– Other tumors
GRANULOSA CELL TUMORS
JUVENILE TYPE
• Ninety percent of GCTs diagnosed in
prepubertal girls are juvenile type.
• Clinical behavior is different from adult
type
GRANULOSA CELL TUMORS
JUVENILE TYPE
PREPUBERTAL SYMPTOMS
•
•
•
•
•
•
Breast enlargement
Pubic and axillary hair
Advanced somatic development
Vaginal bleeding
Increasing abdominal girth
Abdominal pain
– Spontaneous rupture
• Rarely virilization syndrome
JUVENILE GRANULOSA CELL TUMORS
STAGE AT DIAGNOSIS
%
90
80
70
60
50
40
30
20
10
0
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 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
SEX CORD-STROMAL TUMORS
SERTOLI- LEYDIG CELL TUMORS
%3
• Stage is the most important
prognostic factor.
• Despite an average size of
16 cm,only 2-3% of SLCTs
have extraovarian diseaseLow malign potential.....
• Testesteron, inhibin, AFPTumor marker?
%97
Stage 1
Diğer
%20
%80
Malign
Benign
SEX CORD-STROMAL TUMORS
STEROID CELL TUMORS
• Stromal luteoma
• Leydig cell tumor
– Hilus cell
– Leydig cell
• Steroid cell tumors not
otherwised specified.
(SCTNOS)
• SCT-NOS
SCTs constitute only
0.1%
of 47
all avarian
•Avarage
age...
neoplasms.
•Produce
Hormones,
-Testesterone
• Old name:Lipid-cell
-Estrogen
tumors.
-Corticosteroids
• Stromal luteoma and
•Sometimes,
Leydignonpalpable
Cell tumor are
•In adults,25-43%
are malignant
always benign.
•SCTNOS are not malignant less
than 20 years of age.
SEX CORD-STROMAL TUMORS
TREATMENT
• Definitive management
–
–
–
–
Surgical stage
Desire of future childbearing
Histologic subtype
Patients age
SEX CORD-STROMAL TUMORS
TREATMENT
•
•
•
•
•
•
Midline incision
Peritoneal washings
Abdominal exploration,inspection,palpation
Multiple biopsies
Omentectomy
Retroperitoneal sampling/dissection
SEX CORD-STROMAL TUMORS
TREATMENT
• 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 TUMORS
TREATMENT
CYTOREDUCTİVE SURGERY
SEX CORD-STROMAL TUMORS
TREATMENT
• Postoperative management must be
individualized.
–
–
–
–
Adjuvant chemotherapy
Radiotherapy
Hormonal therapy
Expectant therapy
?
SEX CORD-STROMAL TUMORS
TREATMENT
Platinum based chemotherapy
Bleomycin Bleomycin
Etoposide
Vinblastine
CisPlatinum CisPlatinum
Taxol
CisPlatinum
GRANULOSA CELL TUMORS
TREATMENT
Days
Bleomycin 20 units/m2
Etoposide 75 mg/m2
CisPlatinum 20 mg/m2
1
1-5
1-5
Every 3 weeks, 4 courses
Homesly,1999. A GOG study)
GRANULOSA CELL TUMORS
TREATMENT
• 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)
ADVANCED GRANULOSA CELL TUMORS
ADJUVANT 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
JUVENILE GRANULOSA CELL TUMORS
TREATMENT
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
SEX CORD-STROMAL TUMORS
ÇUMF
OVARIAN CARCINOMA ,256 CASES
%11
%89
Over ca
SCSTs
SEX CORD-STROMAL TUMORS
ÇUMF
STAGEs
12
10
8
6
4
2
0
11
2
1
9
2
1a 1c 2b 2c
5
3c
4
3
?
3-B Sütun 1
SEX CORD-STROMAL TUMORS
SURVIVAL
ÇÜMF
– Stage 1 and 2
– Stage 3c
16/16
1/5 (4of 9?)
%100
%20
» Ex.. 24 ,52,54(myocardiopathy), 58 mounths.
– Stage 4
1/5
» Ex .. 2 Post op , 13, 49 months
%20
SEX CORD-STROMAL TUMORS
OPERATION,OUTSIDE OF THE
ÇUMF
RESIDUEL DISEASE, DETERMINED
IN ÇUMF
2
16
17
6
YES
NO
YES NO