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SEX CORD-STROMAL TUMORS

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SEX CORD-STROMAL TUMORS Dr.Aytekin Alt nta ADANA SEX CORD-STROMAL TUMORS CLASSIFICATION Granulosa cell tumor Adult Juvenile Thecoma-fibroma Thecoma Fibroma,sarkoma ... – PowerPoint PPT presentation

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Title: SEX CORD-STROMAL TUMORS


1
SEX CORD-STROMAL TUMORS
  • Dr.Aytekin Altintas

ADANA
2
SEX 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
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
GRANULOSA CELL TUMORS
  • In all ovarian malignancies
  • In malignant sex cord-stromal tumors

70
6
GRANULOSA CELL TUMORSADULT TYPE
  • SYMPTOMS
  • Abnormal vaginal bleeding
  • Abdominal distension
  • Abdominal pain
  • Rarely,virilizing effect
  • The adult type is 95 of all GCT

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


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
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.
  • 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)

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
SEX 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

21
SEX CORD-STROMAL TUMORSTREATMENT
  • CYTOREDUCTIVE SURGERY

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

?
23
SEX CORD-STROMAL TUMORSTREATMENT
Platinum based chemotherapy
Taxol CisPlatinum
  • Bleomycin
  • Etoposide
  • CisPlatinum

Bleomycin Vinblastine CisPlatinum
24
GRANULOSA 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)
25
GRANULOSA 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)
26
ADVANCED 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
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
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