SEX CORD-STROMAL TUMORS

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SEX CORD-STROMAL TUMORS

• Dr.Aytekin Altintas

ADANA
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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
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SEX CORD-STROMAL TUMORS CLASSIFICATION

• GRANULOSA-THECA CELL
• JUVENILE
• ADULT
• ANDROBLASTOMA
• OTHERS

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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.

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GRANULOSA CELL TUMORS

• In all ovarian malignancies

• In malignant sex cord-stromal tumors

70
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GRANULOSA CELL TUMORSADULT TYPE

• SYMPTOMS
• Abnormal vaginal bleeding
• Abdominal distension
• Abdominal pain
• Rarely,virilizing effect

• The adult type is 95 of all GCT

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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.

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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.

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GRANULOSA CELL TUMORSADULT TYPE

• PROGNOSTIC FACTORS
• Tumor size
• Rupture
• Histologic subtype
• Nuclear atypia
• Mitotic activity
• Ploidy status
• p53 expression

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GRANULOSA CELL TUMORS

• TUMOR MARKERS
• Serum estrogens
• Inhibin
• Follicle-regulatory protein
• Mullerian inhibitory substance

• Elevated Inhibin
• Menstrual cycle
• Pregnancy
• Other tumors

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GRANULOSA CELL TUMORSJUVENILE TYPE

• Ninety percent of GCTs diagnosed in prepubertal
  girls are juvenile type.
• Clinical behavior is different from adult type

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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

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JUVENILE GRANULOSA CELL TUMORSSTAGE AT DIAGNOSIS

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SEX CORD-STROMAL TUMORS

• TUMORS IN THECOMA-FIBROMA GROUP
• Thecoma
• Fibroma, Fibrosarkoma
• Sclerosing stromal tumor

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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.

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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?

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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)

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SEX CORD-STROMAL TUMORSTREATMENT

• Definitive management
• Surgical stage
• Desire of future childbearing
• Histologic subtype
• Patients age

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SEX CORD-STROMAL TUMORSTREATMENT

• Midline incision
• Peritoneal washings
• Abdominal exploration,inspection,palpation
• Multiple biopsies
• Omentectomy
• Retroperitoneal sampling/dissection

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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

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SEX CORD-STROMAL TUMORSTREATMENT

• CYTOREDUCTIVE SURGERY

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SEX CORD-STROMAL TUMORSTREATMENT

• Postoperative management must be individualized.
• Adjuvant chemotherapy
• Radiotherapy
• Hormonal therapy
• Expectant therapy

?
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SEX CORD-STROMAL TUMORSTREATMENT
Platinum based chemotherapy
Taxol CisPlatinum

• Bleomycin
• Etoposide
• CisPlatinum

Bleomycin Vinblastine CisPlatinum
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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)
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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)
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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
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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

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SEX CORD-STROMAL TUMORSÇUMF OVARIAN CARCINOMA
,256 CASES
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SEX CORD-STROMAL TUMORSÇUMFSTAGEs
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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

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SEX CORD-STROMAL TUMORS