SEX CORD-STROMAL TUMORS Dr.Aytekin Altıntaş ADANA.

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


• 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.


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


• 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

%


• 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


• 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

CYTOREDUCTİVE SURGERY



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

?

Bleomycin

Etoposide

CisPlatinum


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


OPERATION,OUTSIDE OF THE ÇUMF

1716

YES NO

RESIDUEL DISEASE, DETERMINED IN ÇUMF

6

2

YES NO

SEX CORD-STROMAL TUMORS Dr.Aytekin Altıntaş ADANA.

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