The third MSG RAO Workshop E. Oriol-Pibernat MSG Mission Manager GIST, Exeter, 24 October 2006.
Gist Workshop
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Transcript of Gist Workshop
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WORKSHOP GISTSEMARANG 14 SEPTEMBER 2013
INTESTINAL GIST
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ANAMNESIS
Laki-laki 44 tahun,
12 bulan perut merasa tidak enak,kadang-kadangmules, pernah berak kehitaman. Hanya diobatkan
ke dokter puskesmas 3 bulan teraba benjolan yang makin lama makin
membesar, perut terasa sebah dan mules sekalidan berak hitam makin jelas
Nafsu makan kurang dan berat badan menurun
Riwayat family dengan tumor abdomen disangkal
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PEMERIKSAAN FISIK
Laki-laki berat badan: 42 Kg, TB 165 cm, BMI :15,44
Kurus, nampak pucat
Abdomen kembung ringan, terlihat bulging perutdibagian tengah, gambaran dan gerakan usustidak terlihat.
Palpasi teraba tumor diameter sekitar 12 cm,
bulat dengan permukaan tidak rata, konsistensikeras, nyeri tekan (-), relatif mobile
Auscultasi: Peristaltik normal, tidak terdengarbising diatas tumor
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LABORATORIUM
Hb 7,8 Mg%, Ht 24, Lekosit 6500
Alb 2,8 mg%, Glob 2,3 mg%
Na 134, K 3,2, Cl 98, Ca 2,10 Lain-lain baik.
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DIAGNOSA BANDING
LYMPHOMA MALIGNA
HODGKIN DISEASE
SCWANOMA MALIGNA LEIOMYOSARCOMA
INSTESTINAL GIST
PERLU KEPASTIAN DIAGNOSIS SEBELUM OPERASI?
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PERSIAPAN PRA-BEDAH
ANEMIA:
BLOOD TRANFUSION
SEVERE MALNUTRITION
PRE-OPERATIVE NUTRITIONAL SUPPORT
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LAPARATOMI
TUMOR USUS HALUS PERTENGAHAN
(PERBATASAN ILEUM-JEJENUM) SEKITAR 14
CM, KERAS, LN MESENTERIKA (-), PERITONEAL
SEEDING (-), METASTASE HEPAR (-)
TANDA PARTIAL OBSTRUKSI USUS (+)
DILAKUKAN RESEKSI USUS HALUS ISTAL DAN
PROKSIMAL TUMOR, ANASTOMOSIS END TO
END
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PASCA BEDAH
PASIEN BAIK PULANG TANPA KOMPLIKASI
PA SPINDEL CELL TUMOR CURIGA GIST, BATASRESEKSI BEBAS TUMOR, MITOSIS(?)
IHC CD 117 (+)
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PEMBAHASAN
Diagnostic procedure?
Grade?
Surgery? Chemotherapy (preoperative? post-
operative?)
Prognosis?
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DIAGNOSTIC PROCEDURE?
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PREOPERATIVE DIAGNOSIS
ENDOSCOPY:
DOUBLE BALLON ENTEROSCOPY?
CAPSULE ENDOSCOPY?
LAPARATOMY/ LAPAROSCOPY BIOPSY?
NO NEED DIRECT LAPARATOMY &
RESECTION OR BIOPSY.
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GRADING
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POLSKIE ARCHIWUM MEDYCYNY WEWNTRZNEJ 2008; 118 (4)
HUMAN PATHOLOGY Volume33, No. 5 (May 2002)
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NIH Classification for Risk of
RecurrenceVery Low Risk Low Risk Intermediate Risk High Risk
NIH consensuscriteria1 Tumor size < 2 cmMitotic index < 5 Tumor size 2-5 cmMitotic index < 5 Tumor size 5-10 cmMitotic index < 5
ORTumor size < 5 cmMitotic index 6-10
Tumor size > 5 cmMitotic index > 5
ORTumor size > 10 cmMitotic index, any
ORTumor size, any
Mitotic index > 10Modified NIHconsensusclassification2
Any location:Tumor size < 2 cmMitotic index 5
Any location:Tumor size 2.1-5 cmMitotic index 5
Any location:Tumor size < 5 cmMitotic index 6-10
Gastric:Tumor size 2.1-5 cmMitotic index > 5
ORTumor size 5.1-10 cmMitotic index 5
Any location:Tumor rupture
ORTumor size > 10 cm
ORMitotic index > 10
OR
Tumor size > 5 cmMitotic index > 5
Nongastric:Tumor size 2.1-5 cmMitotic index > 5
ORTumor size 5.1-10 cmMitotic index 5
Abbreviations: Mitotic index, number of mitoses per 50 high-power fields; NIH, National Institutes of health.1. Fletcher CD, et al. Hum Pathol. 2002;33(5):459-465; 2. Joensuu H. Hum Pathol. 2008;39(10):1411-1419.
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GRADE
TUMOR > 10 CM
MITOTIC INDEX: NOT REPORTED
NEGATIVE MARGIN LOCATION: NON GASTRIC
HIGH RISK GROUP FOR RECCURRENCE
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SURGERY
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Surgical treatment of gist
The radical surgical treatment is the most effectivetreatment
The 5-year survival rate after surgery amounts to 2865%
It is not necessary to resect the regional lymph nodesbecause GIST do not metastasize to the regional
lymphatic system 2040% of the surgery patients have intra-abdominal
dissemination or liver metastasis paliatif surgery(sympotomatic treatment)
endoscopic dissection (submucosal-mucosal resection)
allows a radical therapy of small tumors withoutmalignancy features and limited to the submucosal layer.
POLSKIE ARCHIWUM MEDYCYNY WEWNTRZNEJ 2008; 118 (4)
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CHEMOTHERAPY
Preoperative : need histologicaldiagnosis
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PROGNOSIS
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The 5-year survival rate after surgery of GIST
amounts to 2865%
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778 patients
18 y
Localized and
primary GIST
KIT-positivetumors 3 cm
Complete surgical
resection Placebo for 1 y
Imatinib400 mg/d for 1 y
Imatinib
400/800 mg/d
713 patients
randomized
Imatinib400 mg/d
DeMatteo RP, et al. Lancet. 2009;37(9669)3:1097-1104.
Phase 3 ACOSOG Z9001: Trial Schema
Endpoints:
Primary: Recurrence-free survival
Secondary: Overall survival, safety
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At time ofrecurrence
At time of
recurrence
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Abbreviations: CI, confidence interval; HR, hazard ratio.a All randomized patients were included in the analysis; recurrence-free survival was defined as the time from patient
registration to the development of tumor recurrence or death from any cause. Intention-to-treat analyses were done for
recurrence-free survival (ie, analyzed patients by randomized group).Adapted from DeMatteo RP, et al. Lancet. 2009;373(9669):1097-1104.
Recurrence-Free Survivala
HR = 0.35 (95% CI = 0.22, 0.53); P< .0001100
90
80
70
6050
40
30
20
100
Recurrence-FreeandAlive,
%
0 6 12 18 24 30
Time, mo
36 42 48
Imatinib
Placebo
359
354
30
70
Total Events
Median follow-up: 19.7 mo
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Size 10 cm
Size 3 and < 6 cm Size 6 and < 10 cm
Recurrence-Free Survival (Tumor Size)
Imatinib adjuvant therapy results
in significantly longer RFS in eachof the tumor size categories
compared with placebo
100
90
80
7060
5040
30
2010
0Recurrence-FreeandAlive,
%
0 6 12 18 24 30 36 42 48
100
9080
7060
50
40
302010
0Recurrence-Freean
dAlive,
%
0 6 12 18 24 30 36 42 48
Imatinib, n = 143Placebo, n = 149
HR = 0.23 (95% CI = 0.07, 0.79); P= .011
Imatinib, n = 93
Placebo, n = 86
HR = 0.29 (95% CI = 0.16, 0.55); P< .001
Time, mo
Time, mo
10090
80
70
60
50
40
30
20
10
0Recurrence-FreeandAlive,
%
0 6 12 18 24 30 36 42 48Time, mo
Imatinib, n = 123Placebo, n = 119
HR = 0.50 (95% CI = 0.25, 0.98); P= .041
Abbreviations: CI, confidence interval; HR, hazard ratio; RFS, recurrence-free survival.Adapted from DeMatteo RP, et al. Lancet. 2009;373(9669):1097-1104.
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Follow-up
Follow-up
Phase 3 SSGXVIII: Study Design
Randomized
1:1
Imatinib12 mo
400 patients
KIT-positive
histologically
confirmed GIST
High
recurrence riskaccording to
modified NIH
consensus
criteria
Endpoints:
Primary: Recurrence-free survival
Secondary: Overall survival, safety
Imatinib36 mo
Abbreviation: NIH, National Institutes of Health.Data from Joensuu H, et al.JAMA. 2012;307(12):1265-1272.
Key Elements:
Patient stratification:R0 resection, no tumor rupture
R1 resection OR tumor rupture
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HR = 0.46 (95% CI = 0.32, 0.65); P< .0001
SSGXVIII: Recurrence-Free Survival
(ITT)
Abbreviations: CI, confidence interval; HR, hazard ratio; ITT, intent to treat.Adapted from Joensuu H, et al.JAMA. 2012;307(12):1265-1272.
60.1%
47.9%
86.6%
65.6%
36 mo, n = 198
12 mo, n = 199
0 1 2 3 4 5 60
20
40
60
80
100
Median follow-up,
54 mo
Time, y
Recu
rrence-Freeand
Alive,
%
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