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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Documents

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