A Patient With Gastrointestinal Stromal Tumors-final

7/27/2019 A Patient With Gastrointestinal Stromal Tumors-final

1/47

Departement of Internal Medicine

Airlangga School of Medicine-dr.Soetomo Teaching HospitalSurabaya

2009

CASE PRESENTATION

Dian Fajarwati

Pangestu Adi


2/47

INTRODUCTION

GIST

Immunohistochemically KIT

+, mesenchymal neoplasmof the GI tract and abdomen

Term GIST:

Mazur and Clark (1983)

rare tumors ; incidence of 10-

20/ 1 million /year ;


3/47

CASEChief complaint :

Vomitus of coffee ground

material

Black stool

Early satiety, nausea, abdominal

discomfort

Decreased appetite

Fatigue, pale

History of past illness:

Hospitalized three times , same

complaint

No traditional medicine and NSAID

consumption

No icteric

No cancer in family

Mrs. F, 48 years old

Javanesse

Stay in Surabaya

Housewife


4/47

Physical Examination

H/L: unpalpable, mass in epigastrium

to left hypocondrium region 8x8 cm,

fixed, not well defined

Edema -/- , eritema palm -/-

Alert, weak, GCS 456,

BP 110/70 mmHg, pulse 92 x/m,RR 20x/m, axillary temp. 36.8C

Anemia (+)

Heart & Lung : no abnormality,

spider nevi - /-


5/47

Laboratory studies

Hb 5.1 g/dl

WBC 9,800/mm3

Plt 820,000/mm3

LED 20/60Coagulation test noabnormality

Urinalysis no abnormality

Blood sugar 128 mg/dl

Creatinine serum 0.7 mg/dlBUN 10 mg/dl

AST 62 IU/L

ALT 35 IU/L

total protein 4.8 g/dLalbumin 2.42 g/dL

globulin 2.4 g/dL

potassium 3.47 mmol/L

sodium 132 mmol/L

chlorida 99.3 mmol/L

ECG : sinus rhytm 90 x/ minutes


6/47

Imaging studies

Abdominal USG :

mass 4x 10 cm trace from gaster, liverwas normal ( no nodul/ cyst/abses)

Chest X-ray :No abnormality


7/47

1st Endoscopy (Sept,17,

2008): tumor at the corpus

that covered posterior wall,minor and major curvatura.

Conclusion suspect gaster

carcinoma.

1st Biopsy : erosive gastritis

2nd Endoscopy (Nov,3,

2008): no erosion and

esophagus varices.

Conclusion : mass in the

corpus gaster that

caused obstruction

(suspected as

malignancy)

2nd biopsy : chronicgastritis

Upper endoscopy


8/47

Imaging studiesSolid mass in the gaster wall 12.2 x

9.4 cm , unclear border,inhomogen contrast enhancement.

No liver enlargement. No

nodul/mass/cyst.

Spleen/ Pancreas/Ren D/S were

normal. No bone destruction.Conclusion : mass in the gaster

wall.

FNAB,CT Scan guiding (Nov, 27,

2008) : hypercellular consist ofgroup and spreading of spindle cell

nucleus partly plump and lobulated,

pleiomorfik , coarse chromatin, and

matrix myxoid. Conclusion spindle

mesenchymal tumor


9/47

Initial assessment :

Obs.Hematemesis

melena

Anemia Hypoalbumin

S. Gastric tumor

Planning Dx :

Upper GI X-Ray

CEA; Ca 19-9

Planning Tx :

infus PZ

Blood tranfusion Gastric lavage/ 6 hours

Lavement/12 hours

Inj. Octreotid iv.

Inj.proton pump inhibitor iv. albumin infusion


10/47

Imaging studies

Upper GI X-Ray( Des, 5, 2008)

: ground glass appearance at

the upper left , minimal gas in

the gaster.Filling defect with fungating

type and ulcerative type,

destroyed mucosal pattern in

the corpus until anthrum gaster


11/47

Dec, 2,2008

No hematemesis melena. Consultation to

digestive surgery : Gastric malignancy andpost HM. Planning Dx. Upper GI X-Ray , CEA,Ca19-9

Dec, 5,

2008

Upper GI : filling defect , fungating & ulcerative type, destroyedmucosal pattern in the corpus until anthrum gaster .

CEA 0.8 ng/ml ( < 5 ) ; Ca 19-9 1.2 U/ml ( < 37)

Dec,22,2008

The patient underwent operation. Tumor was unresectablebecause the tumor adhered at the porta vein and multiplenodul in liver. Jejunostomy was done for enteral feeding

Jan,16,2009

No complaint

The patient discharged from the hospital and refused to betreated with chemotheraphy

Advice : controlled to gastrohepatology ,hematology-oncology & surgery clinic

Progress Note


12/47

Hystopatology

Malignant tissue tumor:

anaplastic cell proliferation,

pleiomorphic round nucleus,hyperchromatic, mitosis >20/10

HPF, trabecular formed,

Conclusion: high grade

malignancy dd.

adenocarcinoma poorlydifferentiated ; stromal tumor.

Imunohistochemistry :CD 117 positive .

Conclusion :

Gastrointestinal Stromal

Tumor (c.KIT positive).

Imunohistochemistry


13/47

DISCUSSION


14/47

This patient : hematemesis-melena, weight

loss, anemia, palpable mass in the abdomen

Sign and Symptom non-specific : early satiety, bloating, non

specific abdominal pain, gastrointestinal

bleeding , fatigue from anemia, or obstruction.

Abdominal pain, melena and weight loss

most common symptoms

Rarely, an abdominal mass is palpable

Gastrointestinal Stromal Tumor


15/47

Laboratory examination

No laboratory test specifically confirm GIST.

This patient : anemia and hypoalbumin. The

tumor markers (CEA and Ca 19-9) were negative


16/47

Imaging studies

no standard imaging protocol, all imaging techniques

may be used.

preoperative diagnosis based on clinical and radiologic

data is difficult

nonspecific presentation typically grow as bulky, well-defined, endo- or exophytic

masses parallel to the bowel lumenstromal origin

normally present with the typical signs ofsubmucosal or

extrinsic GI lesions on imaging studies

overlyingmucosa can be normal or show signs of necrosis or

ulceration.

i di


17/47

Imaging studies Endoscopic examination :smooth protrusion of the bowel

wall, lined with mucosa, some cases show signs of bleeding

and ulceration

Full layer biopsy true histopathologic Dx. GIST: 27-50% by endoscopic biopsy

The results : gastric tumor and the biopsy revealed erosive

and chronic gastritis

Barium series detect GIST sufficient size filling defect : sharply demarcated and is elevated compared

with surrounding mucosa

overlying mucosa is smooth unless ulceration

the information is limited because of striking image

Upper GI : mass in the corpus to anthrum gaster , fungating

& ulcerative type with destroyed mucosal pattern

d


18/47

Imaging studies USG studies : well-defined or polylobulated solid

masses. Cystic changes,necrosis, or calcifications.

image quality is often degraded by intervening bowel

gas

USG studies : mass 4x10 cm and no abnormality in the

other structures

CT Scan : important in the diagnosis and staging of GIST

Detect multiple tumors and provide evidence of metastatic spread

less aggressive GIST : < 5 cm, well-defined, round or oval, exophytic

masses and homogeneous enhancement

Aggressive GIST : irregular and lobulated margins, >10 cm, centralnecrosis, ulceration, and heterogeneous contrast enhancement

CT Scan : solid mass 12.2 x 9.4 cm in the gastric wall , irregular,

heterogenous contrast enhancement , no metastatic to adjacent

organs.


19/47

Biopsy

GIST tend to fall into

three categories of

morphology

epitheloid, spindlecell, or mixed

The diagnosis of

GIST relies on

histopatology andimunohistochemistry

CD 117 is generally

positive

FNAB with CT scan

guiding: spindle

mesenchymal tumor

Biopsy durante op :

adenocarcinoma dd.

Stromal tumor

Imunohistochemistry:CD 117 positive


20/47

Risk Classification in GIST

Mass 12.2x 9.4 cm , mitosis > 20/10 HPF high risk

Kim, 2005


21/47

Therapy Surgical : definitive therapy

1. Complete resection : recurrent 36 months Recurrence Rate 40-52%

This patient was classified in to high risk

GIST, metastatic disease and unresectable

tumor. The prognosis was poor.


23/47

Summary

We have reported a patient withGastrointestinal stromal tumor with liver

metastases

The diagnosis of GIST based onhistopathology and imunohistochemistry

examination

The patient underwent operation but the tumor

was unresectable. Jejunostomy was done forenteral feeding

The patient was classified in to high risk GIST

the prognosis was poor


24/47

THANK YOU


25/47


26/47


27/47


28/47

Kim,2005


29/47

Kim,2005


30/47

Rubin,2007


31/47

Rubin,2007


32/47

Rubin,2007


33/47

Rubin,2007


34/47

Chemical structure of Imatinib mesylate

Rubin,2007


35/47

Rubin,2007


36/47

Rubin,2007


37/47

Rubin,2007


38/47

Rubin,2007


39/47

Rubin,2007


40/47

Miettinen,2006


41/47

Miettinen, 2006


42/47

Immunohistochemical differential diagnosis of themost important mesenchymal tumor of the GI tract

Miettinen, 2003


43/47

Miettinen, 2003


44/47

Demetri, 2004

N l d M t t d KIT d th A ti f I ti ib


45/47

Normal and Mutated KIT and the Action of Imatinib

Demetri, 2004


46/47


47/47

A Patient With Gastrointestinal Stromal Tumors-final

Documents

Transcript of A Patient With Gastrointestinal Stromal Tumors-final