gastro-intestinal stromal tumor (gist) the experience of chia-yi chang- gung hospital 葉重宏...
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Gastro-Intestinal Stromal Tumor (GIST)
The experience of Chia-Yi Chang-Gung Hospital
葉重宏 王正儀 嘉義長庚紀念醫院 大腸直腸外科
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Introduction Gastro-intestinal Stromal Tumors (GIST
s) Mesenchymal tumors which express sp
ecially c-kit (CD117) and/or CD34
less than 0.1 % of all colorectal malignancies.
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Cases Presented
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Case 1 Name : x x x
Age : 62 Y/O
Sex : Male
Chart No. : 360xx07
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Chief Complaint
A perianal mass was noted for 2 years
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Brief history The 62 Y/O male patient was generally
healthy before. A perianal mass was noted since 2 years ago. The mass became larger recently, and he came to our OPD for help.
Past History : denied history of systemic disease Personal History : allergic history of penicillin Family history : Non-contributory
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Physical examination Consciousness : Clear HEENT : Not icteric, not anemic Chest : Symmetric expansion Heart : RHB, No murmur Abdomen : No palpable mass Extremities : Full and free Perineum : A perianal tumor over L’t lat. aspect – Hard, fixed, pain +, tenderness (–) Diameter : 5 cm x 5 cm
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C X R Film
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Fibrocolonoscopy
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ABD CT Scan (I)
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ABD CT Scan (II)
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Trans-rectal ultrasound sonography
5 cm level 4 cm level
2 cm level Anal verge
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Preoperative Diagnosis
Para-rectal tumor R/O Rectal GIST
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Operative procedure
Abdomino-Perineal Resection ( APR )
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Operative finding
A para-rectal tumor over L’t lat. to L’t post. aspect, locating beneath the dentate line.
Well circumscribed, lobulated, solid, light tan, firm, Adhering with the lower rectal wall and anal sphincter
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Pathological Report Anorectum, Abdominoperineal resection -- An anorectal tumor composed of lobules
of spindle cells with mild nuclear atypia and high mitotic rate ( > 20 MFS/10 HPF )
Immunohistochemical Stain : c-KIT (+), CD34 (+), S-100 (-), SMA (-) Lymph Node, Regional --Negative for malignancy ( 28/28 ) Gastrointestinal Stromal Tumors
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H. E. Stain
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C-KIT Positive Stain
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CD34 Positive Stain
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ABD CT Scan follow up (I)
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ABD CT Scan follow up (II)
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Case 2 Name : x x x
Age : 69 Y/O
Sex : Female
Chart No. : 9208xx80
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Chief Complaint
Lower back pain was noted for 2 months
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Brief history The 69 Y/O female patient was a case 0f malignant ne
urilemoma post ATH +BSO at TCVGH 2 years ago. The post-operative courses was smooth. Lower back pain was noted since 2 months ago, and recurrent pelvic tumor was noted. She accepted therapy of STI-571 for 7 weeks at MMH. Then she came to our OPD for help.
Past History : denied history of systemic disease except thrombocytopenia Personal History : Denied any allergic history Family history : Non-contributory
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Physical examination Consciousness : Clear HEENT : Not icteric, not anemic Chest : Symmetric expansion Heart : RHB, No murmur Abdomen : No palpable mass Extremities : Full and free Perineum : An extramural tumor over mid-
rectum with moderate induration, pain + Diameter : 3 cm x 2 cm
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C X R Film
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Fibrocolonoscopy
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ABD CT Scan (I)
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ABD CT Scan (II)
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ABD CT Scan (III)
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ABD CT Scan (IV)
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Preoperative Diagnosis
Recurrent pelvic Gastrointestinal Stromal Tumor (GIST)
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Operative procedure
Restorative proctectomy with colonic-J-pouch anal anastomosis with diverting T-loop colostomy
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Operative finding
A para-rectal tumor over R’t lat. pelvic side wall with mesorectal invasion, 2 cm above the dentate line. Vaginal stump invasion (+)
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Pathological Report Rectum, restorative proctectomy -- A 2x1.5 cm induration tumor composed of
lobules of spindle cells with mild nuclear atypia and low mitotic rate ( <3 MFS/50 HPF )
Invasion the rectal serosa, Vaginal (-), pelvic side wall (-) Immunohistochemical Stain : c-KIT (+), CD34 (+), S-100 (+), SMA (-) Lymph Node, Regional --Negative for malignancy ( 11/11 ) Recurrent Gastrointestinal Stromal Tumors
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Case 3 Name : x x x
Age : 43 Y/O
Sex : Male
Chart No. : 354xx29
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Chief Complaint
Tenesmus and perineal soreness were noted for several months
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Brief history The 43 Y/O male patient was a case of huge rectal
GIST ( 9 x 8 x 5 cm3 ) post restorative proctectomy with T-loop colostomy at LKCGMH one and a half years ago. The post-operative courses was smooth. Frequent bowel movement, tenesmus and perineal soreness were noted for several months, and recurrent GIST was noted after trans-rectal needle biopsy. He came to our OPD for help.
Past History : denied history of systemic disease Personal History : Denied any allergic history Family history : Non-contributory
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Physical examination Consciousness : Clear HEENT : Not icteric, not anemic Chest : Symmetric expansion Heart : RHB, No murmur Abdomen : No palpable mass, Op Scar + Extremities : Full and free Perineum : An induration lesion over anastomotic ring, R’t post. Aspect . pain + Tenderness + Diameter : 2 cm x 2 cm
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C X R Film
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ABD CT Scan (I)
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ABD CT Scan (II)
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Preoperative Diagnosis
Recurrent Rectal Gastrointestinal Stromal Tumor (GIST)
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Operative procedure
Abdominoperineal Resection (APR)
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Operative finding
An induration lesion around previous anastomotic site with extension to L’t para-prostate tissue and R’t pelvic side wall with R’t ureter invasion ( U-V junction ), 1 cm above the dentate line.
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Pathological Report Anorectum, APR -- A 2x2 cm induration tumor composed of highly cell
ular spindle cells with moderate cell pleomorphism and high mitotic rate ( 1-2 MFS / HPF ), located within the muscular layer
Local abscess formation(+) Immunohistochemical Stain : c-KIT (+), CD34 (+), S-100 (-), SMA (-) Lymph Node, Regional --Negative for malignancy ( 11/11 ) Recurrent Gastrointestinal Stromal Tumors
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H. E. Stain
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C-KIT Positive Stain
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CD34 positive stain
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Gastrointestinal Stromal Tumor
G I S T
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INTRODUCTION (1)
1983 – Mazur and Clark –the term Describe gastrointestinal non-epithelial neop
lasms lacking the immunohistochemical features of Schwann cells and the ultrastructural characteristics of smooth-muscle cell
1988 – Hirota and colleagues Discovery of gain-of-function mutation in the
KIT proto-oncogene in GISTs
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INTRODUCTION (2) Mesenchymal tumors of GI tract which
express specially c-kit (CD117) and/or CD34
Previously regarded as : Leimyoma Leimyosarcoma Leiomyoblastoma Neurilemoma
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INTRODUCTION (3) Histology Highly cellular spindle cell or epithelioid me
senchymal tumors Immunohistochemical study Expression of c-kit protein CD34 70% Smooth muscle actin 20~30% S-100 protein 10% Desmin 2~4%
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Prevalence– 0.2% of gastrointestinal (GI) tumours– Incidence: 3000 to 5000 cases in the US
Similar male-to-female ratio Highest incidence in 5th to 7th decade
s of life
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Distribution Stomach 60 ~ 70% Small intestine 25 ~ 35% Colon, rectum 5% Esophagus < 2% Omentum, mesentery, retroperitoneum GISTs of the colon and the rectum are
less than 0.1 % of all colorectal malignancies.
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Clinical Presentation Vague GI pain or discomfort
GI hemorrhage
Other symptoms include anorexia, weight loss, nausea, anemia, and additional GI complaints
Often asymptomatic, especially early in tumor development
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Malignant GISTs are about 20% ~ 30 %
Prediction of malignancy >= 5 mitosis / 50 HPF Tumor size > 5 cm -- High frequency of intra-abdominal recurrenc
e and liver metastasis
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Malignant Behaviour of the GISTs
Distant metastasis : Liver Lung Bone Brain Local recurrence Intra-abdominal dissemination
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Treatment
Surgical resection **
Resistant to chemotherapy
Resistant to radiotherapy
Imatinib mesylate (STI-571) ***
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Outcome of Surgical treatment Surgery is primary treatment modality for
GISTs– 5-year survival 50% to 65%– Recurrence after a decade or more
If incomplete resection/metastatic at presentation– Median survival <1 year– 5-year survival <35%
If disease unresectable– Median survival 9 to 12 months
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Outcome of Surgical treatmentof Rectal GISTs Local resection : 67.5% ~ 86 % local recurrence
Abdominoperineal Resection : : 19.5% local recurrence
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Surgical Outcome of Rectal GISTs of CGMH 40 cases of rectal GISTs with radic
al resection
1-year 3-year 5-yearOverall survival 97% 90% 75%Disease-free survival 90% 59% 46%
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Imatinib mesylate (STI-571)
( Gleevec, Glivec )
A specific inhibitor of c-Kit tyrosine kinase activity, and blocks c-Kit–mediated downstream signaling
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The Biology of c-Kit c-Kit is found in many normal tissues and is
essential for– Haematopoiesis – Melanogenesis– Gametogenesis– Interstitial cells of Cajal development
Activation of c-Kit plays a critical role in different cell functions– Proliferation – Differentiation – Apoptosis/survival – Adhesion/chemotaxis
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Gain-to-function mutation in the c-kit proto-oncogene
constitutive activation of Kit receptor tyrosine kinase
induce cellular proliferation and decrease
apoptosis
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Imatinib: CML Phase II Results
% of Patients (CI95%)
Study 0110 Chronic Phase IFN- Failure
(N=454)
Study 0109 Accelerated
Phase (N=235)
Study 0102 Blast Crisis
(N=229)
Haematological response 95%
_
71%
(61.8–75.7)
31%
(24.7–37.0)
Major cytogenetic response
60%
_
26%
(17.8–30.6)
16%
(11.6–21.6)
Complete 41% 17% 7% Partial 19% 7% 9%
Kantarjian et al. N Engl J Med. 2002;346:645-652.Talpaz et al. Blood. 2002;99:1928-1937.Sawyers et al. Blood. 2002;99:3530-3539.
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GIST Phase II: Best Confirmed Responses
-- July 10, 2001
n (%)Complete response (CR) 0Partial response (PR) 59 (40)Stable disease (SD) 61 (41)
Unconfirmed PR 28 (19)25% to 49% reduction in tumor19 (13)
Progressive disease (PD) 18 (12)
Nonevaluable 7 (5)
Unknown (UNK) 2 (1)
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Summary Colon and rectal GISTs are rare colon
and rectal malignancies
Radical resection provide the only chance of curative treatment.
STI-571 ( Glivec ) may be beneficial for the cases of unresectable tumors, distant metastasis, and carcinomatosis.
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Thank You !