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Gastrointestinal stromal tumor(gist)

Gastrointestinal stromal tumor(gist)






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    Gastrointestinal stromal tumor(gist) Gastrointestinal stromal tumor(gist) Presentation Transcript

    • Dr. Amit Goswami
    • Introduction Mazur and Clark(1983) Mesenchymal tumor From embryological mesoderm of gastrointestinal tract <1% of all GIT tumors Hirota et.al(1998):Mutation in KIT Interstitial cell of Cajal: Common precursor?
    • Demography Incidence:15-20 per million M>F Age:40-80yrs(median age 60yrs) Mostly sporadic Familial( Neurofibromatosis, Carney triad) Eisenberg BL,Judson I.Surgery and imitanib in the management of GIST:emerging approaches to adjuvant and neoadjuvant therapy.Ann Surg Oncol 2004;11:465-475 Gold JS,Matteo RP.Combined surgical and molecular therapy: The gastrointestinal stromal tumor model.Ann surg 2006;244:176 DeMatteo RP,Lewis JJ,Leung D et al.Two hundred Gastrointestinal stromal tumors: recurrence patterns and prognostic factors for survival.Ann surg 2000;231(1):51-8 Takazawa Y,sakurai S,Sakuma Y et al.Gastrointstinal stromal tumors of neurofibromatosis type I.Am J surg Pathol 2005;29(6):755-63
    • Location Stomach :50% MC Esophagus:5% Small Intestine:25% Colon and rectum:10% Extra-intestinal:10% Rubin BP.Gastrointestinal stromal tumors: an update.Histopathology 2006;48:83-96 Clin Cancer Res 9(9):2003
    • Clinical Presentation Non specific Depends on site GIST of GIT: GI bleeding MC Others -Abd. Mass -Pain abdomen -Abd.distension -Intestinal obstruction Asymptomatic:30%
    • Pathology Most commonly involves muscularis propria Ulceration:50% Well circumscribed Cut surface: Tan/Grey, fibrous to fleshy Spindle cell type: MC
    • Malignant Potential• Features favoring benign lesions : – Size less than 5 cm – Low number of mitosis per HPF – No mucosal invasion – Low cellularity – Low markers of cell proliferation Tumor site: Stomach vs bowel Site of metastasis: Liver(50%),peritoneum(20-40%)
    •  M. Miettinen, et al. Am J Surg Pathol. 2005
    • Diagnosis Clinical, radiological and pathological characteristics CECT- Imaging modality of choice Endoscopic ultrasound: Small tumor MRI: Rectal GISTs PET scan: Assessment of therapy Blay JY,Bonvalot S,Casali P et al.Consensus meeting for the management of gastrointestinal stromal tumors.Ann Oncology 2005;16:566-578
    • CECT Heterogenous appearance with central necrosis and areas of cystic degeneration Extension to other structures Distant spread Low attenuating liver metastasis King DM.The radiology of gastrointestinal stromal tumors(GIST).Cancer Imaging 2005;5:150-156
    • MRI Solid portion-low intensity on T1 weighted and high intensity on T2 weighted images Enhancement with gadolinium
    • Endoscopic Ultrasound Smooth protrusion of bowel wall lined by normal mucosa Hypoechoic mass contiguous with fourth hypoechoic layer(muscularis propria) Benign Vs Malignant
    • Endoscopy Gastric and colorectal GIST Submucosal mass
    • Pre-op Biopsy Usually not done -Tumor seedling -Bleeding Endoscopic biopsy -Less bleeding -Confirm diagnosis
    • Treatment Surgical resection is preferred Locally advanced: Targeted therapy Radiation/Chemotherapy: Ineffective DemetriGD,BenjaminRS,BlankeCD,etal.NCCNTaskForcereport:managementof patientswithgastrointestinalstromaltumor(GIST)dupdateoftheNCCNclinicalpractice guidelines.JNatlComprCancNetw2007;5(Suppl2):S1–29
    • Surgical therapy Complete en-block removal Site specific Avoidance of tumor rupture Lymphadenectomy not advocated Final goal: complete tumor resection with a negative margin, intact pseudocasule Positive resection margin: Re-excision DeMatteo RP,Lewis JJ,Leung D et al.Two hundred Gastrointestinal stromal tumors: recurrence patterns and prognostic factors for survival.Ann surg 2000;231(1):51-8 Blay JY,Bonvalot S,Casali P et al.Consensus meeting for the management of gastrointestinal stromal tumors.Ann Oncology 2005;16:566-57
    • Site specific surgery Esophagus: esophagestectomy/esophageal sparing wide local excision Stomach Small-wedge resection Large-subtotal/total gastrectomy BlumMG,BilimoriaKY,WayneJD,etal.S urgical considerations for the management and Resection of esophageal gastrointestinal stromal tumors.AnnThoracSurg2007;84(5): 1717–23. WinfieldRD,HochwaldSN,VogelSB,etal. Presentation and management of gastrointes- tinal stromaltumors of the duodenum.AmSurg2006;72(8):719–22[discussion:722–3 WayneJD,BellRHJr.Limited gastric resection.SurgClinNorthAm2005;85(5):1009–20, vii.
    •  Small intestine Duodenum: Partial duodenal resection/Whipple’s Small Intestine: Segmental resection Colorectum Colon: Colectomy Rectum: Anterior resection/Abdominoperineal resection Extra-intestinal: En block resection with adequate margin Berman J,O’Leary TJ.Gastrointestinal stromal tumor workshop.Hum Pathol 2001;32:578-582 Blay JY,Bonvalot S,Casali P et al.Consensus meeting for the management of gastrointestinal stromal tumors.Ann Oncology 2005;16:566-57
    • Molecular targeted therapy(TKI) Joensuu and colleague(2001) Success: Lack of progression Standard starting dose :400 mg/day Ideal dose: not determined Neoadjuvant role: -Severe organ dysfunction (eg: for rectal or esophageal tumors) -Negative margin difficult Resistance: Primary/Secondary
    • Imitanib trialsTRIALS DOSE PARTIAL STABLE PROGRES COMMENTS RESPONSE DIS SEORTC 400,600,800 51% 31% 8% TTR 1WK2001,2002 or 1000mg/d MTD 800mg/dUS 400mg/d 67% 16% 17% No differenceMULTICENTER 600mg/d 66% 18% 8%2002,2004EORTC 400mg/d 50% 32% 13% 32% severe tox2003 800mg/d 54% 32% 8% 50%severe tox Improved PFS for 800mg/dINTERGROUP 400mg/d 49% 22% 36%severe tox2003 800mg/d 48% 22% 52%severe tox No difference in PFS TTR=Time to recurrence, MTD=Maximal tolerated dose, PFS=Progression free survival GoldJS,DeMatteoRP.Combined surgical and moleculartherapy:the gastrointestinal stromal tumor model. AnnSurg2006;244:176
    • Newer Approaches SUNITINIB: multitargated tyrosine kinase inhibitor HACE/RFA: liver metastasis Other TKI: -Nilotinib -Mastitinib -BMS-354,825 KobayashiK,GuptaS,TrentJC,etal.Hepatic artery chemoembolization for 110 Gastrointestinal stromal tumors.Cancer2006;107(12):2833–41.
    • Summary Rare Mostly sporadic and single Anywhere in GI Tract- Stomach MC Evaluation – EUS, CT, PET CT Varied clinical presentation- GI bleed MC Treatment of choice – Surgery, potentially curative
    • Summary Regular follow up Imatinib mesylate ( both neoadjuvant and adjuvant) Definite role Improved outcome Problem - Resistance to imatinib High recurrence
    • Currently Available TrialsNeoadjuvant study  RTOG S-0132/ACRIN 6665  Patients with recurrent or measurable peritoneal disease  8 wks Imatinib followed by resection
    • Currently Available TrialsAdjuvant study EORTC 64024 Patients with R0 resections eligible Patients stratified according to risk factors Patients randomized to either  Imatinib 400 mg/day X 2 years  Observation