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Dr ganesh
GISTs
Originally classified as other tumors: leiomyoma,
leiomyoblastoma, leiomyosarcoma or schwannomas
GISTs
The most common mesenchymal neoplasm of the GI tract. 1%-3% of GI malignant tumors Recently described as a distinct clinical and histopathological entity: Type of sarcoma, a tumor of mesenchymal (connective tissue) origin
Epidemiology
4000-6000 new cases in the USA each year with annual incidence of 11-14 per 106 Occur mostly in patients with a median age of 60 years (40-80) No predilection for either gender Familial GISTs- autosomal dominant Type I neurofibromatosis (7% mostly in the small intestine without KIT mutations) Carney triad (GISTs + paraganglioma +pulmonary chondroma)
Incidence %
60-70
Small intestine
Colon Other (omentum, mesentery, esophagus)
20-30
<5 <5
Clinical Presentation
Often asymptomatic, especially early in tumor
development, discovered incidentally by CT or endoscopy
Symptomatic:
GI hemorrhage
Anorexia, weight loss, nausea, anemia, and
additional GI complaints
Diagnostic studies
CT for initial evaluation and surveillance for recurrence
EUS determines size and extent of the tumor FDG-PET reveals small metastases and establish baseline metabolic activity and assess therapy response Routine use of PET for surveillance after resection is not yet recommended
GIST
GIST The cellular morphology of GISTs ranges from 1.) Spindle cell type 70 percent 2.) Epithelioid type 20 percent 3.) Mixed type 10 percent
Staging
Tx -can not be assessed T0 no evidence of primary tumor T1 2 cm T2 >2 cm but 5cm T3 >5 cm but 10cm T4 >10 cm
Regional node
Nx- can not be assessed N0 - no nodal mets N1 regional node mets
metastasis
M0 no metastasis M1 distant metastasis
If incomplete resection/metastatic at presentation Median survival <1 year 5-year survival <35%
Treatment
Surgery remain primary Rx of GIST of any location Simple wedge resection is favoured For tumors of lesser curvature segmental resection to prevent luminal narrowing A resection margin of 1-2 cms sufficient Routine lymphedenectomy not recomended
Conclusions
Surgery in M1 patients still an individual decision. No data from randomized or prospectively controlled yet available Residual tumor resection is safe Resection of progressive tumor is less rewarding Multifocal resection is not recommended without considering the patients personal situation Our experience with systemic therapy is: it more often avoids emergency surgery
Therapy revolutionised by develoment of TK1 inhibitors like imatinib & sunitinib. Neoadjuant chemo : decrease extent of resection required, diminish the vascularity of tumor, R0 resection feassible <5% develop progressive ds during neoadjuant therapy
Resistance to Imatinib
Primary resistance : no achievement of stable disease or
progressing disease within 6 months of an initial clinical response (KIT exon 9 mutation or no detectable kinase mutation wild-type tumors)
Secondary resistance: disease progression after more than 6
months clinical response (new acquired kinase mutation in KIT or PDGF-R that interfere with Imatinib activity) Dose escalation of imatinib is the first step Use of other kinase inhibitors (Sunitinib) Surgery, radiofrequency ablation or hepatic artery chemoembolisation
Thank you