B. Parish Budiono Surgery Department Digestive Surgery Division Faculty of Medicine Diponegoro University/Dr.

Kariadi Hospital

GIST
Rare mesenchymal tumors US : 5000-6000 cases per year Affect men and women equally Age 40-80 years old (median age 60)

Tumor identified biopsy is typically reserved for cases of :

Diagnostic uncertainly Unresectable lesions When Neoadjuvant therapy is being considered

Biopsy
Prevent rupture of pseudocapsule and intraperitoneal tumor dissemination Fine-needle aspirates / Core-needle biopsy inconclusive because of the submucosal location and the necrotic centers

Surgical Management of GIST depending :

The Size The exact anatomical Location Specific location of the GIST relative to the blood supply of the involved organ Wheter or not the GIST has adhered to surrounded or invaded adjacent structures The patients overall medical condition

Small (<2cm) asymptomatic tumors (incidentally) controversy treatment

Chest X-Ray Abdominal-Pelvix CT EUS small lession (<2cm) Percutaneous biopsy should not be used if tumor is considered resectable

Primary Resectable Disease

Primary Unresectable Disease Recurrent and Metastatic Disease

Surgery : mainstay treatment of Localized resectable GIST A complete gross resection with preservation of an intact (pseudo) capsule and negative microscopic margins (R0) Wide margins not improve outcomes Lymphadenectomy is not routinly required En bloc resection is needed when adjacent organs appear to be involved

The abdomen should be thoughly explored for evidence of metastatic disease GIST tend to displaced stucture (most sarcomas) rather than invade them Final pathologic examination reveals microscopically positive margins
Contoversy Reoperation and marginal resection ? R0 and R1 long term outcome : similar R2 worst outcome subtotal resection should be avoided

Microscopically (+) Margins :

Risk and benefit of re-exicision Watchful waiting Postoperative Imatinib

Laparoscopy has emerged as a helpful tool in the treatment of GIST (<5/10 cm)
Safe and feasible Oncologic safety Minimizing tumor manipulation Tumor Rupture Intraperitoneal Spread Wound protecting device (hand port) Endobag

Endoscopic resection in not recomended (>2cm)

Risk of positive margins Perforation and Tumor spillage

GIST <2cm EUS no High Risk

Surveillance endoscopy every 6-12 months Endoscopi resection

High Risk on EUS Surgical resection

Large size Irregular extraluminal border Heterogenous echo pattern Presence of cystic spaces Echogenic foci

85% of Px with Localised Primary GIST : Complete resection 70-95% : Negative microscopic margins Surgery alone recurrence rate 50% (irrespective of negative margins) 5-year survival : 50%

Most common site (70%) Surgical approach :

Omentectomy : not routinly Location :

Size and Location Biopsy is not necessary (unless with M1)

En-bloc resection : splenectomy and/or distal pancreatectomy

Greater Curv/Fundus : Sleeve or Wedge gastrectomy Incisura/Antrum : Distal Gastrectomy Lesser Curv/GE junction : Partial Gastrectomy/Total Gastrectomy

Second most common (20%) : Jejunal - Ileal - Duodenal Resection of the involved bowel with Negative Margins Primary Anastomosis Can be performed Laparoscopically either intra- or extracorporeal anastomosis

Duodenal Lesions : <5% Difficult surgical problem Location :

Small Proximal D1/D2 Tumor : Wide excision Large tumor/Close to the major papilla : Pancreaticoduodenectomy Distal D3/D4 : Segmental resection + duodenojejunostomy anastomosis

Rare : 3-5% Increases in the more distal colon, rectum for majority of tumor Rectal GIST : difficult
Involvement of surrounding structure R0 difficult to obtain Average positive margin up to 40%

Operative plan : depends on proximity of the tumor to the internal sphincter Mesorectal excision and wide margin are not needed
LAR APR

Colonic GIST
Marginal resection with primary anastomosis In the setting of perforation and gross contamination colostomy and mucous fistula may be prefered risk of anastomotic dehiscence Formal lymphadenectomy is not necessary

Rare : <5% Operative approach : tumor size and location relation to the GE junction
Small lession (<2cm) : Transmurally resected alonf the longitudinal axis w/ Transverse closure Larger lession/within GE junction : esophagectomy

Treatment options were very limited in the pre-TKI era GIST : respond poorly to chemo-tx or radiation-tx General rule :
R1/R2 resection Debulking Surgery Mutilating Surgery Not recommended

Imatinib is the standart treatment

Neoadjuvant Imatinib 2 CT scan evaluation surgical option Incomplete resection/debulking only performed in the setting of palliation for bleeding, obstruction, perforation Timing of resection : imatinib for 6 months

Cytoreductive Surgery :
Stable or responsive to TKI therapy when complete gross resection is possible Emergencies situation : hemorrhage, perforation, obstruction or abscess