Treatment and Prognosis

Surgical resection of the primary disease followed by observation is the conventional treatment for patients with GISTs and offers the best cure rate.48,56 For resectable GISTs, preop-erative histologic confirmation is usually not necessary as these tumors may bleed, rupture, or disseminate as a result of biopsy.17 For the same reasons, the tumor should be removed en bloc during surgery.57 A safety margin of normal surrounding soft tissue or bowel should be included if pos-

figure 30.10. Tumor recurrence in a 75-year-old man 14 months after resection of malignant mesenteric GIST. (A) Axial CT of the pelvis reveals a rim-enhancing soft tissue mass along the right ilio-psoas muscle (arrow) suspicious for tumor recurrence. Surgical sutures from prior bowel resection are identified in the left lower quadrant. (B) Axial CT of the pelvis at a level more inferior than (A) reveals a hypervascular mass (arrow) anterior to the urinary bladder compatible with tumor recurrence.

figure 30.10. Tumor recurrence in a 75-year-old man 14 months after resection of malignant mesenteric GIST. (A) Axial CT of the pelvis reveals a rim-enhancing soft tissue mass along the right ilio-psoas muscle (arrow) suspicious for tumor recurrence. Surgical sutures from prior bowel resection are identified in the left lower quadrant. (B) Axial CT of the pelvis at a level more inferior than (A) reveals a hypervascular mass (arrow) anterior to the urinary bladder compatible with tumor recurrence.

sible to reduce the risk of recurrence.48,58 Because lymph node metastases are rare, extensive lymphadenectomy is not routinely performed.

Because of the unpredictable behavior of GIST, follow-up by imaging is performed to assess for disease recurrence, even though there is no proof that early detection of recurrent GIST results in survival benefit.31 Disease recurrence has been reported in up to 80% of cases despite complete resection with pathologically proven negative margins.57 Although most recurrences occur within 2 years, tumors with low mitotic index may take more than 10 years to metastasize.35 Recurrence is commonly local and peritoneal, often associated with liver metastases (Figure 30.11). Lymph node involvement is unusual. Most metastatic GISTs are confined to the abdomen, unlike other soft tissue sarcomas, which metastasize to the lungs.59

Patients with recurrence have a poor prognosis.30 Arterial embolization, surgery, and irradiation have been ineffective in treating patients with metastases and recurrent disease. Until recently, drug therapy for patients with GISTs has also been ineffective. However, a promising new drug, STI 571 (imatinib mesylate, gleevec) has been recently introduced. This new drug inhibits tyrosine kinase and was first reported in a case of recurrent metastatic GIST that failed extensive surgery and chemotherapy.56 The authors reported favorable response to therapy after 1 month of treatment, using MRI and PET. The safety and effectiveness of this new therapy were subsequently demonstrated in patients with advanced unresectable or metastatic disease.60,61 Current studies demonstrate up to 69% of patients showing favorable response to therapy.61 However, it is still unclear how long the response to therapy will last and whether maintenance therapy is required.

figure 30.11. Recurrent malignant GIST of the small bowel in a 62-year-old woman. (A) Axial CT of the upper abdomen in the portal venous phase reveals a large partially necrotic liver mass, with peripheral enhancement and central low attenuation. (B) Axial CT at a level lower than (A) reveals additional enhancing paraaortic (arrows) and mesenteric root (arrowhead) masses, with central necrosis. These findings are consistent with peritoneal recurrence.

figure 30.11. Recurrent malignant GIST of the small bowel in a 62-year-old woman. (A) Axial CT of the upper abdomen in the portal venous phase reveals a large partially necrotic liver mass, with peripheral enhancement and central low attenuation. (B) Axial CT at a level lower than (A) reveals additional enhancing paraaortic (arrows) and mesenteric root (arrowhead) masses, with central necrosis. These findings are consistent with peritoneal recurrence.

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