Summary | Surgery and Nutritional Support | Surgery or Gleevec?
Gleevec After Surgery (Adjuvant Treatment)| Surgery for Metastatic GIST

Surgical removal of the tumor(s) has historically been the primary treatment for Pediatric GIST. The types of operations have historically varied from a simple local excision to a total gastrectomy. In some cases, surgery provides long-term relief. In other cases, tumors return, either locally (near the original site, such as another stomach tumor(s)), or distant from the original site (metastases). The goal is to remove the tumor and to achieve clear margins. The nature of the surgery will depend on the size and location of the GIST tumor.

As Pediatric GIST is usually slow growing, but does seem to recur, you may wish to talk to the surgeon about the pros and cons of immediate versus delayed surgery.

Surgery and Nutritional Support

Short-Bowel Syndrome
Synonyms and related keywords: SBS, short gut syndrome, anenteric malabsorption syndrome, malabsorption, maldigestion, malnutrition, diarrhea, fluid disturbances, electrolyte disturbances, total parenteral nutrition (TPN), Living with a Gastrectomy, Diet after surgery, Nutrition problems and their solutions

Surgery or Gleevec?

In patients with adult GIST, Gleevec might be considered instead of surgery for some patients. In patients with Pediatric GIST however, Gleevec is not nearly as effective as it is in adults. Therefore, surgery is typically preferred in most cases. Gleevec or another drug is considered in cases where tumors have returned or when surgery is not possible.

Gleevec after surgery (Adjuvant Gleevec)?

On April 12th 2007, the American College of Surgeons announced that the Z9001 phase III adjuvant Gleevec trial for adult GIST had successfully met its endpoint. In adults, Gleevec does increase time to recurrence in a highly significant manner. However, since Gleevec is not as effective in Pediatric GIST, its use for preventative treatment for Pediatric GIST must be viewed with caution and a good deal of skepticism. The rare exception to this would be those few patients that are defined as Pediatric GIST because they were diagnosed under the age of 18, but they have a mutation in the KIT or PDGFRA gene that is sensitive to Gleevec. These rare cases probably resemble adult GIST more than Pediatric GIST and might be considered as better candidates for adjuvant Gleevec.

Surgery and Metastatic GIST

The role of surgery for metastatic Pediatric GIST is not well defined. In considering the role for this type of surgery two important aspects of Pediatric GIST should be considered:

1. The slow growing nature of the disease. Patients with metastatic Pediatric GIST and Carney's Triad frequently live long lives even with metastatic disease.
2. Gleevec, the mainstay of treatment for metastatic adult GIST, appears to be less effective in Pediatric GIST.

1. Choi H, Charnsangavej C, Macapinlac HA, et al. Correlation of computerized tomography (CT) and proton emission tomography (PET) in patients with metastatic GIST treated at a single institution with imatinib mesylate [abstract].
Proc Am Soc Clin Oncol. 2003;22:819. Abstract 3290.

2. DeMatteo RP, Lewis JJ, Leung D, et al. Two hundred gastrointestinal stromal tumors: Recurrence patterns and prognostic factors for survival.
Ann Surg. 2000;231:51-58.

3. Hohenberger P, Bauer S, Schneider U, et al. Tumor resection following imatinib pretreatment in GI stromal tumors [abstract].
Proc Am Soc Clin Oncol. 2003;22:818. Abstract 3288.

4. van Coevorden F, Peterse H, Rodenhuis S. Is there a role for post imatinib (salvage) surgery in gastrointestinal stromal tumors [abstract]?
Program of the Connective Tissue Oncology Society 9th Annual Scientific Meeting. 2003:40. Abstract 147.