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The Heart, Lung, and Esophageal Surgery Institute, University of Pittsburgh Medical Center, 200 Lothrop St, Suite C-800, Pittsburgh, PA 15213
(Email: apennathur{at}aol.com).
Gastrointestinal stromal tumors (GIST) are rare mesenchymal tumors of the gastrointestinal tract. They are found most commonly in the stomach, followed by the small bowel and colon, and they are least common in the esophagus. A characteristic feature of these tumors is the expression of CD-117 (c-KIT) protein, which is a transmembrane tyrosine kinase receptor. In this issue of The Annals, Lee and colleagues [1] present their experience in the treatment of esophageal GISTs.
This is an interesting study of a rare condition. However, there are several controversial issues in the management of this disease, including preoperative diagnostic workup, the extent of resection (ie, esophagectomy or enucleation), the approach to surgery (ie, open or minimally invasive), and the use of neoadjuvant or adjuvant therapy. Although all these controversies can not be settled with this series, this article [1] is interesting because it raises all these questions.
There are some issues that require emphasis. Appropriate patient selection is critical to optimize outcomes after treatment. The authors suggest that for small GISTs with low mitotic activity, local resection (ie, enucleation) may be sufficient, but larger, more aggressive GISTs require esophagectomy with adjuvant therapy. It is evident that complete surgical resection, achieved either by minimally invasive techniques or an open technique, is a critically important factor in the treatment of these patients. However, of concern, is a high incidence of recurrence despite complete resection, which in the current series was approximately 33% during a median follow-up of 4.4 years. This underscores the need for not only effective surgical resection, but also for effective systemic therapies to decrease recurrences.
Of interest is the American College of Surgeons Oncology Group (ACOSOG) Z9001 phase III randomized study of adjuvant imatinib (c-KIT inhibitor) versus a placebo, which was administered after complete resection of GISTs (> 3 cm). The preliminary results of this trial demonstrated that patients who received adjuvant therapy had a significantly better recurrence-free survival at 1 year (97%) than patients in the placebo arm (83%) [2]. While we await the final results of this trial, these early results are encouraging, and adjuvant therapy after complete resection may become the standard in the future, particularly for GISTs with high-risk features such as increased mitotic activity.
In summary, the previously described article [1] reports the results of surgical treatment of a rare disease, which we believe the readers will find very interesting. It is clear that we need larger multi-institutional studies to develop the best treatment strategy for our patients with esophageal GISTs.
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