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Esophageal GIST

Gastrointestinal stromal tumors (GISTs) of the esophagus are rare, comprising only about 1% of reported GISTs.  Dr. Blum and colleagues recently reported on a series of four esophageal GIST cases treated since 2002 at Northwestern Memorial Hospital. A link to the abstract is given at the end of this piece.  In addition, they reviewed the literature on esophageal GIST and its treatment.

Matthew G. Blum, MD is the head of the general thoracic surgery section at Northwestern Memorial Hospital in the Chicago area.  He practices general thoracic surgery and has special interests in surgical management of lung and esophageal malignancies and minimally invasive thoracic surgery.   As a faculty member of the Northwestern University Feingold School of Medicine, Dr. Blum has won several teaching awards.  He has numerous publications and serves as a reviewer for a number of medical journals.

Below are Dr. Blum's answers to questions about esophageal GIST posed by GIST Support International.

1. What unique anatomical challenges to surgical resection does GIST of the esophagus present?

The esophagus is located beneath the breastbone (sternum), posterior to the heart and between the lungs, making it dificult to access.

2. How do the layers of tissue in the wall of the esophagus differ from those of the stomach wall, and why is this important?

Unlike the walls of the stomach, small bowel, and colon, which have 3 layers (mucosa, muscularis and serosa), the esophagus lacks the serosa and thus has only 2 layers.  GISTs arise from the middle layer, and the serosa provides an additional layer through which a GIST would have to penetrate before growing into adjacent organs.  The lack of the serosal layer means that tumors of the esophagus more readily grow into adjacent organs and tissues.  

3. What operations may be used for esophageal GIST and what general factors determine which one is needed for an individual patient? 

Operations for esophageal GISTs should generally include removing the tumor and the surrounding esophagus (esophagectomy). There are several ways of performing esophagectomy and several different methods for replacing the esophagus.  For esophageal GISTs the best resection would be one that includes a chest approach (Ivor Lewis, Ivor Lewis-McKeowan, or left thoracoabdominal).  For small esophageal GISTs (<2cm) or for patients with limited lifespan or who can not tolerate esophagectomy, enucleation or “shelling-out” of the tumor from the esophagus might be considered.  In a patient who had a tumor enucleated, but could undergo esophagectomy, I would recommend esophagectomy if the tumor had large numbers of mitosis or other factors that suggested that it was more aggressive.   Segmental resection of the esophagus is generally not possible.  The esophagus is a straight tube without extra length.  Removing a section of it is like removing a piece of pipe. The gap has to be spanned by something else (stomach, colon, or small bowel).

4. Can pre-operative (neo-adjuvant) imatinib potentially reduce the scope of surgery? 

Although yet to be proven in randomized studies, larger esophageal GISTs may be easier to resect completely if their size can be reduced by giving imatinib. 

5. If a patient must lose the gastro-esophageal junction in surgery, what consequences does this have for eating and for digestive function? 

Resections of the gastroesophageal junction require removing a portion of the stomach and/or the esophagus.  Changes in the types of food, quantity of food and meal frequency often need to be made depending on the resection necessary and what type of reconstruction is undertaken.  Esophagectomy patients (like those undergoing esophagectomy for esophageal cancer) usually eat several smaller meals a day.  Every person is different will have to experiment to see what foods they tolerate.  Reflux is very common after esophagectomy.

6. Can endoscopic biopsy differentiate esophageal GIST from the more common esophageal leiomyomas? 

Endoscopic biopsy can differentiate most GISTs from leiomyomas by staining the cells for c-KIT.   The biopsy is relatively safe.  My preference is to have large (>2cm) esophageal leiomyomas biopsied to rule out GIST.   Smaller tumors that appear to be leiomyomas will not always be biopsied prior to enucleation.  If they are found to be GIST after enucleation consideration should be given to completion esophagectomy. 

7. Is esophageal GIST more likely to metastasize to the lungs than GIST at other anatomical locations?

No.

8. Please summarize your major conclusions from your recent case series paper plus literature review. 

Esophageal GISTS    

  • are difficult to differentiate from leiomyomata without biopsy
  • are best treated with complete resection if possible
  • usually require esophagectomy and reconstruction 
  • may be easier to remove if treated with Imatinib first
  • should be treated at centers familiar with GIST management and thoracic approaches to esophageal surgery. 

For more details, see the authors’ recent paper.  Click the title below for the abstract in PubMed.

Blum MG, Bilimoria KY, Wayne JD, de Hoyos AL, Talamonti MS, Adley B.
 Surgical considerations for the management and resection of esophageal gastrointestinal stromal tumors.
Annals of Thoracic Surgery. 2007 Nov;84(5):1717-23.
PMID: 17954092

For a drawing illustrating esophagectomy, see Surgical Procedures for Primary GIST.



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