Gastrointestinal stromal tumors (GISTs) of the esophagus are rare, comprising only about 1% of reported GISTs. Dr. Blum and colleagues who reported in 2007 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.
Dr. Matthew Blum is a general thoracic surgeon at UCHealth Thoracic Surgery – Memorial Hospital Central who specializes in chest diseases, including lung and esophageal cancer as well as benign diseases of the trachea, mediastinum, chest wall, lungs, and esophagus.
“Within the last several years, minimally invasive surgical techniques have been developed that have radically improved the recovery of thoracic surgery patients. I currently use minimally invasive techniques for over 80 percent of procedures,” Blum says. “However, the best surgical techniques are not effective if they are used without consideration of the patient’s physical, emotional and social situation. Additionally, proper selection of procedures requires an experienced, in-depth understanding of the diseases for which they are being used. I have developed this understanding through 15 years of writing scholarly articles, teaching thoracic surgery and participating in national and international organizations.”
Memorial Hospital’s affiliation with the University of Colorado School of Medicine has enabled Blum to continue pursuing research interests in clinical trials for lung cancer patients and early detection of lung cancer.
Dr. Blum won several teaching awards as a faculty member of the Northwestern University Feingold School of Medicine. 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 â€œshelling-outâ€ 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?
8. Please summarize your major conclusions from your recent case series paper plus literature review.
- 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’ 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.
For a drawing illustrating esophagectomy, see Surgical Procedures for Primary GIST.