Esophageal gastrointestinal stromal tumor (GIST) is a rare neoplasm that arises from interstitial cells of Cajal that typically requires surgical resection due to its potential for aggressive behavior. These tumors can affect any site of the digestive tract, from the esophagus to the rectum. Though they arise from the submucosal layer, they can ulcerate through the mucosa or form pedunculated masses. Esophageal GIST generally has a worse outcome compared to tumors arising in the stomach. The preoperative evaluation includes imaging and endoscopic ultrasound (EUS) to obtain a tissue biopsy. For large, locally advanced, or metastatic tumors, neoadjuvant tyrosine kinase inhibitor therapy should be strongly considered. Tumor genotyping can help identify imatinib non-responders or those requiring a higher dose. Due to the rarity of regional nodal metastasis, surgical options include esophagectomy, tumor enucleation, and submucosal tunneling endoscopic resection (STER). Given a high risk of postoperative morbidity, esophagectomy should be avoided in favor of the less invasive enucleation or endoscopic resection. Thoracoscopic/laparoscopic and robotic-assisted enucleation and STER are minimally invasive operative approaches that appear to be safe with adequate oncologic outcomes based on currently available evidence. Adjuvant therapy should be considered for high-risk tumors, though the optimal duration of therapy remains under investigation.
- Endoscopic resection
- Gastrointestinal stromal tumor (GIST)
- Minimally invasive surgery
ASJC Scopus subject areas
- Radiology Nuclear Medicine and imaging