Key Points

• Adenocarcinoma and squamous cell carcinoma comprise 98% of all esophageal tumors. The remaining 2% of tumors include a wide range of diagnoses.
• Leiomyomas are the most common benign esophageal tumor and can potentially be resected endoscopically depending on the size and location of the tumor.
• Presenting symptoms of unusual esophageal tumors are often similar to adenocarcinoma and squamous cell carcinoma.
• Diagnosis is usually made after surgical resection, however endoscopic ultrasound (EUS) can be a useful tool to help diagnose these rare tumors before surgical resection (Figure 1).
• While endoscopic resection of rare malignant lesions is not recommended.