BACKGROUND: Although surgical resection of adenocarcinoma or squamous cell carcinoma of the esophagus is standard practice, the treatment strategy for other malignant rare esophageal cancers is still under debate. The aim of this study was to examine the treatment of rare malignant esophageal cancers and to evaluate the survival of these patients. METHODS: A retrospective review of all esophagectomies performed at Mayo Clinic from 1980 to 2014 (approximately 4,000 cases) identified 24 patients with histologic features other than adenocarcinoma or squamous cell carcinoma. Their medical records were reviewed for demographics, presenting symptoms, evaluation, surgical management, pathologic features, and short-term and long-term outcome. RESULTS: Pathologic identifications included small cell carcinoma, lymphoma, and undifferentiated carcinoma in 4 (16.7%) patients each and neuroendocrine, melanoma, leiomyosarcoma, sarcomatoid, sarcoma, and gastrointestinal stromal tumor in 2 (8.3%) patients each. The most common presenting symptoms included dysphasia in 91.7% patients (22/24), pain in 75.0% (18/24), and weight loss in 62.5% (15/24). Preoperative evaluation included barium swallow in 91.7% (22/24), computed tomography in 91.7% (22/24), positron emission tomography in 54.2% (13/24), esophagogastroduodenoscopy in 100% (24/24), and endoscopic ultrasonography in 29.2% (7/24) patients. The location of the tumor was at the gastroesophageal junction in 41.7% (10/24). There was no operative mortality, and 13 patients (54.16%) had at least one postoperative adverse event. The 1-year survival after esophagectomy was 69.7%, the 5-year survival was 42.7%, and the 10-year survival was 37.4%. CONCLUSIONS: Esophageal cancer with pathologic features other than squamous cell carcinoma or adenocarcinoma is rare. Esophagectomy for rare types of malignant esophageal cancers should be considered part of the effective treatment paradigm.
BACKGROUND: Although surgical resection of adenocarcinoma or squamous cell carcinoma of the esophagus is standard practice, the treatment strategy for other malignant rare esophageal cancers is still under debate. The aim of this study was to examine the treatment of rare malignant esophageal cancers and to evaluate the survival of these patients. METHODS: A retrospective review of all esophagectomies performed at Mayo Clinic from 1980 to 2014 (approximately 4,000 cases) identified 24 patients with histologic features other than adenocarcinoma or squamous cell carcinoma. Their medical records were reviewed for demographics, presenting symptoms, evaluation, surgical management, pathologic features, and short-term and long-term outcome. RESULTS: Pathologic identifications included small cell carcinoma, lymphoma, and undifferentiated carcinoma in 4 (16.7%) patients each and neuroendocrine, melanoma, leiomyosarcoma, sarcomatoid, sarcoma, and gastrointestinal stromal tumor in 2 (8.3%) patients each. The most common presenting symptoms included dysphasia in 91.7% patients (22/24), pain in 75.0% (18/24), and weight loss in 62.5% (15/24). Preoperative evaluation included barium swallow in 91.7% (22/24), computed tomography in 91.7% (22/24), positron emission tomography in 54.2% (13/24), esophagogastroduodenoscopy in 100% (24/24), and endoscopic ultrasonography in 29.2% (7/24) patients. The location of the tumor was at the gastroesophageal junction in 41.7% (10/24). There was no operative mortality, and 13 patients (54.16%) had at least one postoperative adverse event. The 1-year survival after esophagectomy was 69.7%, the 5-year survival was 42.7%, and the 10-year survival was 37.4%. CONCLUSIONS:Esophageal cancer with pathologic features other than squamous cell carcinoma or adenocarcinoma is rare. Esophagectomy for rare types of malignant esophageal cancers should be considered part of the effective treatment paradigm.