Question: A 78-year-old man presented with a 2-month history of progressive dysphagia to solids. His past medical history revealed heavy tobacco use and chronic obstructive pulmonary disease. He denied any recent history of weight loss or a history of food impaction. On presentation, there were no palpable lymph nodes or liver enlargement. Laboratory data were not pertinent except for slight elevation of SCC to 3.6 ng/mL. A barium esophagogram revealed a 5-cm filling defect in the esophagus 20 cm distal to the incisors. Upper GI endoscopy demonstrated a large, elastic, circumferential mass almost entirely occupying the lumen in the upper esophagus, but the endoscope easily passed the lesion (Figure A). What is your diagnosis? See the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI. Pathologic findings obtained from biopsy specimens revealed spindle-shaped cells as well as moderately differentiated squamous cell carcinomas in the same view, and there was a transitional zone (Figure B, arrow) between the sarcomatous (above the arrow) and the carcinomatous components (below the arrow). Computed tomography showed regional lymph node metastasis, but no distant metastasis. Immunohistochemical staining for cytokeratin AE1/AE3 antibodies were positive in the spindle-shaped cells (Figure C), which confirmed the diagnosis of carcinosarcoma of the esophagus (cT3N1M0, stage III). This patient was not a candidate for esophagectomy because of his age and advanced lung disease, so he was treated with chemoradiation with remarkable response (Figure D).View Large Image Figure ViewerDownload Hi-res image Download (PPT) Carcinosarcoma of the esophagus is an uncommon malignancy of the esophagus with both carcinomatous and sarcomatous components, and it represents only 1%–2% of all esophageal neoplasms. There were 2 major proposed explanations for the pathogenesis of carcinosarcomas: The metaplastic and the collision concepts. The metaplastic concept is that individual elements might be derived from a single common ancestor cell (so-called carcinosarcoma), whereas the collision concept is that 2 individual stem cells may independently and simultaneously undergo malignant transformation (true carcinosarcoma).1Iwaya T. Maesawa C. Uesugi N. et al.True carcinosarcoma of the esophagus.Dis Esophagus. 2006; 19: 48-52Crossref PubMed Scopus (17) Google Scholar Recent studies have supported the metaplastic concept of carcinosarcoma. Therefore, most esophageal carcinosarcomas are diagnosed as a so-called carcinosarcoma. This tumor often presents as a bulky intraluminal polypoid lesion, and thus often causes symptoms of dysphagia.2Madan A.K. Long A.E. Weldon C.B. et al.Esophageal carcinosarcoma.J Gastrointest Surg. 2001; 5: 414-417Crossref PubMed Scopus (48) Google Scholar Esophageal carcinosarcomas are usually treated according to same protocol as other esophageal cancers. This case highlights that esophageal carcinosarcoma should be considered in the differential diagnosis of esophageal tumors growing intraluminally.
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