Sarcomatoid Carcinoma of the Esophagus

Background: Sarcomatoid carcinoma of the esophagus is an unusual malignancy with a biphasic histological appearance containing both epithelial and mesenchymal elements. The most common epithelial component is squamous cell carcinoma; the spindle cell component is typically high grade. These tumors usually present as large pedunculated masses with intraluminal growth but show minimal invasion. Aims: We report three cases of esophageal sarcomatoid carcinoma with relatively uncommon morphologic and clinical features, and further discuss the biological and clinical behaviors of this rare tumor. Methods: The pathology database was retrospectively searched and three cases of esophageal sarcomatoid carcinoma were retrieved. These cases were thoroughly reviewed and summarized. Results: All three tumors exhibited a prominent spindle cell component with variable nuclear pleomorphism and cytokeratin/p63 expression. Osteoid formation was present in one case. The epithelial component also varied. Case1 contained keratinizing squamous cell carcinoma with in situ component, composing of 10% of the tumor volume. In case 2, the bulk of the tumor was non-keratinizing basaloid squamous cell carcinoma without carcinoma in situ. Case 3 was biopsy only and no carcinomatous component was present. These tumors also showed unusual clinical behaviors. In case 1, the patient developed peritoneal metastasis and anastomotic recurrence at 4 months after surgery despite clear margins and exophytic tumor growth with only focal muscularis porpria invasion. In contrast, in the other two cases with invasive tumor growth, both patients survived more than 5 years. Conclusion: Many aspects of sarcomatoid carcinoma remain unclear. Further investigation is necessary to elucidate the etiology, pathogenesis, biological and clinical behaviors of this rare tumor.