Primary Myxofibrosarcoma of the Esophagus: A Case Report and Literature Review

Background: Squamous cell, and adenocarcinoma are the most common histological types of esophageal tumors. Primary sarcomas of the esophagus are extremely rare, and even more rare myxofibrosarcoma. Case Presentation: We describe the case of a 42 years old male with a history of Plummer-Vinson syndrome, presenting with progressive dysphagia and weight loss. Clinical and radiological investigations were inconclusive. Histopathological analysis of biopsy specimens revealed a poorly differentiated spindle cell tumor with a myxoid stroma. Immunohistochemical profiling confirmed the diagnosis of grade II myxofibrosarcoma according to the FNCLCC grading system. Given the lesion's inaccessibility for surgical resection, the patient was treated with exclusive radiotherapy using Volumetric-modulated arc therapy (VMAT) at a total dose of 66 Gy in 33 sessions. The patient tolerated the treatment well, with a complete resolution of dysphagia by the end of treatment. Conclusion: This is the first reported case of a primary esophageal myxofibrosarcoma treated with exclusive radiotherapy. This case supports the role of radiotherapy as a curative option in non-operable cases.