Epithelioid Sarcoma of the Tongue: An Unusual Tumor Mimic for Squamous Cell Carcinoma

Abstract

Introduction: Epithelioid sarcoma is a rare soft tissue malignancy that usually presents in the distal extremities along fascial planes, aponeuroses or tendon sheaths. Very rarely, it presents as a primary or metastatic lesion of the head neck. Methods: Chart review and comprehensive literature review using PubMed and Google Scholar. Results: A 17-year-old non-smoker was referred for evaluation of an ulcerative lesion of the right anterior-lateral tongue, progressing over several months. Incisional biopsy was concerning for squamous cell carcinoma. He underwent partial glossectomy and bilateral selective neck dissections, with reconstruction using a radial forearm free flap. Final pathology was consistent with epithelioid sarcoma, proximal type, demonstrating perineural invasion and close margins. Post-operative PET scan showed no persistent nor metastatic disease. He underwent post-operative radiation therapy to a total dose of 56 Gy to the primary site. Conclusion: Epithelioid sarcoma is a rare malignancy usually presenting in the extremities of young adults, which uncommonly presents in the head and neck as a primary or metastatic lesion. The infrequency of these lesions has prevented development of evidence-based treatment recommendations. As with most sarcomas, surgery is the mainstay of therapy for epithelioid sarcoma, while radiation has been used in an adjunctive role. Although a rare lesion, epithelioid sarcoma should be considered in the differential diagnosis for atypical epithelioid lesions of the tongue and oral cavity when patient demographics, gross lesion characteristics, or histopathology are not entirely consistent with more common lesions, such as squamous cell carcinoma.