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.

Primary Epithelioid Sarcoma of the Conchal Bowl in a 64-Year-Old Male: A Case Report and Review of the Literature

“…2,4,5,[16][17][18] Of note, conventional ES has rarely been reported in the head and neck, where it may be easily confused with basal cell carcinoma or other nonmelanoma skin cancer clinically and confused with poorly differentiated carcinoma or granuloma microscopically. 1,30 The presence of atypical epithelioid cells with abundant eosinophilic cytoplasm and EMA and pancytokeratin AE1/AE3 expression in the ear of an older adult made us initially consider squamous cell carcinoma. However, the palisaded growth pattern and pale nuclear chromatin made us also consider ES in the differential diagnosis.…”

Primary epithelioid sarcoma of the oral cavity: Review of literature and presentation of a rare entity

“…In the oral cavity, only four cases of primary ES and two cases of ES with metastatic spread to the oral cavity have been reported (Table 1). 3,5–8 …”

“…In the oral cavity, only four cases of primary ES and two cases of ES with metastatic spread to the oral cavity have been reported (Table 1). 3,[5][6][7][8] The first case of primary intraoral ES, reported in 1990 by Jameson et al, described a 20-year-old male with a nonpainful chronic ulcer of the anterior hard palate. 6 Epithelioid sarcoma with metastatic spread to the oral cavity was first published by Winter et al (2002).…”

“…1,2,4,5 It is exceedingly rare in the head and neck region, particularly in the oral cavity where only four cases of primary ES have been reported (refer to Table 1). 3,[5][6][7][8] Herein, we present a young female patient with primary epithelioid sarcoma of the anterior tongue. To the best of our knowledge, this is the third case of primary ES of the tongue to be reported in the literature.…”

Loading and release of cancer chemotherapy drugs utilizing simultaneous temperature and pH-responsive nanohybrid

“…Cancer is a category of disease implicating abnormal cell growth that invades or spreads to other destinations of the body [ 1 , 2 ]. Based on the tissue origin, solid malignancies is mainly divided into the following two types [ 3 , 4 ]: Carcinoma of epithelial cell origin (such as adenocarcinoma) [ 5 ] Sarcoma of non-epithelial cell origin (such as liposarcoma and osteosarcoma) …”