Clear Cell Sarcoma of the Nasal Soft Tissue Envelope

Abstract Objective(s) Clear cell sarcoma (CCS) is a rare clinical entity with limited head and neck reports. This is the first known case of primary CCS of the nasal soft tissue envelope. The primary objective is to understand the clinical and histopathologic features of CCS of the head and neck. Secondary measures are to recognize the wide differential diagnosis of nasal soft tissue masses and identify treatment strategies to achieve complete resection of nasal soft tissue tumors while optimizing functional and aesthetic results. Methods Case report. Results A 38-year-old otherwise healthy Caucasian male presented with a 2 cm subcutaneous mass of the nasal soft tissue envelope. Confined to the lateral nasal wall, the painless mass gradually increased in size over two years and contributed to nasal obstruction. The patient underwent complete resection via an open septorhinoplasty approach with concomitant functional nasal airway surgery. Pathology, immunohistochemistry, and cytogenetics were consistent with CCS (melanoma of soft parts). As no definitive or established adjuvant medical therapy exists, further treatment was suspended in favor of serial imaging. The patient remains clinically free of disease 24 months following diagnosis with subjective improvement in nasal airflow and cosmesis. Conclusion The differential diagnosis of soft tissue nasal masses is varied. Surgeons should maintain a high index of suspicion for more insidious pathology in otherwise benign-appearing nasal soft tissue masses. An open rhinoplasty approach may be prudent for larger lesions to achieve complete resection with restoration of form and function.