Hyalinizing Clear Cell Carcinoma of Base of the Tongue – Case Report and Review of Literature

Hyalinizing clear cell carcinoma (HCCC) is a type of salivary gland carcinoma which is a low grade epithelial tumor and follows an indolent course. The main modality of treatment is surgical excision with or without adjuvant radiotherapy. A 33-year-old female patient presented to us with dysphagia, change in voice, and right ear ache. Clinical examination revealed an 8 cm × 6 cm submucosal growth over the right side base of the tongue crossing midline and also extending to anterior two-third of the tongue. Magnetic resonance imaging and positron emission tomography scan revealed tumor involving bilateral base of the tongue as well as anterior tongue. Biopsy from the lesion revealed HCCC with Ewing sarcoma breakpoint region 1 gene arrangement. Upfront tracheostomy followed by intraoral surgery was performed and the specimen was removed in toto and wound closed primarily. Postoperatively, she was decannulated within a week and started full oral diet within 10 days. The final histopathology report indicated HCCC. The patient underwent 30 fractions of adjuvant RT and is on regular monthly follow-up with no signs of residual disease. HCCC is a low-grade epithelial tumor with an indolent course and rarely seen in the head-and-neck region. The ideal treatment modality is excision of the lesion with adequate margins with or without adjuvant treatment.