Lymphoepithelial carcinoma of the submandibular gland: a rare case / Prempreet Kaur ... [et al.]

Lymphoepithelial Carcinoma (LEC) is a rare malignancy of the salivary gland, which is associated with Epstein-Barr virus (EBV). LEC of the head and neck is commonly seen in the nasopharynx. In Asia, the common head and neck cancer associated with dermatomyositis is nasopharyngeal carcinoma. We report a case of a 46-year-old Chinese lady with underlying dermatomyositis who presented with a left submandibular swelling of two years duration that was associated with numbness of the left hemi-tongue. The left submandibular swelling was firm, non-tender, mobile, and measured 2 x 2cm in size. Computed tomography (CT) showed a heterogeneous enhancement of the left submandibular gland with surrounding inflammatory changes suspicious of malignancy. Preoperative fine needle aspiration revealed reactive lymphadenopathy. Left sub-mandibulectomy was performed, which was subsequently confirmed as LEC. Patient then underwent a modified radical left neck dissection followed by chemoradiotherapy. One-year post-treatment the patient showed no evidence of recurrence. LEC is a rare salivary gland tumour that has been associated with the EBV. Patients with dermatomyositis have a high risk of developing nasopharyngeal carcinoma, particularly, in Southeast Asian populations. This case, highlights that clinicians should also have high index of suspicion of salivary gland malignancy in patients with dermatomyositis, as this is rare and not widely seen and reported.