Sinonasal Lymphoepithelial Carcinoma: Two Case Reports of a Rare Malignancy

Introduction: Lymphoepithelial carcinoma is a rare malignancy that was originally described in 1921 by Schminke and Regaud. There are few cases of this malignancy reported to have occurred in the sinonasal cavity. It shares some histological features with nasopharyngeal carcinoma, but is a histologically distinct disease. Lymphoepithelial carcinoma is associated with men in their fifth to seventh decades of life, Epstein-Barr virus (EBV), and regions where EBV is endemic, though not all cases test positive for EBV on immunohistochemical testing. Because of its rarity, no clinical practice or treatment guidelines currently exist for this type of tumor. Case reports have described treatments with surgery, chemotherapy, radiation therapy, and combinations thereof, all with varying patient outcomes.

Methods: Here we report two unique cases of invasive sinonasal lymphoepithelial carcinoma.

Results: One patient presented with a painful left nasal bridge and underlying mass that had eroded through the nasal septum. On imaging, the mass was centered on the right ethmoid sinus and nasal septum. Biopsy was consistent with sinonasal lymphoepithelial carcinoma, and staged as T4aN0M0. The patient subsequently underwent neoadjuvant chemotherapy (gemcitabine and cisplatin) followed by definitive proton radiotherapy, and has had a complete response with no recurrence for the past 3 years.

The other patient was found to have a 3cm by 2cm mass centered in the left posterior ethmoid sinus with imaging also demonstrating resorption of the medial lamella of the cribriform plate bilaterally and extension into the olfactory cleft on the left. There was bilateral neck lymphadenopathy in levels 2 and 3. After biopsy in the operating room to confirm the diagnosis of invasive sinonasal lymphoepithelial carcinoma, the patient was recommended to undergo an MRI to determine dural involvement of the lesion and a PET/CT scan for final staging prior to undergoing chemoradiation therapy.

Conclusions: We hope to add to the sparse existing literature on this rare pathology for which no treatment guidelines currently exist. Our results thus far suggest that lymphoepithelial carcinoma may respond well to a treatment algorithm similar to that used for nasopharyngeal carcinoma, considering the duration of cancer free survival for a patient treated with platinum-based chemotherapy followed by proton beam radiation. Further follow-up and additional cases are needed to make stronger recommendations regarding treatments.

 

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No conflict of interest has been declared by the author(s).

Publication History

Article published online:
05 February 2024