Sir,

Lymphoepithelial carcinoma (LEC), which often occurs in nasopharynx, is a rare entity in ocular adnexa. Only two cases involving lacrimal gland and one case involving nasolacrimal duct have been reported.1, 2, 3 We present the first case of LEC initially presenting in the lacrimal sac.

Case report

An 82-year-old Taiwanese man presented with a slowly enlarging lump in the left medial canthus with epiphora for 18 months. Ocular examination findings showed a non-tender mass with a 3 mm left proptosis and exotropia (Figure 1). Lower punctal irrigation produced a clear reflux through the upper punctum. CT revealed a soft tissue mass involving the left lacrimal sac (Figure 1). Otolaryngological evaluation excluded nasopharyngeal involvement. The patient underwent dacryocystectomy and the histological examination showed an LEC (Figure 1). Immunohistochemical stain was positive for cytokeratin 5, cytokeratin 6 in tumour cells and positive for CD3, CD20 in the stromal lymphoid cells (Figure 2). Marked reactivity for Epstein–Barr virus (EBV) early RNA was detected in tumour cells using in situ hybridization (Figure 2). The patient subsequently received 56 Gy of radiotherapy to the left orbit. However a 3 × 3 cm left submandibular mass was noted 6 months later. Fine needle aspiration of the left neck mass revealed an undifferentiated carcinoma. The patient underwent further modified neck dissection and the immunohistochemical staining of the lesion showed strong positive for cytokeratins in the tumour cells and positive for CD3, CD20 in the stromal lymphoid cells, consistent with LEC. As the patient rejected further chemotherapy, he was transferred for adjunct radiotherapy. He currently has no evidence of ocular and neck recurrence (12 months after initial diagnosis).

Figure 1
figure 1

(a) A firm mass is noted in medical canthal region of the left eye. (b) CT shows a soft tissue mass in the left lacrimal sac and extending above the medial canthal tendon. (c and d) The tumour shows fibrous septa, with aggregates of malignant epithelial cells surrounded by lymphoid cells (haematoxylin–eosin, × 100 and × 400).

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Figure 2
figure 2

Immunohistochemical staining shows positivity for cytokeratin 5 in the tumour cells (a, immunoperoxidase, × 200), and positivity for CD3, CD20 in the lymphoid cells (b and c immunoperoxidase, × 200). In situ hybridization tests for EBV early RNA were positive in the epithelial cells (d, EBV–encoded RNA in situ hybridization, × 200).

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Comment

LECs are neoplasms composed of undifferentiated malignant epithelial cells with dense lymphoid stroma. LECs in nasopharynx and salivary glands are often associated with EBV, particularly in the endemic areas where nasopharyngeal carcinoma is common.4, 5 However, in other sites, such association is still controversial. None of reported ocular adnexal LEC except the present case was noted to be associated with EBV.

From the published study, optimal treatment is based on surgery and radiotherapy. LEC seems to have a better prognosis than other types of malignancies of the same origin, perhaps because the lymphoid stroma could limit the aggressiveness of this carcinoma.5, 6 However, long-term follow-up is mandatory because of its tendency to metastasize.

Our case suggests that lacrimal sac LEC may be associated with EBV in the endemic area. Although rare, LEC must be considered in the differential diagnosis of the lacrimal sac tumour.