A 72-year-old man presented with a 2-year history of gradually progressive swelling of the upper eyelids (Fig. 1). Physical examination revealed swollen nontender eyelids, enlarged submandibular and parotid glands, and enlarged neck lymphadenopathy. T2-weighted magnetic resonance images showed enlargement of both lacrimal glands (Fig. 2). Excisional biopsy of the eyelid exhibited mantle cell lymphoma. Fluorodeoxyglucose positron emission tomography revealed abnormal uptake in the axillary and inguinal lymph nodes and stomach wall. A diagnosis of Ann Arbor stage IV mantle cell lymphoma was made.

Figure 1
figure 1

Bilateral, nontender, swollen eyelids with bilateral enlarged submandibular glands (green arrows) and parotid glands (green arrowheads).

Figure 2
figure 2

T2-weighted magnetic resonance images showing enlargement of both lacrimal glands.

Although most eyelid lesions are benign,1 malignancy may present in one or both eyelids. The most common primary eyelid malignancy is basal cell carcinoma, whereas the most frequent causes of eyelid metastasis are melanoma and breast carcinoma.2 Lymphoma is the most common orbital malignancy; lymphomatous involvement of the ocular adnexa can result from either primary or systemic disease.3 Lymphoproliferative lesions of the ocular adnexa represent 1–2% of all lymphomas and approximately 8% of all extranodal lymphomas.3

The patient was treated with chemotherapy, which resulted in improvement of the eyelid swelling, enlarged glands, and lymph nodes.