Primary lacrimal sac Burkitt lymphoma: A case report and literature review

PURPOSE: To highlight a case report of high-grade primary lacrimal sac Burkitt lymphoma in a young adult.

OBSERVATION: A 25-year-old gentleman was referred to the oculoplastic center for left eye medial canthal progressive swelling at the level below the medial canthal tendon for two months associated with tearing. He was initially treated for preseptal cellulitis but failed to respond to antibiotics. Both eyes vision were 6/9, and the ocular examination showed a left medial canthal non-tender, firm mass 2cm by 3 cm in size, immobile, smooth surface, and inflamed overlying skin. Otherwise, both anterior segment and fundus examinations were unremarkable. Contrasted CT orbit revealed a 3cm enhanced lesion around the lacrimal system region with extension into the middle nasal hiatus and maxillary bone destruction. Left incisional biopsy and tumor debulking were done. Intraoperative findings were a whitish, firm mass with bony destruction at the left maxillary bone. Histopathological examination showed high-grade B cell lymphoma and areas of starry-sky appearance. Immunological markers (CD20, CD79a, CD10, MUM1, BCL6, and BCL2) were positive. Left nasolacrimal sac Burkitt lymphoma diagnosis was made, and CT-thorax-abdomen-pelvis reported no distant metastasis. The patient was referred to a haemato-oncologist and completed six cycles of R-block chemotherapy. The patient is currently in complete remission at six months.

CONCLUSIONS AND IMPORTANCE: Lacrimal sac Burkitt lymphoma can occur in young, healthy adults; however, a high index of suspicion is mandatory in young patients with preseptal cellulitis associated with medial canthal swelling and recurrent acute dacryocystitis.