Lymphoproliferative tumors involving the lacrimal drainage system: a major review

ABSTRACT

Purpose

To provide a literature review on lymphoproliferative lesions involving the lacrimal drainage system.

Methods

The authors performed a pubmed search of all articles published in English on lymphoma/leukemia of lacrimal sac and the nasolacrimal duct. Data analyzed include prevalence, demographics, clinical presentations, treatment outcomes of primary versus secondary lacrimal involvement, and recurrence rates.

Results

Lymphoma/leukemia of lacrimal sac presented at a mean age of 55 years. The majority of the tumors (63%) were primary involvement of lacrimal sac, bilateral involvement being more common in secondary than primary lacrimal sac lymphoma. Epiphora (96%, 98/102), swelling in the lacrimal sac region (75%, 77/102), and acute dacryocystitis (31%) were the most frequent presenting features. Thirty-six percent of the lesions were diagnosed incidentally while performing a dacryocystorhinostomy (DCR). Among primary sac lymphomas, diffuse large B-cell lymphoma (DLBCL) (43%) was the most common sub-type followed by MALToma (24%), unclassified B-cell lymphoma (21%), lymphoid hyperplasia (5%) and 3% each small lymphocytic lymphoma (SLL) and NK/T cell lymphoma. Management usually involves chemotherapy and/ or radiotherapy with or without surgical resection. Successful outcomes in terms of local disease control could be achieved in all the cases; however, 15% died of the systemic disease after a mean 18 months from the time of sac involvement. Aggressive lymphomas like NK/T-cell have the worst prognosis.

Conclusion

Lymphoproliferative involvement of lacrimal sac has a high incidence of acute dacryocystitis with a good response to chemotherapy. Epiphora in patients with leukemia/lymphoma should arouse suspicion of a lacrimal drainage involvement.

KEYWORDS:

• Nasolacrimal duct
• chemotherapy
• DLBCL
• lacrimal
• lacrimal sac
• leukemia
• lymphoma
• MALToma
• radiotherapy

Acknowledgment

We wish to thank Ms. Sabera Banu, LVPEI Hyderabad for her assistance in literature search.

Disclosure statement

The authors report no conflict of interests.

Financial disclosure

Mohammad Javed Ali receives royalties from Springer for his treatises “Principles and Practice of Lacrimal Surgery” and ‘Atlas of Lacrimal Drainage Disorders’ and His research is supported by the Alexander von Humboldt Foundation, Germany.