Lymphoma of the Lacrimal Sac: The Massachusetts Eye and Ear Experience With a Comparison to the Previously Reported Literature

Abstract

Purpose: To describe the frequency, clinical features, and histologic subtypes of biopsy proven lacrimal sac lymphomas, and to compare these results to the previously published literature. Methods: A retrospective chart review was performed at a single institution from 2004 to 2017. Pathology reports, operative notes, and patients’ medical charts were reviewed. Results: Of 566 lacrimal sacs submitted for routine histopathologic evaluation, 16 cases of lymphoma were identified. All were low-grade, non-Hodgkin B-cell lymphomas, biopsied at an average age of 71 years. Thirteen patients (81.25%) had a pre-existing lymphoma diagnosis; the average interval between the diagnosis of systemic or nonocular adnexal lymphoma and lacrimal sac lymphoma was 7.9 years (range 2–26 years; median 5.5 years). Three cases of primary lacrimal sac lymphoma were identified. Histopathology showed 3 cases (18.75%) of follicular lymphoma, 3 (18.75%) of extranodal marginal zone lymphoma, and 10 (62.5%) of chronic lymphocytic leukemia/small lymphocytic lymphoma. Primary cases presented with epiphora and nasolacrimal duct obstruction, while secondary cases predominantly manifested as dacryocystitis. All lacrimal sac neoplasms were locally responsive (without local recurrence) to chemotherapy, radiation, or both. Conclusions: Lacrimal sac lymphoma is uncommon but should be suspected among patients with known lymphoma who develop dacryocystitis. In this series, primary lacrimal sac lymphoma most often presented as a mass or nasolacrimal duct obstruction. Chronic lymphocytic leukemia/small lymphocytic lymphoma was the most commonly identified cause of secondary lacrimal sac lymphoma. Distinguishing primary from secondary lacrimal sac lymphomas is important, as the extent of disease and histopathologic subtypes differ, which may affect patient management.

Lacrimal sac lymphoma: a case series and literature review

“…Lymphoma is the major type of non-epithelial malignant LSTs, accounting for about 6%-13% of all lacrimal sac malignancies [2,7,10,[13][14] . LSL may be primary and solitary without systemic involvement [14][15][16] or secondary to leukemia [17] . Older patients who develop symptoms of dacryocystitis with a known course of systemic lymphoma should be highly suspected for lymphoproliferative infiltration in the lacrimal drainage system [18] .…”

Primary lacrimal sac lymphoma: a case-based retrospective study in a Chinese population

“…Although Singh and Ali [4] found more reports of primary than secondary lacrimal sac lymphoma, there could have been a possible publication bias. Neerukonda et al [8] recently reported more cases of secondary than primary lacrimal sac lymphoma. Extranodal NK/T-cell lymphoma, nasal type is a rare and highly aggressive non-Hodgkin's lymphoma (NHL) [20] that is more common in Asia and Central America than in the United States and Europe [21] and is highly associated with EBV infection [6,[21][22] .…”

“…Although lymphoma patients typically have systemic symptoms, lacrimal sac lymphoma manifests as an isolated ocular appendage lesion [5] . Primary lacrimal sac lymphoma refers to a lesion that originates from the lacrimal sac without systemic lesions, or the early stage of disease when there is no systemic disease [5] Previous studies have characterized populations with lacrimal sac lymphoma in Europe [5] , the United States [8] , Japan [7] , and China [9] . No literature has been found to assess tumor markers Int J Ophthalmol, Vol.…”

Ocular adnexal lymphoma: Subtype‐specific clinical and genetic features

“…In the conjunctiva, the mass typically has an appearance of a salmon pink patch, which may be lobulated in the case of FL (Taghipour Zahir et al 2013;Mahdavi Fard et al 2018). Lacrimal sac tumours frequently present with epiphora due to obstruction by the tumour (Venkitaraman & George 2007;Neerukonda et al 2022).…”

“…In the conjunctiva, the mass typically has an appearance of a salmon pink patch, which may be lobulated in the case of FL (Taghipour Zahir et al 2013; Mahdavi Fard et al 2018). Lacrimal sac tumours frequently present with epiphora due to obstruction by the tumour (Venkitaraman & George 2007; Neerukonda et al 2022). Orbital tumours and tumours of the lacrimal gland frequently entail exophthalmos, limited eye movement and displacement of the globe (Olsen et al 2019; Vest et al 2020).…”