Case Series and Literature Review: Lymphoma of the Lacrimal Sac

Lymphoma of the lacrimal sac is quite uncommon, representing only about 2.5% of ocular adnexal lymphomas, and representing only about 1% to 2% of all non-Hodgkin's lymphomas. Unlike lymphomas in other areas of the ocular adnexa, lacrimal sac lymphoma more often arises secondary to systemic lymphoproliferative disorders, and should be considered a distinct entity from other ocular adnexal lymphomas.

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Clinician scientists at Mass Eye and Ear/Mass General Hospital Department of Ophthalmology reviewed all cases of lacrimal sac lymphoma diagnosed there over a 13-year period and compared them with previously reported literature. Senior author Natalie Wolkow, MD, PhD, an oculoplastic surgeon, and her colleagues Vamsee K. Neerukonda, MD, a former pathology fellow at Mass Eye and Ear, and Anna M. Stagner, MD, the Frederick A. Jakobiec director of the David Glendenning Cogan Laboratory of Ophthalmic Pathology, shared their findings in Ophthalmic Plastic and Reconstructive Surgery.

Case Series

Between November 1, 2004, and November 1, 2017, 566 lacrimal sac specimens underwent histology, most after being removed during routine dacryocystorhinostomy for nasolacrimal duct obstruction. Lacrimal sac lymphoma was found in 16 cases (2.8%):

• Primary lymphoma diagnosis—Three patients (19%), average age 52 (range, 35–71). Nasolacrimal duct obstruction and epiphora were the most common presentations. One case presented as a preoperative mass; the other two were detected intraoperatively
• Secondary lymphoma diagnosis—13 patients (81%), average age 75 (range, 61–85). The most common presentation was dacryocystitis

Classified by type of lymphoma:

• 10 patients (62.5%) had chronic lymphocytic leukemia/small cell lymphoma (CLL/SLL)—Average age 74. All but one had CLL diagnosed an average of seven years previously. Four cases were bilateral (sequential). Eight patients were treated, usually with chemotherapy with or without external-beam radiation therapy. Six survived and two died of CLL (follow-up <1 year to 14 years)
• Three patients (18.5%) had extranodal marginal zone lymphoma (EMZL)—Average age 76. All had a pre-existing lymphoproliferative disorder (average interval between diagnoses, 19.5 years). Two cases were bilateral. Treatment information was available for two patients; both had sustained local response to chemotherapy
• Three patients (18.5%) had follicular lymphoma—Average age 57. Two cases were primary, and all were unilateral. Initial radiation therapy resulted in local control. One patient died of heart failure; the other two have survived nine and five years, respectively, although one required radiation and intrathecal chemotherapy for recurrent lymphoma

Literature Review

A review of the literature to date (individual case studies excluded) revealed 53 cases of primary lacrimal sac lymphoma and 21 cases of secondary lacrimal sac lymphoma:

• Pediatric (all primary)—Six patients, average age 11 years (range, 9–13 years). All cases were localized to the lacrimal sac, responsive to single-mode or multimodal therapy and were in complete remission at the time of each respective publication
• Adult primary—47 patients with low- to high-grade non-Hodgkin lymphomas. Many case series predated the current lymphoma classification and used older nomenclature, making comparisons with newer studies challenging. Treatment modalities varied, with remission achieved in many cases
• Adult secondary—21 patients, nearly all previously had low-grade B-cell lymphoma, most commonly CLL (81%). Contrary to the Mass Eye and Ear experience, some series reported epiphora was the most common manifestation

Commentary

Determining the histologic subtype of lymphoma and whether it is primary or secondary is important for prognosis and treatment. In atypical cases, it can be challenging to differentiate between CLL, EMZL, and lymphoplasmacytic lymphoma (LPL). Immunohistochemistry, flow cytometry, cytogenetics, and molecular genetics may all be needed.

Routine biopsy during dacryocystorhinostomy remains a matter of debate. Its advocates note that neoplasia can remain subclinical on examination. Many others recommend biopsy only if there is a preoperative concern for malignancy or if the lacrimal sac is atypical intraoperatively. In this Mass Eye and Ear series, all lymphoma cases had worrisome features either pre- or intraoperatively.

For patients with a pre-existing lymphoproliferative disorder who present with dacryocystitis or nasolacrimal duct obstruction, physicians should have a low threshold to biopsy the lacrimal sac.

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