January 2018 Case of the Month - Further Information

Submitted by Dr Yamini Krishna, Prof Sarah Coupland 

Clinical History:  An 89 year-old male presenting with a painful, cystic swelling in the right lacrimal sac area.  Treated with antibiotics.  Lacrimal sac was noted to be inflamed during right external dacryocystorhinostomy.  Incisional biopsy of lacrimal sac wall taken - ?inflammation, need to exclude malignancy.  Past medical history of CLL.

Macroscopic Description:  A piece of pale tissue measuring 8x5x3mm.

Microscopic Description:  Sections show connective tissue densely infiltrated by an atypical lymphocytic proliferation comprising intermediate to large cells with prominent nucleoli.  No epithelial elements are identified.  The atypical cells are strongly CD20+ and CD79a+; also CD5+, CD23+ (weak) and BCL-2+.  There is moderate scattering of CD3+ cells.  The cells are negative for: cyclin-D1, CD21, CD10 and BCL-6.  Ki67 immunostaining shows a moderate growth fraction with mitotic cells localised in proliferation centres.  


Discussion: Lymphomatous involvement of lacrimal sac may be primary or secondary.  Previous reporting would have been variable due to lack of global consensus regarding lymphoma classifications until the Revised European-American Classification of Lymphoid Neoplasms, the WHO classification, and later the EORTC ophthalmic oncology task force study defining the clinical and histopathological characteristics.  


Lymphomas account for ~2-6% of lacrimal sac malignant tumours, present in generally older patients with symptoms typical of secondary acquired nasolacrimal obstruction including epiphora, medial canthal mass; thus often misdiagnosed as acute or chronic dacryocystitis.  It should be suspected in patients with known CLL presenting with epiphora and dacryocystitis.  Dacryocystorhinostomy (DCR) with stenting is well-tolerated and effective at alleviating symptoms.  During DCR an incisional biopsy of the lacrimal sac is essential for confirming CLL/SLL involvement, even in the absence of obvious swelling, and may guide adjuvant therapy.  Treatment is usually a combination of surgery, irradiation and /or chemotherapy (notably regimens involving chlorambucil) but no commonly agreed treatment regimen for periocular lymphoma exists because of the limited number of patients seen.


For further information please see:


Benger RS, Frueh BR. Lacrimal drainage obstruction from lacrimal sac infiltration by lymphocytic neoplasia. Am J Ophthalmol 1986;101:242-5.

Auw-Haedrich C, Coupland SE, Kapp A, Schmitt-Graff A, Buchen R, Witschel H.Long term outcome of ocular adnexal lymphoma subtyped according to the REAL classification. Br J Ophthalmol 2001;85:63-9.

Yip CC, Bartley GB, Habermann TM, Garrity JA.  Involvement of the lacrimal drainage system by leukemia or lymphoma. Ophthal Plast Reconstr Surg 2002;18:242-6.

 Sjö L, Ralfkiaer E, Juhl BR, Prayse JU, Kivela T, Auw-Haedrich C, Bacin F, Carrera M, Coupland SE, Delbosc B, DucreyN, Kantelip B, Kemeny JL, Meyer P, Sjo NC, Heegaard S. Primary lymphoma of the lacrimal sac: an EORTC ophthalmic oncology task force study.  Br J Ophthalmol 2006;90:1004-9.

Litschel R, Siano M, Nasolacrimal duct obstruction caused by lymphoproliferative infiltration in the course of chronic lymphocytic leukemia. Allergy Rhinol Providence 2015;6:191-4.

 Krishna Y, Irion LD, Karim S, Dharmsena A, McCormick A, Coupland SE. Chronic Lymphocytic Leukaemia/Small-Cell Lymphocytic Lymphoma of the Lacrimal Sac: A Case Series. Ocul Oncol Pathol 2017;3:224-8.

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