J Korean Ophthalmol Soc.  2018 Aug;59(8):779-784. 10.3341/jkos.2018.59.8.779.

Primary Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue of the Lacrimal Sac Mimicking Dacryocystitis

• Kwon JM
• Lee DH
• Ahn JH

• KMID: 2418506
• DOI: http://doi.org/10.3341/jkos.2018.59.8.779

Abstract

PURPOSE
We report a case of a young female patient who was diagnosed with a marginal zone B-cell lymphoma of the mucosa-associated lymphoid tissue (MALT lymphoma) of the lacrimal sac which mimicked dacrocystitis.
CASE SUMMARY
A 23-year-old female suffered from epiphora for 3 years in the right eye. She had swelling and a painful lesion at the lacrimal sac 4 months prior and was referred to our hospital due to nasolacrimal duct obstruction and dacryocystitis. Lacrimal irrigation was performed with no passing and regurgitation with mucoid discharge. We performed orbital computed tomography and magnetic resonance imaging which showed a suspected tumor of the lacrimal sac in the right eye. We then performed excision and biopsy of the tumor through the skin approach. The patient was diagnosed with a MALT lymphoma with no systemic involvement. After six cycles of chemotherapy involving rituximab with cyclophosphamide, vincristine, and prednisone, the lesion of the mass and the painful symptoms decreased, which was regarded as a complete response. However, epiphora and eye discharge persisted, showing a nasolacrimal duct obstruction, so we performed endoscopic dacryocystorhinostomy with a lacrimal sac biopsy. The histological examination showed chronic inflammation, but not lymphoma. There was no recurrence of lymphoma at one year postoperatively.
CONCLUSIONS
If there is an epiphora and palpable lesion in the lacrimal sac area, MALT lymphoma mimicking dacyocystitis may be suspected. After the lymphoma is first diagnosed by excision and biopsy, systemic chemotherapy with dacryocystorhinostomy could be an effective treatment.

Keyword

Chemotherapy; Dacryocystitis; Lacrimal sac; Mucosa-associated lymphoid tissue (MALT) lymphoma

MeSH Terms

Biopsy
Cyclophosphamide
Dacryocystitis*
Dacryocystorhinostomy
Drug Therapy
Female
Humans
Inflammation
Lacrimal Apparatus Diseases
Lymphoid Tissue*
Lymphoma
Lymphoma, B-Cell, Marginal Zone*
Magnetic Resonance Imaging
Nasolacrimal Duct*
Orbit
Prednisone
Recurrence
Rituximab
Skin
Vincristine
Young Adult
Cyclophosphamide
Prednisone
Rituximab
Vincristine

Figure

• 

  Figure 1 External photograph of 23-year-old female patient. The patient had epiphora and mild swelling at right lacrimal sac area.

• 

  Figure 2 Orbit computed tomography scan. (A) Non-enhance axial computed tomography images show ill-defined hyperdense mass (black arrow) at right lacrimal sac. (B) Expansion of nasolacrimal duct (white arrow).

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  Figure 3 Orbit magnetic resonance imaging. (A) Axial T2 weighted image shows homogeneous relatively hyper-intensity in right lacrimal sac (white arrowhead). (B) Contrast-enhanced axial T1 weighted image demonstrates iso signal intensity tubular structure with rim enhancement in the right lacrimal sac (white arrow). (C) Axial T2 weighted image after systemic chemotheraphy showed no mass leision in lacrimal sac. (D) Contrast-enhanced axial T1 weighted image after systemic chemotheraphy showed no enhancing mass leision.

• 

  Figure 4 Histopathologic findings of the surgical specimen. (A) Dense, monotonous neoplastic lymphoid cells surround the lacrimal sac (hematoxylin & eosin stain [H&E], ×40). (B) Lymphoepithelial lesions are identified (H&E ×400). (C) CD20 staining positivity demonstrates extensive B cell infiltration (CD20, ×40). (D) Lymphoid cells showing cystoplasmic kappa light chain positivity (kappa in situ hybridization, ×40). (E) A few lymphoid cells showing cystoplasmic lambda light chain positivity (lambda in situ hybridization, ×40). Kappa light chain predominates over lambda chain, demonstrating monoclonality.

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