Clinical Analysis of Primary Conjunctival Malignant Lymphoma

Jee Myung Yang; Je Jung Lee; Kyung Chul Yoon

doi:10.3341/jkos.2014.55.9.1298

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Yang, Lee, and Yoon: Clinical Analysis of Primary Conjunctival Malignant Lymphoma

Original Article

J Korean Ophthalmol Soc 2014;55(9):1298-1306.

Published online: 2 September 2014

DOI: https://doi.org/10.3341/jkos.2014.55.9.1298

Clinical Analysis of Primary Conjunctival Malignant Lymphoma

Jee Myung Yang, MD¹, Je Jung Lee, MD, PhD², Kyung Chul Yoon, MD, PhD^1,

¹Department of Ophthalmology, Chonnam National University Hospital, Chonnam National University Medical School, Gwangju, Korea

²Department of Hemato-Oncology, Chonnam National University Hospital, Chonnam National University Medical School, Gwangju, Korea

Address reprint requests to Kyung Chul Yoon, MD, PhD, Department of Ophthalmology, Chonnam National University Hospital, #42 Jebong-ro, Dong-gu, Gwangju 501-757, Korea, Tel: 82-62-220-6741, Fax: 82-62-227-1642, E-mail: kcyoon@jnu.ac.kr

Received 27 January 2014 Revised 29 April 2014 Accepted 20 August 2014

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Purpose

To investigate clinical outcomes, response to treatment, and the related factors of recurrence and complication, following treatment of primary conjunctival malignant lymphoma.

Methods

The medical records of 39 patients diagnosed with primary conjunctival malignant lymphoma between January 2005 and June 2013 were retrospectively reviewed.

Results

The mean age of patients was 51.1 years old. The most common presenting symptom was hyperemia (33.3%). The most common anatomical location of the mass was the fornix (38.5%) and 25.6% patients had bilateral involvement. Histopathologically, mucosa-associated lymphoid tissue (MALT) lymphoma (92.3%) was the most common subtype. Every patient underwent radiotherapy (92.3%) or chemotherapy (7.7%) after surgical excision and had 100% complete remission. Local or systemic recurrence was observed in 15.4% of patients after treatment (mean 8.0 ± 3.3 months), but was completely remitted after additional radiation or chemotherapy. International prognostic index and location of tumor were significantly related factors for predicting tumor recurrence (p < 0.01, p = 0.02, respectively). Dry eye disease (DED) was the most common ocular complication (44.4%) after radiotherapy. Total radiation dosage and location of tumor were significantly associated factors for developing DED after radiotherapy (both p = 0.04).

Conclusions

Most conjunctival malignant lymphomas were low grade malignant MALT lymphomas that responded well to treatment but recurrence of tumor and development of DED after radiotherapy were common, therefore predicting and preparing these lymphomas is important.

Keywords: Conjunctival lymphoma, Dry eye disease, Malignant lymphoma, Radiotherapy, Recurrence

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Figure 1.

The representive case of the eye with extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma). (A) Slit-lamp exmanination reveals a ‘salmon-colored’ conjunctival mass in caruncle. (B) Histological examination demonstrates the diffuse proliferation of lymphoid cells in the conjunctiva (Hematoxylin-eosin stain, ×100). Immunohistochemical staining shows positivity of the tumor cells for (C) CD20 (×400) which is a marker for B-cells and (D) CD21 (×100) which reflects follicular dendritic cell meshwork (×100).

Figure 2.

The representive case of the eye with follicular lymphoma. (A) Clinical photograph of the follicular lymphoma presenting classical ‘salmon-colored’ conjunctival mass located in the fornix. Immunohistochemical stain of (B) CD10 and (C) Bcl-6 which are markers of follicular center B-cell differentiation (× 200). (D) Bcl-2 expression by the tumor cells (× 100).

Table 1.

Anatomic distribution of primary conjunctival malignant lymphoma

Conjunctival location	No. of patients (%)
Fornix	15 (38.5)
Bulbar	12 (30.8)
Limbus	5 (12.8)
Caruncle/plica semilunaris	4 (10.3)
Tarsus	3 (7.7)

Table 2.

Histopathologic diagnosis of primary conjunctival malignant lymphoma according to WHO classification

Type	No. of patients (%)
MALT lymphoma	36 (92.3)
Follcular lymphoma	2 (5.1)
Small lymphocytic lymphoma	1 (2.6)
Total	39 (100.0)

MALT lymphoma = extranodal marginal-zone B-cell lymphoma of mucosa-associated lymphoid tissue.

Table 3.

Analysis of predisposing factors of recurrence in patients with primary conjunctival malignant lymphoma

Parameters	OR	95% CI	p-value
Age	0.98	0.93-1.04	0.50
Sex
Male	1
Female	1.50	0.25-8.98	0.66
Bilaterality	1.20	0.19-7.44	0.85
Location
Limbal	1
Extralimbal	11.25	1.42-89.26	0.02
Stage
Stage I	1
Stage II-IV	1.43	0.27-7.57	0.67
IPI
0-1	1
≥2	17.50	2.50-122.50	<0.01

OR = odd ratio; CI = confidence interval; IPI = international prognostic index.

Table 4.

Comparison between patients with dry eye disease and patients without dry eye disease after radiotherapy

	Non-DED (n = 20)	DED (n = 16)	p-value
Age (years)	49.55 ± 15.79	53.19 ± 16.14	0.54^*
Sex (male/female)	8/12	5/11	0.59^†
Total radiation dose (Gy)	30.24 ± 2.59	32.24 ± 2.12	0.03^*
BCVA (log MAR)	0.02 ± 0.05	0.02 ± 0.05	0.54^*
Tear parameters
TBUT (sec)	10.10 ± 1.48	4.31 ± 1.01	<0.01^*
Basal tear secretion (mm)	12.25 ± 1.86	5.94 ± 1.29	<0.01^*
Tear clearance rate ([log₂]^-1)	5.40 ± 0.60	2.94 ± 0.68	<0.01^*
Keratoepitheliopathy score	0.25 ± 0.44	2.19 ± 1.11	<0.01^*

Values are presented as mean ± SD unless otherwise indicated.

DED = dry eye disease; BCVA = best-corrected visual acuity; log MAR = logarithm of the minimal angle of resolution; TBUT = tear film break-up time.

^* Comparison by Mann-Whitney U-test;

^† Comparison by chi-square test.

Table 5.

Analysis of risk factors for developing dry eye disease after radiotherapy in patients with primary conjunctival malignant lymphoma

Parameters	OR	95% CI	p-value
Age	1.02	0.97-1.06	0.49
Sex
Male	1
Female	1.47	0.37-5.86	0.59
Radiation dose (Gy)
<30	1
≥30	10.00	1.09-91.44	0.04
Location
Inferior	1
Superior	4.50	1.06-19.04	0.04
Cornea-lens shielding
Yes	1
No	1.89	0.36-10.03	0.46

OR = odd ratio; CI = confidence interval.

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