Journal List > J Korean Ophthalmol Soc > v.54(1) > 1009447

Lee, Jung, and Choi: Analysis of Clinical Features and Prognostic Factor Analysis of Orbital and Adnexal Lymphoma

Abstract

Purpose

To study the clinical features, pathophysiologic and radiologic characteristics, and prognostic factors in patients with orbital and adnexal lymphoma.

Methods

The medical records of patients who were diagnosed with orbital and adnexal lymphoma, and patients who were treated and observed, were retrospectively reviewed. Information regarding age, sex, initial symptoms and signs when visiting the hospital, mass location and features, histology and staging, response to treatment and side effects, and prognosis were collected.

Results

Fifty-four patients, with an equal number of men and women, were diagnosed with orbital and adnexal lymphoma. The average age was 50.9 years (14 to 82 years). The main symptom was orbital swelling, and the main site was conjunctiva. Histologically, Mucosa-Associated Lymphoid Tissue (MALT) lymphoma was the most observed. The major ocular complication was dry eye syndrome, which showed good response with ophthalmic treatment. Radiologic features did not have prognostic relevance.

Conclusions

Most orbital and adnexal lymphomas were MALT lymphomas. Proper therapy according to stage can result in a good response and remission. In the present study, the therapy complications were well-controlled with medical treatment, and radiologic features of a mass could not be considered as a prognostic factor.

References

1. Uehara F, Iwakiri N, Ohba N. A twenty-year review of orbital tumor. J Eye. 1995; 12:1177–9.
2. Suzuki K, Ogawa M, Sakurazawa T, et al. A statistical study of clinical and pathological findings in patients with exophthalmos. Folia Ophthalmol Jpn. 1998; 49:364–70.
3. Dunbar SF, Linggood RM, Doppke KP, et al. Conjunctival abdominal: results and treatment with a single anterior electron field. A lens sparing approach. Int J Radiat Oncol Biol Phys. 1990; 19:249–57.
4. Wotherspoon AC, Diss TC, Pan LX, et al. Primary low-grade B-cell lymphoma of the conjunctiva: a mucosa-associated abdominal tissue type lymphoma. Histopathology. 1993; 23:417–24.
5. Cockerham GC, Jakobiec FA. Lymphoproliferative disorders of the ocular adnexa. Int Ophthalmol Clin. 1997; 37:39–59.
crossref
6. Coupland SE, Krause L, Delecluse HJ, et al. Lymphoproliferative lesions of the ocular adnexa. Analysis of 112 cases. Ophthalmology. 1998; 105:1430–41.
7. Johnson TE, Tse DT, Byrne GE Jr, et al. Ocular-adnexal lymphoid tumors: a clinicopathologic and molecular genetic study of 77 patients. Ophthal Plast Reconstr Surg. 1999; 15:171–9.
8. Sullivan TJ, Valenzuela AA. Imaging features of ocular adnexal lymphoproliferative disease. Eye (Lond). 2006; 20:1189–95.
crossref
9. Cho EY, Han JJ, Ree HJ, et al. Clinicopathologic analysis of ocular adnexal lymphomas: extranodal marginal zone b-cell lymphoma constitutes the vast majority of ocular lymphomas among Koreans and affects younger patients. Am J Hematol. 2003; 73:87–96.
crossref
10. Jenkins C, Rose GE, Bunce C, et al. Histological features of ocular adnexal lymphoma (REAL classification) and their association with patient morbidity and survival. Br J Ophthalmol. 2000; 84:907–13.
crossref
11. Ji JY, Ahn YC, Kim YD. Radiotherapy for malignant lymphoma of orbit and ocular adnexa. J Korean Ophthalmol Soc. 2005; 46:201–14.
12. Carbone PP, Kaplan HS, Musshoff K, et al. Report of the Committee on Hodgkins's Disease Staging Classification. Cancer Res. 1971; 31:1860–1.
13. Liesegang TJ. Ocular adnexal lymphoproliferative lesions. Mayo Clin Proc. 1993; 68:1003–10.
crossref
14. Jaffe ES, Harris NL, Diebold J, Muller-Hermelink HK. World Health Organization classification of neoplastic diseases of the hematopoietic and lymphoid tissues. A progress report. Am J Clin Pathol. 1999; 111:S8–12.
15. Jereb B, Lee H, Jakobiec FA, Kutcher J. Radiation therapy of abdominal and orbital lymphoid tumors. Int J Radiat Oncol Biol Phys. 1984; 10:1013–9.
16. Esik O, Ikeda H, Mukai K, Kaneko A. A retrospective analysis of different modalities for treatment of primary orbital non-Hodgkin's lymphomas. Radiother Oncol. 1996; 38:13–8.
17. Jereb B, Lee H, Jakobiec FA, Kutcher J. Radiation therapy of abdominal and orbital lymphoid tumors. Int J Radiat Oncol Biol Phys. 1984; 10:1013–9.
18. Minehan KJ, Martenson JA Jr, Garrity JA, et al. Local control and complications after radiation therapy for primary orbital abdominal: a case for low-dose treatment. Int J Radiat Oncol Biol Phys. 1991; 20:791–6.
19. White WL, Ferry JA, Harris NL, Grove AS Jr. Ocular adnexal lymphoma. A clinicopathologic study with identification of abdominals of mucosa-associated lymphoid tissue type. Ophthalmology. 1995; 102:1994–2006.
20. Demirci H, Shields CL, Karatza EC, Shields JA. Orbital abdominal tumors: analysis of clinical features and systemic abdominal in 160 cases. Ophthalmology. 2008; 115:1626–31.
21. Knowles DM, Jakobiec FA, McNally L, Burke JS. Lymphoid abdominal and malignant lymphoma occurring in the ocular adnexa (orbit, conjunctiva, and eyelids): a prospective multiparametric analysis of 108 cases during 1977 to 1987. Hum Pathol. 1990; 21:959–73.
22. Shields CL, Shields JA, Carvalho C, et al. Conjunctival lymphoid tumors: clinical analysis of 117 cases and relationship to systemic lymphoma. Ophthalmology. 2001; 108:979–84.

Table 1.
Presenting symptoms in patients with orbital lymphoma (multiple)
Symptoms No. of patients (%)
Eyelid swelling 26 (38.2)
Palpable mass 15 (22.1)
Proptosis 10 (14.7)
Diplopia 2 (2.9)
Foreign body sensation 8 (11.8)
Injection 3 (4.4)
Asymptomatic 4 (5.9)
Table 2.
Location in patients with orbital lymphoma (multiple)
Localization No. of patients (%)
Conjunctiva 21 (35)
Lacrimal gland 9 (15)
Lacrimal sac or nasolacrimal duct 3 (5)
Eyelid 7 (11.7)
Other orbital cavity 20 (33.3)
Table 3.
Histopathological classification in patients with orbital lymphoma (WHO classification)
Histology No. of patients (%)
MALT lymphoma 45 (83.3)
Diffuse large B-cell lymphoma 3 (5.6)
Other T-cell lymphoma 3 (5.6)
Mantle cell lymphoma 1 (1.9)
Mixed small and large cell lymphoma 1 (1.9)
Burkitt lymphoma 1 (1.9)
Table 4.
Clinical staging in patients with orbital lymphoma (Ann Arbor classification)
Stage No. of patients (%)
I 23 (60.5)
II 12 (31.6)
III 1 (2.6)
IV 2 (5.3)
Table 5.
Therapy (multiple)
Symptoms No. of patients (%)
Dry eye senation 17 (44.7)
Blepharitis 5 (13.2)
Conjunctivitis 3 (7.9)
Keratitis 1 (2.6)
Ptosis 1 (2.6)
Strabismus 1 (2.6)
Table 6.
Analysis of clinical prognostic factors
Clinifal factor Odds ratio (95% confidence interval) p-value
Laterality at presentation (bilateral vs unilateral) 1.380 (0.342–5.601) 0.7
Mass shape (ill defined vs well defined) 0.229 (0.028–2.045) 0.2
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