Lymphoproliferative lesions of the ocular adnexa: Analysis of 112 cases

doi:10.1016/S0161-6420(98)98024-1

Ophthalmology

Volume 105, Issue 8, 1 August 1998, Pages 1430-1441

Presented in part at the Combined Meeting of the International Society for Ocular Pathology and American Association of Ocular Pathology, Chicago, Illinois, October 1996.

https://doi.org/10.1016/S0161-6420(98)98024-1 Get rights and content

Abstract

Objective

Lymphoproliferative lesions of the ocular adnexa were analyzed to examine (1) the suitability of the Revised European-American Lymphoma (REAL) classification for the subtyping of the lymphomas in these sites; (2) the predictive value of the REAL classification for the evolution of these tumors; and (3) the frequency and prognostic impact of tumor type, location, proliferation rate (Ki-67 index), p53, and CD5 positivity and the presence of monoclonality within these tumors.

Design

Retrospective review.

Methods

The clinical, histomorphologic, immunohistochemical, and molecular biologic (polymerase chain reaction [PCR]) features of lymphoid proliferations of the ocular adnexa were studied.

Study materials

The ocular adnexal lymphoproliferative lesions were located as follows: orbit in 52 patients (46%), conjunctiva in 32 patients (29%), eyelid in 23 patients (21%), and caruncle in 5 patients (4%).

Results

Reactive lymphoid hyperplasia was diagnosed in 12 cases and lymphoma in 99 cases; 1 case remained indeterminate. The five main subtypes of lymphoma according to the REAL classification were extranodal marginal-zone B-cell lymphoma (64%), follicle center lymphoma (10%), diffuse large cell B-cell lymphoma (9%), plasmacytoma (6%), and lymphoplasmocytic lymphoma (5%). Age, gender, and anatomic localization of the lymphomas did not have prognostic significance during a follow-up period of 6 months to 16.5 years (mean, 3.3 years). Extent of disease at time of presentation was the most important clinical prognostic factor: advanced disease correlated with increased risk ratios of having persistent disease at the final follow-up and with lymphoma-related death (P <0.001). Histomorphologic features and immunohistochemical markers positively correlating with disseminated disease at presentation, stage at final follow-up, and occurrence of lymphoma-related death included cytologic atypia (P <0.001), MIB-1 proliferation rate (P <0.001), and tumor cell p53 positivity (P <0.001). The MIB-1 proliferation rates greater than 20% in extranodal marginal-zone B-cell lymphoma corresponded to at least stage II lymphoma (P <0.05).

Conclusion

The REAL classification is suitable for the subdivision of the ocular adnexal lymphomas. The MIB-1 proliferation rate and p53 positivity may aid the prediction of disease stage and disease progression, whereas PCR can support the diagnosis and reduce the number of histologically indeterminate lesions.

Section snippets

Tissue samples

One hundred twelve cases of lymphoid proliferation of the ocular adnexa in 112 patients were collected from the consultation files of the Pathology Departments of Universitätsklinikum Benjamin Franklin, Berlin, Germany, and of Western Infirmary, Glasgow, Scotland. Patients with inflammatory pseudotumor or lymphoid proliferations of the choroid, vitreous, or retina were excluded. The majority of the tissue biopsy specimens had been fixed in 10% formaldehyde solution and embedded in paraffin;

Clinical features and anatomic distribution

The patient group consisted of 79 females and 32 males in total with an age range of 14 to 91 years; median, 61 years (Fig 1). There was no significant age difference between the patients diagnosed with RLH and those with lymphoma (median age, 59 and 61 years, respectively) (Fig 1). None of the patients were suffering from Sjögren’s syndrome, Wegener’s granulomatosis, or Mikulicz syndrome.

The ocular adnexal lymphoid proliferations were distributed as follows: orbit in 52 (46%) with involvement

Discussion

Benign and malignant lymphoid proliferations of the ocular adnexa cannot be diagnosed accurately on the basis of clinical or radiologic criteria3, 22, 34, 35: the distinction often requires extensive histomorphologic, immunophenotypical, and molecular biologic analyses. In the current study, the majority of the lymphoproliferative lesions of the ocular adnexa could be divided into two groups—RLH or lymphoma—using conventional histology and immunohistochemistry. A gray zone of so-called

Acknowledgements

The authors thank the following pathologists and clinicians for their cooperation as well as their provision of access to patient records: Dr. D. Bauer-Matyas, Dr. N. Bechrakis, Prof. Bleckmann, Prof. S. Blumcke, Dr. F. Dallenbach, Dr. W. Due, Prof. M. H. Foerster, Prof. P. Grote, Prof. C. Hartmann, Priv. Doz., Dr. Hamisch, Dr S. Klebe, Prof. J. Kunz, Priv. Doz. G. Jautzke, Prof. L. D. Leder, Priv. Doz., U. Pleyer, and Dr. A. Walkow.

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