eMedicine Specialties > Ophthalmology > Orbit

Ocular Lymphoma: Follow-up

Author: Smita Menon-Mehta, MBBS, DOMS, DO, DNB, FRCS(Glasg), Consulting Staff, Department of Ophthalmology, Bahrain Specialist Hospital
Contributor Information and Disclosures

Updated: Jun 25, 2008

Follow-up

Further Outpatient Care

Despite usually demonstrating an indolent course, extranodal marginal zone B-cell lymphomas are renowned for recurrence in extranodal sites, including other ocular adnexal sites.38 These sites can also include the lung, parotid gland, and bone marrow.51  
 
Close clinical follow-up every 6 months for 2 years upon completion of treatment and annually thereafter is recommended. The evaluation should include neuroimaging studies (eg, ultrasound, CT scan, or MRI of the orbits) to look for residual or recurrent local disease. Serial imaging can be a very useful tool to detect recurrence. Residual fibrosis of involved extraocular muscles or other orbital structures can appear similar to tumor. Re-biopsy or exploration should be a clinical decision often aided by serial imaging.
 
A clinical oncologist should also follow up with the patient for a thorough systemic evaluation every 6 months for 2 years and annually thereafter.

Complications

Complications of radiotherapy include cataract, dry eye, corneal ulcer, neovascular glaucoma, radiation retinopathy, and optic neuropathy.47,51

Prognosis

Even with chemoradiation, the prognosis remains poor for patients with PCNSLO, and many succumb to CNS disease within 2 years. Yet, median survival of PCNSL has increased from 1-1.5 years to over 3 years with newer therapies. Features affecting the prognosis of PCNSLO are not well understood.52 Death ensues by CNS dissemination. Ocular lymphoma may be the initial manifestation of PCNSL.
 
Age, sex, and anatomical localization of the lymphomas did not have prognostic significance during a follow-up period of 6 months to 16.5 years.40 The major prognostic criteria for ocular adnexal lymphomas include anatomical location of the tumor, stage of disease at first presentation, subtype of lymphoma, immunohistochemical markers determining factors such as tumor growth rate, and the serum lactate dehydrogenase level.38 The extent of disease at the 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 lymphoma-related death.40  
 
The longest survival has been seen in patients with low-grade lymphomas (ie, marginal zone lymphoma, follicular lymphoma).53 However, T-cell lymphomas are associated with high mortality with conventional treatment, as there is a high incidence of systemic involvement.54
 
The overall prognosis for ocular adnexal lymphoid tumors is excellent; when lumped together, 67% are not found to be associated with systemic disease with a mean follow-up of over 4 years. Lesions of the conjunctiva fare the best; those of the orbit have an intermediate prognosis; and lid lesions are known to have the worst prognosis.55  
 
Over the course of follow-up, it could be anticipated that 20-25% of patients not known to have systemic lymphoma develop evidence of disseminated disease within 5 years.55  
 
With radiotherapy for orbital disease, the 5-year disease-free survival and overall survival rate has been between 65-73.6% and 65.5-78%.47,49  
 
Most relapses occur in the first 2 years after therapy. The risk of late relapse is higher in patients with a divergent histology of both indolent disease and aggressive disease.

Miscellaneous

Special Concerns

Research is ongoing to determine if lower doses of local radiation are effective and still reduce associated complications.
 
Lymphomas respond well to monoclonal antibody therapy, and research is ongoing to determine if this therapy can replace chemotherapy. Rituximab, Zevalin, and epratuzumab are some such drugs either already in use or being tested for use in lymphoma treatment. 
 
Antiangiogenic drugs, such as thalidomide, are also being researched for use in lymphoma treatment, as they are shown to slow the growth of cancer cells.

 


More on Ocular Lymphoma

Overview: Ocular Lymphoma
Differential Diagnoses & Workup: Ocular Lymphoma
Treatment & Medication: Ocular Lymphoma
Follow-up: Ocular Lymphoma
Multimedia: Ocular Lymphoma
References
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Further Reading

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Keywords

Hodgkin lymphoma, ocular disease, non-Hodgkin lymphoma, NHL, vision problems, uveitis, malignant orbital tumor, ocular cancer, eye cancer, lymphoproliferative disease, proptosis, conjunctival tumor, orbital lymphoma, adnexal lymphoma, intraocular lymphoma, HIV infection, AIDS, human immunodeficiency virus, acquired immunodeficiency syndrome, primary CNS lymphoma, PCNSL, primary CNS lymphoma with ocular involvement, PCNSLO, MALT lymphoma, diffuse large cell lymphoma, small lymphocytic lymphoma, intraocular lymphoma

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Contributor Information and Disclosures

Author

Smita Menon-Mehta, MBBS, DOMS, DO, DNB, FRCS(Glasg), Consulting Staff, Department of Ophthalmology, Bahrain Specialist Hospital
Smita Menon-Mehta, MBBS, DOMS, DO, DNB, FRCS(Glasg) is a member of the following medical societies: All India Ophthalmological Society
Disclosure: Nothing to disclose.

Medical Editor

Brian A Phillpotts, MD, Former Vitreo-Retinal Service Director, Former Program Director, Clinical Assistant Professor, Department of Ophthalmology, Howard University College of Medicine
Brian A Phillpotts, MD is a member of the following medical societies: American Academy of Ophthalmology, American Diabetes Association, American Medical Association, and National Medical Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Simon K Law, MD, PharmD, Assistant Professor of Ophthalmology, Jules Stein Eye Institute; Chief of Section of Ophthalmology Surgical Services, Department of Veterans Affairs Healthcare Center, West Los Angeles
Simon K Law, MD, PharmD is a member of the following medical societies: American Academy of Ophthalmology, American Glaucoma Society, and Association for Research in Vision and Ophthalmology
Disclosure: Nothing to disclose.

Managing Editor

Mark T Duffy, MD, PhD, Consulting Staff, Division of Oculoplastic, Orbito-facial, Lacrimal and Reconstructive Surgery, Green Bay Eye Clinic, BayCare Clinic; Medical Director, Advanced Cosmetic Solutions, A BayCare Clinic
Mark T Duffy, MD, PhD is a member of the following medical societies: American Academy of Ophthalmology, American Medical Association, American Society of Ophthalmic Plastic and Reconstructive Surgery, Sigma Xi, and Society for Neuroscience
Disclosure: Allergan - Botox Cosmetic Consulting fee Consulting; Quest medical - lacrimal balloons Honoraria Speaking and teaching; Ortho-Neutrogenia Consulting fee Consulting

CME Editor

Lance L Brown, OD, MD, Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri
Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences
Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology
Disclosure: Nothing to disclose.

 
 
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