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Working closely with New York Eye and Ear Infirmary of Mount Sinai, Continuum Cancer Centers of New York (CCCNY) has become a national leader in treatment of orbital (eye) lymphoma (OL).
Non-Hodgkin’s lymphoma (NHL) rarely occurs in the orbit, only 1% of all NHL. If present only in the eye, OL is treated with radiotherapy alone with excellent success. In 10%-15% of cases, OL involves both eyes. It commonly appears as a slowly growing orbital mass that causes no symptoms, or, depending on where it occurs, proptosis (a bulging eyeball), ocular dysmotility (reduced eye movement, often causing double vision), periorbital swelling, blurring of vision and chemosis (swelling of the surface of the eye).Because it is rare and requires balancing the needs to kill the cancer but save the eye orbit, radiation treatment is technically difficult.
Because OL is so rare, published studies often group all types of lymphomas together, making it hard to know how it applies to a particular patient. Most orbital lymphomas are mucosa-associated lymphoid tissue (MALT) tumors, other histologic subtypes occur, such as follicular, diffuse large B-cell (DLBCL), and mantle cell lymphomas. Although many studies have found that modest radiation doses alone have produced excellent local control and long-term survival for MALT OLs, others found that they later spread more often than MALT tumors starting at other sites.
Orbital lymphomas usually grow slowly and stay at the original site for a long time, making it hard to compare the results of different treatments. However, radiotherapy has been generally considered the most effective treatment for localized tumors.
We recently looked at the long-term outcomes and patterns of failure of 79 patients (28 male and 51 female) with Stage IE OL treated with curative radiation from 1995 through 2012. The median age was 59years (range: 21-89). Most were the two most common histologic subtypes, MALT and follicular lymphomas. The cancer involved conjunctivas in 23 (29%), orbit in 37 (47%), and lacrimal glands in 19 (24%) patients. Tumors were on the left in 42 patients , on the right in 31, and bilateral tumors in six patients.
The median radiation dose was 30.6 Gy. No tumors recurred locally. Five recurred outside of the treatment fields, one in the other orbit, two in the general head and neck area and two metastasized elsewhere. The long-term relapse-free survival and overall survival for all OL patients were both approximately 98% (see Figures 1 & 2). Not all patients received chemotherapy to achieve these high cure rates of over 95%.
This long-term study showed that definitive radiotherapy to a dose of 30Gy was highly effective and safe for indolent OL, based on cancer control and overall survival. This experience at CCCNY is one of the largest sets of patients ever studied. As radiotherapy remains the treatment mainstay of localized orbital lymphoma, we plan to measure and reduce treatment side effects, including conjunctivitis, skin erythema, keratitis (scaling), palpebral edema (eyelid swelling), later cataracts, and retinopathy(damage to the retina) as we use complex, modern radiation therapy to maintain these high cure rates.
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