How are orbital and adnexal lymphoma treated?

Updated: Mar 15, 2019
  • Author: Manolette R Roque, MD, MBA, FPAO; Chief Editor: Hampton Roy, Sr, MD  more...
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The recommended therapy for stage IE tumors is radiotherapy, whereas disseminated disease is treated with chemotherapy. [41]

Lymphoid tumors of the conjunctiva have traditionally been treated with local radiation therapy. Some cases of conjunctival lymphoma were seen to spontaneously regress after biopsy; hence, follow-up without radiation has been considered an option for patients with mucosa-associated lymphoid tissue (MALT) lymphoma of conjunctival origin after the pathologic diagnosis by biopsy. [50] Cryotherapy for certain conjunctival lymphomatous tumors has been suggested because of fewer ocular and systemic complications and lower cost. [51]

Radiotherapy alone is a highly effective modality in the curative management of primary orbital lymphoma. [47] Bilateral orbital disease itself, in the absence of systemic disease, is not an indication for chemotherapy.

Approximately 50% of orbital lymphomas are confined to the orbit at the time of diagnosis. In such cases, where there is no evidence of systemic lymphoma, local orbital low-dose (1500-3000 cGy) irradiation in fractionated doses is advised. High-grade tumors may require up to 4000 cGy. Appropriate shielding of the globe (lens-sparing technique) is recommended to minimize ocular complications of radiotherapy. [47]

In cases of massive orbital involvement, both chemotherapy and radiation therapy can be simultaneously given.

Because secondary orbital lymphomas often exhibit widespread systemic involvement and usually have a more aggressive histologic classification than primary orbital lymphomas do, treatment of these lesions with systemic chemotherapy or systemic immunotherapy is warranted. In some cases, combining such systemic therapy with local radiation treatment is beneficial. [52] After radiotherapy, local control was achieved in 97-100% of patients. [47, 53, 54]

Lymphomas respond well to monoclonal antibody (mAb) therapy, and research is ongoing to determine if such therapy can replace chemotherapy. Rituximab, ibritumomab, and epratuzumab are examples of mAbs that either are already in use or are being tested for use in lymphoma treatment. Intravenous rituximab has been used to treat low-grade lymphoma, with good results. [55, 56]

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.

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