- Department of Neurosurgery, Max Superspeciality Hospital, Saket, New Delhi, India
Department of Neurosurgery, Max Superspeciality Hospital, Saket, New Delhi, India
DOI:10.4103/sni.sni_270_18Copyright: © 2018 Surgical Neurology International This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.
How to cite this article: Ashwin U. Borkar, Kapil Jain, Vijendra K. Jain. A large primary orbital lymphoma with proptosis: A case report and review. 04-Dec-2018;9:249
How to cite this URL: Ashwin U. Borkar, Kapil Jain, Vijendra K. Jain. A large primary orbital lymphoma with proptosis: A case report and review. 04-Dec-2018;9:249. Available from: http://surgicalneurologyint.com/surgicalint-articles/9111/
Background:Primary orbital lymphomas are a rare subset of tumors constituting 1–2% of non-Hodgkin's lymphoma. They are mostly indolent B-cell lymphomas presenting with gradual progressive proptosis, decreased visual acuity, restricted ocular mobility, and diplopia. The role of surgery is mainly for obtaining a biopsy. Most of these tumors require multimodality treatment including chemotherapy, radiation, or both, which have major role.
Case Description:We report one such case of marginal zone lymphoma of the orbit in a female with significant proptosis who was treated with multimodality treatment, including surgical excision as a major treatment modality. Decompression of symptomatic proptosis was followed by chemotherapy and radiation.
Conclusion:Primary orbital lymphoma is a rare clinical entity with diverse clinical outcomes. It can be successfully managed with surgical excision for decompression of mechanical proptosis followed by chemotherapy, radiation, or both.
Keywords: Marginal zone lymphoma, orbital lymphoma, proptosis
Primary orbital lymphomas are a rare subset of non-Hodgkin's lymphomas. They are mostly indolent, slow-growing B cell lymphomas, usually presenting with progressive proptosis. The role of surgery is mainly for obtaining a biopsy, and the management of such tumours involves multimodality treatment. Here, we report the case of a marginal zone lymphoma of the orbit that was treated with surgical excision as a major treatment modality. Decompression of symptomatic proptosis was followed by chemotherapy and radiation.
A 47-year-old lady presented with a history of gradual progressive bulging of the right eye for 5 years. The proptosis was significant and restricting the right eye movement laterally [
Orbital lymphoma is reported as the most common malignant tumor of ocular adnexa constituting 55% of all orbital tumors.[
Cases reported tumors with high vascularity where either wedge biopsy or total tumor clearance was done.[
Multimodality treatment including surgery, chemotherapy, and radiation or a combination have been advocated in the management of orbital lymphomas. Staging of tumor is an important guide to selection of an appropriate treatment strategy. Surgery alone; however, is not recommended due to the high rate of local relapse. Surgery is usually utilized as a diagnostic tool. Complete remission without significant complications has been reported with radiation alone with a median dose of 3,060 cGy (centigrays).[
To conclude, primary orbital lymphoma is a rare clinical entity with diverse clinical course. It can be successfully treated with multimodality treatment after appropriate staging.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patients have given consent for their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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