Orbital lymphoma associated with Graves’ disease: A case report
Abstract
Introduction. The presence of bilateral exophthalmos and palpebral, periorbital edema associated with hyperthyroidism is most often considered as an initial sign of Graves’ ophthalmopathy. However, in up to 20% of cases, Graves’ ophthalmopathy might precede the occurence of hyperthyroidism, which is very important to be considered in the differential diagnosis, especially if it is stated as unilateral. Among other less common causes of non-thyroid-related orbitopathy, orbital lymphoma represents rare conditions. We presented of a patient with Graves’ disease, initially manifested as bilateral orbitopathy and progressive unilateral exophthalmos caused by the marginal zone B-cell non-Hodgkin lymphoma of the orbit. Case report. A 64-year-old man with the 3-year history of bilateral Graves’ orbitopathy and hyperthyroidism underwent the left orbital decompression surgery due to the predominantly left, unilateral worsening of exophthalmos resistant to the previously applied glucocorticoid therapy. A year after the surgical treatment, a substantial exophthalmos of the left eye was again observed, signifying that other non-thyroid pathology could be involved. Orbital ultrasound was suggestive of primary orbital lymphoma, what was confirmed by orbital CT scan and the biopsy of the tumor tissue. Detailed examinations indicated that the marginal zone B-cell non-Hodgkin lymphoma extended to IV – B-b CS, IPI 3 (bone marrow infiltration: m+ orbit+). Upon the completion of the polychemiotherapy and the radiation treatment, a complete remission of the disease was achieved. Conclusion. Even when elements clearly indicate the presence of thyroid-related ophthalmopathy, disease deteriorating should raise a suspicion and always lead to imaging procedures to exclude malignancy.
References
Lacey B, Chang W, Rootman J. Nonthyroid causes of extraocular muscle disease. Surv Ophthalmol 1999; 44(3): 187−213.
Boyce PJ. Orbital lymphoma masquerading as thyroid ophthal-mopathy. J Am Optom Assoc 1998; 69(10): 666−73.
Abdullah A, Elsamaloty H, Patel Y, Chang J. CT and MRI find-ings with histopathologic correlation of a unique bilateral or-bital mantle cell lymphoma in Graves' disease: a case report and brief review of literature. J Neurooncol 2010; 97(2): 279−84.
Bahn RS, Dutton CM, Natt N, Joba W, Spitzweg C, Heufelder AE. Thyrotropin receptor expression in Graves' orbital adi-pose/connective tissues: potential autoantigen in Graves' oph-thalmopathy. J Clin Endocrinol Metab 1998; 83(3): 998−1002.
Nutting CM, Shah-Desai S, Rose GE, Norton AP, Plowman PN. Thyroid orbitopathy possibly predisposes to late-onset of pe-riocular lymphoma. Eye 2006; 20(6): 645−8.
Bartalena L, Brogioni S, Valeriano R, Nardi M, Cartei F, Bogazzi F, et al. Non-autoimmune hyperthyroidism associated with iso-lated bilateral ocular lymphomamimicking Graves' disease with ophthalmopathy: a cause of misdiagnosis. J Endocrinol Invest 1995; 18(10): 817−9.
Buescu A, Teixeira P, Coelho S, Donangelo I, Vaisman M. Orbital lymphoma misdiagnosed as Graves' ophthalmopathy. Endocir Pract 2001; 7(2): 110-−2.
Payne JF, Shields CL, Eagle RC, Shields JA. Orbital lymphoma simulating thyroid orbitopathy. Ophthal Plast Reconstr Surg 2006; 22(4): 302−4.
Woolf DK, Ahmed M, Plowman PN. Primary Lymphoma of the Ocular Adnexa (Orbital Lymphoma) and Primary Intraocular Lymphoma. Clin Oncology 2012; 24(5): 339−44.
Stark JJ, Newsom RW, Roman J, Larocque JC, Richardson DW. Or-bital MALT lymphoma in a patient with Graves' ophthalmopathy: a unique observation. Cancer Invest 2005; 23(7): 593−5.