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Show !ournal of CliniCilI Neuro-ophthalmology 8(3): 209-210, 1988. Letter to the Editor Lymphoproliferative Disorders © 1988 Raven Press, Ltd., New York To the Editor: I was most interested in the last issue of the Journal of Clinical Neuro-ophthalmology because of the presentation on lymphoproliferative disorders. In view of this, I would like to call your attention to a paper which Dr. Michael Bray and I wrote entitled, "Evolution of a primary lymphoma of the orbit" (1). I thought that this case report would be interesting to your readership and serve to make two important points. The patient reported in the above paper was a 67-year-old woman who was referred for evaluation on January 6, 1976, with a lO-month history of pain and proptosis of the right eye. Her symptoms had been relieved by corticosteroid therapy ordered by another ophthalmologist but recurred on withdrawal of the medication. Examination showed the right globe to be displaced upward by a mass on the floor of the orbit. The eye was painful to palpation and movement. Visual acuity was 20/40 bilaterally. Hertel exophthalmometry noted the right eye to be 22 mm and the left 20.5 mm, measured at an interorbital distance of 95 mm. Ultrasonography revealed an anechoic area in Tenon's space, contiguous with the optic nerve, and mottling of the retrobulbar fat. This presented as a partial "T" sign and was interpreted as an inflammatory process as described by Coleman and Restori in their papers on ultrasonography (2,3). A CT scan with the second generation EMI scanner noted a poorly defined mass within the retrobulbar muscle cone and thickening of the lateral sclerouveal rim. This finding was felt to be consistent with orbital inflammation as described by Bernardino et al. and Alper (4,5). Laboratory studies were normal including thyroid function studies, serum immunoglobulin levels, and collagen vascular disease evaluation. Steroid therapy was continued but was complicated because of development of upper gastrointestinal bleeding. It was decided to first obtain a tissue diagnosis and then institute radiation therapy. A Kronlein operation was performed 2 years after the first onset of symptoms. At surgery, an ectopic lacrimal gland was found in the inferotem- 209 poral portion of the outer surgical space and an adjacent inflammatory mass was present within the muscle cone, infiltrating Tenon's space. Microscopic examination noted chronic dacryoadenitis and there was extensive infiltration within the lacrimal gland, consistent with benign pseudotumor. The specimen taken from Tenon's space furthermore demonstrated a sheet of lymphocytes which were interpreted as benign lymphoproJiferative disease. After the operation, the patient received 1000 rads of radiation therapy to the right orbit with resolution of all symptoms, except for some dryness of the eye. Four years later the mass recurred and at this time an area of swelling in the soft palate was also noted. The right eye was more displaced upwardS and a mass was palpable in the right lower lid anterior to the orbital septum. Orbital exploration was once again performed, after appropriate radiologic studies. The pathologic examination at this time revealed a well-differentiated, lymphocytic lymphoma. Biopsy of the soft palate also exhibited a lymphocytic lymphona. Immunohistologic studies were then performed and demonstrated the presence of an IgM lambda monoclonal lymphoma. The peripheral blood noted IgM lambda monoclonal paraproteinemia in the patient's serum on immunoserology evaluation. No coagulopathy or other complications of macroglobulinanemia were observed. Bone scan, liver-spleen scan, and other studies were normal. This time, the patient received 3400 rads to the right orbit and palate with resolution of all symptoms. In 1987, she died of generalized lymphoma. The original specimen was studied at a later date with immunoperoxidase and was categorized as polyclonal in character. As noted in the opening paragraph, this patient demonstrated two important features: (a) A benign lymphoproliferative disease can ultimately change from a benign polyclonal to a malignant monoclonal process. (b) Although Drs. Davis and associates and I reported enhancement of sclerouveal rim as a "sine qua non" sign of inflammation, 210 LETTER TO THE EDITOR it can also occur in lymphomatous processes. This may very well be true for the "T" sign as described by Coleman. The fact that benign lymphoproliferative tumors can change to become malignant implies that careful follow-up of these patients is indicated. Enhancement of the sclerouveal rim and/or a "T" sign upon B-ultrasonography of the orbit may represent a lymphoma rather than inflammation. It then becomes important to make an accurate tissue diagnosis. Melvin G. Alper, M.D. 5454 Wisconsin Ave. Chevy Chase, Maryland 20815 J Gin Neuro-ophthalmol, Vol. 8. No.3, 1988 REFERENCES 1. Alper MG, Bray M. Evolution of a primary lymphoma of the orbit. Br JOphthalnIOI1984;68:255-60. 2. Coleman OJ. Reliability of ocular and orbital diagnosis with B-scan ultrasound. Am JOphthalmoI1972;74:704-18. 3. Restori M. Ultrasound in orbital diagnosis. Trans Ophthalmol Soc UK 1979;99:223-5. 4. Bernadino M, Zimmerman R, Citrin CM, David DO. Uveal scleral enhancement seen in pseudotumor. Am J Radial 1977;129:703-6. 5. Alper MG. Computerized tomography (CT) in diagnosis of inflammatory orbital pseudotumor and Graves' disease. In: Thompson HS, ed. Topics of neuro-ophthalmology. Williams and Wilkins, Baltimore: 1979:347-68. 6. Brubaker DO, Whiteside TL. Differentiation between benign and malignant human lymph nodes by means of immunologic markers. Cancer 1979;43:1165-76. |
