Rosai–Dorfman disease presenting as bilateral lacrimal gland enlargement

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Text of Rosai–Dorfman disease presenting as bilateral lacrimal gland enlargement

  • compression of the trochlear nerve may produce sponta-neous discharges in trochlear axons in the same way that itdoes in the facial nerve in patients with hemifacialspasms.5 Neuroimaging of such a lesion in a case of superioroblique myokymia has not been reported previously be-cause it is nearly impossible to see the trochlear nerve inconventional MRI.

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    1. Hoyt WF, Keane JR. Superior oblique myokymia: report anddiscussion on five case of benign intermittent uniocularmicrotremor. Arch Ophthalmol 1970;84:461467.

    2. Leigh RJ, Tomsak RL, Seidman SH, DellOsso LF. Superioroblique myokymia. Quantitative characteristics of the eyemovements in three patients. Arch Ophthalmol 1991;109:17101713.

    3. Geis TC, Newman NJ, Dawson RC. Superior obliquemyokymia associated with a dural arteriovenous fistula.J Neuro-Ophthalmol 1994;112:10631067.

    4. Hashimoto M, Ohtsuka K, Akiba H, Harada K. Vascularcompression of the oculomotor nerve disclosed by thin-slicemagnetic resonance imaging. Am J Ophthalmol 1998;125:881882.

    5. Jannetta PJ. Observations on the etiology of trigeminal neu-ralgia, hemifacial spasms, acoustic nerve dysfunction andglossopharyngeal neuralgia: definitive microsurgical treatmentand results in 117 patients. Neurochirgica 1977;145154.

    RosaiDorfman Disease Presenting asBilateral Lacrimal Gland EnlargementMatthew Lee-Wing, MD, FRCSC,Allan Oryschak, MD, FRCPC,Gurcharan Attariwala, MD, FRCSC, andMichael Ashenhurst, MD, FRCSC

    PURPOSE: To report a patient with bilateral lacrimal glandenlargement as the initial manifestation of RosaiDorf-man disease.METHODS: Case report.RESULTS: A 14-year-old female presented with left lacri-mal gland enlargement followed by right lacrimal glandenlargement 11 weeks later. Bilateral lacrimal glandbiopsies were performed, and histopathologic examina-tion revealed the diagnosis of RosaiDorfman disease.CONCLUSION: Patients with RosaiDorfman disease maypresent with bilateral lacrimal gland swelling in theabsence of lymphadenopathy. RosaiDorfman diseaseshould be considered in the differential diagnosis ofbilateral lacrimal gland enlargement. (Am J Ophthal-

    mol 2001;131:677678. 2001 by Elsevier ScienceInc. All rights reserved.)

    ROSAIDORFMAN DISEASE IS A BENIGN, IDIOPATHIC DIS-order that typically affects children and young adults,producing massive bilateral cervical lymphadenopathy,fever, leukocytosis, an increased erythrocyte sedimentationrate, and hypergammaglobulinemia.1,2 Ophthalmologicmanifestations most frequently occur in the orbit and areseen in 11% of patients with RosaiDorfman disease.3 Insome patients, the ophthalmologic findings constitute theinitial or principal feature of the disease.3 We report apatient with bilateral lacrimal gland enlargement as theinitial presentation of RosaiDorfman disease.

    A 14-year-old East Indian female presented with aswollen left orbit of several months duration. She deniedfever, diplopia, or orbital pain. Her medical history wasunremarkable. On examination, a firm mass was palpablein the superotemporal left orbit. The remainder of theocular examination was normal, and no lymphadenopathywas found. A computed tomographic (CT) scan of theorbits demonstrated an enlarged left lacrimal gland. Sys-temic investigations for immunologic abnormalities, thewhite blood cell count, and an erythrocyte sedimentationrate were normal. At operation, a multilobulated lesioninvolving the lacrimal gland was excised and examinedhistopathologically.

    Eleven weeks after surgery, the patient developed aswollen right orbit. The examination revealed a firm massin the right lacrimal gland region. The remainder of theocular and systemic examinations was again normal withno lymphadenopathy. An excisional biopsy of the rightlacrimal gland lesion was performed. The postoperativecourse of the patient was uneventful. However, 9 monthslater, she developed fever with massive bilateral swelling ofthe salivary glands and cervical lymphadenopathy. A

    Accepted for publication Oct 17, 2000.From the Departments of Ophthalmology (M.L.-W., G.A., M.A.) and

    Pathology (A.O.), University of Calgary, Calgary, Alberta, Canada, andthe Department of Ophthalmology, University of Ottawa, Ottawa,Ontario, Canada (M.L.-W.).

    Inquiries to A. Oryschak, MD, Calgary Laboratory Services, Rocky-view Hospital, 7007 - 14th St SW, Calgary, Alberta, Canada T2V 1P9;fax: (403) 541-3333; e-mail: allan.oryschak@CLS.ab.ca

    FIGURE 1. The normal lacrimal gland microarchitecture iseffaced with a mixed inflammatory infiltrate, including lympho-cytes, histiocytes, and plasma cells (hematoxylin and eosin,original magnification 3 13.2).

    BRIEF REPORTSVOL. 131, NO. 5 677

  • repeat erythrocyte sedimentation rate was abnormal, andthe white blood cell count was increased with a lympho-cytic response. The salivary gland enlargement and lymph-adenopathy spontaneously subsided, and the patient hasremained asymptomatic for a follow-up period of 14 years.

    Histopathologic examination of the left lacrimal glandspecimen demonstrated a mixed inflammatory infiltrate oflymphocytes, histiocytes, and plasma cells (Figure 1). Onhigh-power microscopic examination, some of the histio-cytic cells were found to contain intracytoplasmic lympho-cytic nuclei (Figure 2). Similar histopathologic findingswere found in the right lacrimal gland. A diagnosis ofRosaiDorfman disease was made, based on the histologicfindings. The biopsy specimens were reviewed by Dr. Dorf-man, who confirmed the diagnosis of RosaiDorfman disease.

    An atypical presentation of RosaiDorfman diseaseaffecting the lacrimal glands bilaterally was demonstratedin our patient as the initial manifestation. The CT scanand intraoperative and histopathologic findings estab-lished the diagnosis of RosaiDorfman disease confined tothe lacrimal glands. In a previous report,3 lacrimal glandinfiltration secondary to orbital mass formation was foundin 2 of 11 patients with orbital RosaiDorfman disease;however, no patient demonstrated RosaiDorfman diseaseisolated to the lacrimal glands.3 Another atypical featurein our patient was the delayed appearance of fever andlymphadenopathy. The lacrimal gland disease in our pa-tient preceded fever and lymphadenopathy by approxi-mately 1 year. Although most patients with RosaiDorfman disease have massive lymph nodes, some patientswith orbital involvement may not develop significantlymphadenopathy.4 We conclude that RosaiDorfman dis-ease should be considered in the differential diagnosis ofbilateral lacrimal gland enlargement regardless of theabsence of lymphadenopathy.

    REFERENCES

    1. Rosai J, Dorfman RF. Sinus histiocytosis with massive lymph-adenopathy. A newly recognized benign clinicopathologicalentity. Arch Pathol 1969;87:6370.

    2. Rosai J, Dorfman RF. Sinus histiocytosis with massive lymph-adenopathy: a pseudolymphomatous benign disorder. Analysisof 34 cases. Cancer 1972;30:11741188.

    3. Foucar E, Rosai J, Dorfman RF. The ophthalmologic manifes-tations of sinus histiocytosis with massive lymphadenopathy.Am J Ophthalmol 1979;87:354367.

    4. Friendly DS, Font RL, Rao NA. Orbital involvement insinus histiocytosis. A report of four cases. Arch Ophthalmol1977;95:20062011.

    FIGURE 2. High-power view of the infiltrate, illustratinglymphocytic character of the infiltrate as well as histiocyteswith engulfed lymphocyte nucleus (arrow) seen in the center ofthe field (hematoxylin and eosin, original magnification 3 132).

    AMERICAN JOURNAL OF OPHTHALMOLOGY678 MAY 2001