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Orbital Involvement in Multiple Myeloma as the First Sign of an Insufficient Chemotherapy

1Müller E., 2Kottler U. B., 1Cursiefen C., 1Holbach L.,
1Friedrich-Alexander-Universität, Augenklinik (Erlangen)
2Johannes-Gutenberg-Universität Mainz, Universitäts-Augenklinik (Erlangen)

Purpose: To present a patient with orbital involvement as first sign of insufficient treatment for multiple myeloma.
Method: Interventional case report with clinicopathologic correlation, radiographic and immunohistochemical features.
Results: A 60-year-old male presented with left proptosis, reduced visual acuity, diplopia and progressing signs of globe indentation two months after completion of systemic chemotherapy for multiple myeloma (type IgG-l-light chain). MRI showed a well-defined tumor with homogeneous contrast enhancement filling the mediobasal part of the left orbit and compressing the eyeball compatible with a manifestation of the preexisting multiple myeloma. No evidence of bone destruction was present in computed tomography. An incisional biopsy and debulking were performed using an anterior transconjunctival orbitotomy. Histopathologically, the biopsy specimen revealed an infiltration by monomorphic lymphoid cells with plasmacellular aspect displaying a low proliferation rate. Immunhistochemically, the tumor cells were positive for plasma cell marker VS38c and negative for B-cell marker CD20; monoclonality was shown for IgG-l-light chains. Systemic examination confirmed the reactivation of the multiple myeloma. Proptosis, intraocular pressure and visual acuity (OS 5/20) improved following external beam radiation therapy of the left orbit and repeated systemic chemotherapy.
Conclusions: Knowledge of the clinical features in primary or recurrent orbital involvement in multiple myeloma is essential for the appropriate management of these patients, since this may be the first sign of insufficient chemotherapy.