Published online Nov 26, 2022. doi: 10.12998/wjcc.v10.i33.12365
Peer-review started: August 10, 2022
First decision: September 5, 2022
Revised: September 26, 2022
Accepted: October 24, 2022
Article in press: October 24, 2022
Published online: November 26, 2022
Multiple myeloma (MM) complicated with extramedullary disease (EMD) has a poor prognosis and is a limiting factor in the treatment of MM, and no standard treatment is recommended in international guidelines. Few studies have reported MM with periorbital EMD.
In this paper, the clinical characteristics and survival of seven patients with multiple myeloma and orbital are described and analyzed. The common ocular symptoms were blurred vision, proptosis and/or eye movement disorders, IgG type MM may be a risk factor for orbital involvement. Of them, six patients were treated with bortezomib-based regimens. The median overall survival (OS) and progression free survival for the entire cohort were 48 and 33 mo, respectively, which was much worse than the OS reported for MM patients without orbital EMD.
Orbital MM may have significantly shortened survival for the entire cohort, so multidisciplinary collaboration is emphasized and recommended in the diagnosis and treatment of these difficult cases.
Core Tip: Orbital extramedullary disease (EMD) complicated with Multiple myeloma (MM) is rare. Herein, the clinical characteristics of seven patients from December 2007 to November 2019 were analyzed. IgG type may be a risk factor for orbital EMD. Unilateral disease is more common. General ocular symptoms are blurred vision, proptosis or eye movement disorders. In addition to a biopsy of the lesion, positron emission tomography/computed tomography (CT) or magnetic resonance imaging is more sensitive and useful than CT for the diagnosis and evaluation. Current standard treatment approaches and survival have not improved, therefore, multidisciplinary management of individualized targeted chemotherapy basis is recommended for this aggressive myeloma sub-entity.