Clinical features and prognosis of multiple myeloma and orbital extramedullary disease: Seven cases report and review of literature

Abstract

BACKGROUND 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. CASE SUMMARY 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 . CONCLUSION 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.

Orbital involvement in long-standing multiple myeloma

“…Due to the immunocompromised status of these patients, there are chances of acquiring lung infection and systemic inflammatory response syndrome and resulting multiple intensive care unit admissions, as in our patient, due to which the histopathology got delayed and globe salvage could not be done. Survival rates in patients with extramedullary plasmacytomas are already low regardless of the tissue involved 15 16. Therefore, they need early palliative radiation therapy to prevent disease progression which is usually very rapid and can lead to irreversible vision loss as has happened in our patient.…”