Case Report
Copyright ©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Nov 26, 2022; 10(33): 12365-12374
Published online Nov 26, 2022. doi: 10.12998/wjcc.v10.i33.12365
Clinical features and prognosis of multiple myeloma and orbital extramedullary disease: Seven cases report and review of literature
Wan-Li Hu, Jia-Yin Song, Xin Li, Xiao-Jiao Pei, Jia-Jia Zhang, Man Shen, Ran Tang, Zhen-Yu Pan, Zhong-Xia Huang
Wan-Li Hu, Jia-Yin Song, Xin Li, Jia-Jia Zhang, Man Shen, Ran Tang, Zhong-Xia Huang, Department of Hematology, Multiple Myeloma Medical Center of Beijing, Beijing Chao-Yang Hospital, Capital Medical University, Beijing 100043, China
Xiao-Jiao Pei, Zhen-Yu Pan, Department of Radiology, Beijing Chao-Yang Hospital, Capital Medical University, Beijing 100043, China
Author contributions: Huang ZX was designed, conducted and manuscript writing; The data were collected primarily by Hu WL and Song JY, assisted by Li X, Pei XJ, Zhang JJ, Shen M, Tang R and Pan ZY; Statistical analysis and manuscript writing were partly completed by Hu WL and Song JY; All authors agreed to publish the final manuscript in this journal. Hu WL and Song JY contributed equally to this article.
Supported by Science and Technology Project of Beijing Science and Technology Commission, No. Z171100000417010; Construction Project on Key Medical Disciplines of Shijingshan District Health Committee of Beijing Municipality.
Informed consent statement: Informed consent from patients was obtained to publish this paper.
Conflict-of-interest statement: All the authors have stated that they have no conflicts of interest.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to it.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Zhong-Xia Huang, MD, Chief Physician, Director, Professor, Department of Hematology, Multiple Myeloma Medical Center of Beijing, Beijing Chao-Yang Hospital, Capital Medical University, No. 5 Jingyuan Road, Shijingshan District, Beijing 100043, China. huangzhongxia@sina.com
Received: August 10, 2022
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
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.

Keywords: Multiple myeloma, Extramedullary disease, Periorbital, Treatment, Survival, Case report

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.