Wang Samuel S Y, Lee Mitchell B, George Adarsh, Wang Sarah B, Blackwell Jonathan, Moran Steve, Francis Ian C
a Faculty of Medicine , University of New South Wales , Sydney, Australia.
b Sydney Medical School , University of Sydney , Sydney, Australia.
Orbit. 2019 Jun;38(3):218-225. doi: 10.1080/01676830.2018.1490437. Epub 2018 Jul 9.
: Multiple myeloma is an insidious haematological malignancy characterised by monoclonal proliferation of plasma cells in the bone marrow. Extramedullary plasmacytoma is a rare manifestation of multiple myeloma and usually occurs in the upper respiratory tract. Orbital involvement is particularly uncommon, but may be associated with devastating visual impairment and poor clinical outcomes. Therefore, this article aims to highlight the need for multidisciplinary management of orbital extramedullary plasmacytoma. : This is a retrospective observational case series of five patients. All presented to the authors for management of orbital extramedullary plasmacytomas from 2004 to 2015 at Prince of Wales and Mater Hospitals in Sydney, Australia. Medical records were reviewed for pertinent information including demographics, disease features, management strategy, and clinical progress. The study met Medical Ethics Board standards and is in accordance with the Helsinki Agreements. : This case series of five patients underscores the poor prognosis of orbital extramedullary plasmacytoma. Despite aggressive multidisciplinary management, four of these five patients succumbed to their illness during the study period. However, multidisciplinary management did manage to minimise symptoms and preserve quality of life. : On a case-by-case basis, patients may derive palliative benefit from orbital surgery in conjunction with radiotherapy and chemotherapy. Orbital surgeons are encouraged to work within a multidisciplinary framework of medical specialists, including haematologists and radiation oncologists, when determining the optimal management plan in cases of orbital extramedullary plasmacytoma.
多发性骨髓瘤是一种隐匿性血液系统恶性肿瘤，其特征为骨髓中浆细胞的单克隆增殖。髓外浆细胞瘤是多发性骨髓瘤的一种罕见表现形式，通常发生在上呼吸道。眼眶受累尤为罕见，但可能导致严重的视力损害和不良的临床结局。因此，本文旨在强调眼眶髓外浆细胞瘤多学科管理的必要性。
这是一项对5例患者的回顾性观察病例系列研究。所有患者于2004年至2015年期间在澳大利亚悉尼威尔士亲王医院和圣母医院就诊，接受眼眶髓外浆细胞瘤的治疗。对病历进行了回顾，以获取包括人口统计学、疾病特征、治疗策略和临床进展等相关信息。该研究符合医学伦理委员会的标准，并遵循赫尔辛基协议。
这个包含5例患者的病例系列研究强调了眼眶髓外浆细胞瘤的预后不良。尽管采取了积极的多学科管理措施，但在研究期间，这5例患者中有4例因病死亡。然而，多学科管理确实成功地减轻了症状并维持了生活质量。
在个案基础上，患者可能从眼眶手术联合放疗和化疗中获得姑息治疗益处。在确定眼眶髓外浆细胞瘤病例的最佳治疗方案时，鼓励眼眶外科医生在包括血液科医生和放射肿瘤学家在内的医学专家多学科框架内开展工作。
