Lee Weiyong, Chen Robert Chun, Swaminathan Saravana Kumar, Ho Chi Long
Department of Radiology, Sengkang General Hospital, Singapore.
Department of Diagnostic Radiology, Singapore General Hospital, Singapore.
J Radiol Case Rep. 2019 Dec 31;13(12):1-12. doi: 10.3941/jrcr.v13i12.3784. eCollection 2019 Dec.
Multiple myeloma is a hematologic malignancy due to monoclonal plasma cell proliferation. It is usually confined to the bone marrow, although extramedullary involvement is known to occur in almost any organ system; myelomatous spread to the central nervous system is a rare manifestation of myeloma. Extramedullary disease is thought to be related to hematogenous spread when myeloma cells show decreased cell surface receptor expression, allowing cells to escape from the bone marrow. Disease outside of the bone marrow generally indicates a poor prognosis; central nervous system involvement is associated with a median prognosis of less than 6 months, thereby requiring more aggressive treatment paradigms. We herein describe an unusual case of a patient with extramedullary multiple myeloma with central nervous system and multiorgan involvement. Despite an aggressive treatment strategy, the patient died a few months later after the initial diagnosis. The etiology, diagnostic criteria, clinical presentation, radiological features and differential diagnosis of this patient with extramedullary multiple myeloma are discussed here. The current treatment strategies are also briefly discussed.
多发性骨髓瘤是一种由于单克隆浆细胞增殖引起的血液系统恶性肿瘤。它通常局限于骨髓,尽管已知几乎在任何器官系统中都可发生髓外受累;骨髓瘤扩散至中枢神经系统是骨髓瘤的一种罕见表现。当骨髓瘤细胞表面受体表达降低,使细胞能够从骨髓中逸出时,髓外疾病被认为与血行播散有关。骨髓外疾病通常提示预后不良;中枢神经系统受累的患者中位预后小于6个月,因此需要更积极的治疗方案。我们在此描述了一例罕见的髓外多发性骨髓瘤患者,该患者同时累及中枢神经系统和多个器官。尽管采取了积极的治疗策略,但患者在初次诊断后数月死亡。本文讨论了该髓外多发性骨髓瘤患者的病因、诊断标准、临床表现、影像学特征及鉴别诊断。同时也简要讨论了当前的治疗策略。