Orbital Service, Moorfields Eye Hospital, London, United Kingdon.
Ophthalmic Plast Reconstr Surg. 2022;38(2):180-184. doi: 10.1097/IOP.0000000000002023.
To describe patients with periocular solitary extramedullary plasmacytoma (SEMP) and multiple myeloma (MM), together with an estimate of the risk of progression from SEMP to MM.
A retrospective case-note review for patients seen between 1978 and 2020, examining demographics, presentation, imaging, pathology, management, and outcome.
Twenty patients (10 male; 50%) presented at a mean age of 60.9 years, with an average symptom duration of 4.5 months. Ten (50%) patients had known systemic myeloma at ophthalmic presentation (the MM group) and, on average, they presented one decade earlier than those with occult MM discovered after orbital biopsy (p = 0.06); the majority (9/15; 60%) of patients with MM were female, whereas there was a male bias (4/5; 80%) with SEMP (p = 0.30). Most tumors (15/20; 75%) were within the anterior part of the orbit, especially superolaterally (16 patients; 80%), and the soft-tissue mass often appeared to "explode" from the frontal bone or greater wing of the sphenoid (16/20; 80%). Full treatment details were known for 19 patients: 6 (32%) had solely orbital radiotherapy, 4 (21%) chemotherapy, 6 (32%) combined chemoradiation, and 3 (16%) had combined chemoradiation with stem-cell transplant (Table 3). After an average follow up of 58 months, 1/5 (20%) patients with SEMP and 11/15 (73%) with MM had tumor-related death. The overall survival probability for all 20 patients with periocular plasmacytoma was 34% at 5 and 10 years, with MM patients having a worse outlook (27% 5-year, and 18% 10-year survival) compared with SEMP (53% survival at 5 and 10 years) (p = 0.18). None of the 5 patients with SEMP progressed to systemic MM over an average follow up of 9.1 years.
Although 50% patients with periocular plasmacytoma appear to have a SEMP at ophthalmic presentation, a half of these patients were found to have occult MM within 6 months of biopsy. Of those without systemic disease around the time of biopsy, none developed MM over an average follow up of more than 9 years.
描述发生眼眶髓外孤立性浆细胞瘤（SEMP）和多发性骨髓瘤（MM）的患者，并评估从 SEMP 进展为 MM 的风险。
对 1978 年至 2020 年间就诊的患者进行回顾性病历审查，检查患者的人口统计学、表现、影像学、病理学、治疗和结果。
20 名患者（10 名男性；50%）的平均年龄为 60.9 岁，平均症状持续时间为 4.5 个月。10 名（50%）患者在眼部表现时患有已知的系统性骨髓瘤（MM 组），他们的平均发病年龄比眼眶活检后发现隐匿性 MM 的患者早十年（p=0.06）；大多数（9/15；60%）患有 MM 的患者为女性，而 SEMP 患者则存在男性偏倚（4/5；80%）（p=0.30）。大多数肿瘤（15/20；75%）位于眼眶前部，尤其是外上方（16 名患者；80%），软组织肿块常从额骨或蝶骨大翼“爆炸”而出（20 名患者中的 16 名；80%）。19 名患者的治疗细节均已知：6 名（32%）仅接受眼眶放疗，4 名（21%）接受化疗，6 名（32%）接受放化疗联合治疗，3 名（16%）接受放化疗联合干细胞移植（表 3）。在平均 58 个月的随访后，5 名 SEMP 患者中有 1 名（20%）和 15 名 MM 患者中有 11 名（73%）因肿瘤相关死亡。20 名眼眶浆细胞瘤患者的总体生存率为 5 年和 10 年分别为 34%，MM 患者的预后较差（5 年生存率为 27%，10 年生存率为 18%），与 SEMP 相比（5 年和 10 年的生存率分别为 53%）（p=0.18）。在平均 9.1 年的随访中，没有 5 名 SEMP 患者进展为系统性 MM。
尽管 50%的眼眶浆细胞瘤患者在眼部表现为 SEMP，但其中一半患者在活检后 6 个月内被发现患有隐匿性 MM。在活检时无系统性疾病的患者中，在平均随访超过 9 年后，无一人发生 MM。
