Clinical findings in 25 patients with sinonasal or nasopharyngeal extramedullary plasmacytoma in a four-decade single-centre series

Abstract

Objectives: Extramedullary plasmacytoma in the sinonasal tract or nasopharynx is rare. The aim of the study was to review data on symptoms, clinical findings, treatment and follow-up of plasmacytomas in the sinonasal and nasopharyngeal regions in order to delineate the main clinical characteristics and the optimal management.

Method: Twenty-five patients with sinonasal or nasopharyngeal plasmacytoma, diagnosed and treated at the Helsinki University Hospital during a 39-year period from 1975 to 2013 were retrospectively reviewed.

Results: There were 18 males and 7 females with a median age of 66 years (range, 36–80). Sixty-eight percent received only radiotherapy or (chemo)radiotherapy. Forty-seven percent of them had a complete response to primary radiotherapy and one patient had a complete response after receiving additional brachytherapy. Four patients were treated primarily with surgery only. Two of them had a local recurrence, but were then successfully treated with radiotherapy. Altogether, four patients received a combination of surgery and (chemo)radiotherapy. Forty-four percent were alive with no evidence of disease after a median follow-up time of 78 months. Forty percent died of their disease and 16% died of other causes.

Conclusions: Our study supports radiotherapy as a treatment of choice, but for small tumours surgery alone or in combination with radiotherapy may also be considered.

Chinese abstract

目的：鼻窦或鼻咽的髓外浆细胞瘤是罕见的。本研究的目的是审查鼻窦和鼻咽区血浆细胞瘤的症状、临床表现、治疗和随访情况, 以描写其主要临床特征和最佳治疗方案。

方法：回顾性重审了1975年至2013年39年间赫尔辛基大学医院诊断和治疗的25例鼻窦或鼻咽浆细胞瘤患者。

结果：这些患者中男性18例, 女性7例, 平均年龄为66岁（范围36-80岁）。百分之八十八只接受放射治疗或（化疗）放疗。 47%的患者对原发性放射疗法有完整的反应, 一名患者在接受额外的近距离放射治疗后有完整的反应。 4例患者主要仅用手术治疗。其中两例局部复发, 随后成功接受了放疗。总共有4名患者接受了手术和（化疗）放疗的联合治疗。中位随访时间78个月后, 百分之四十四的人活着, 没有显示任何疾病证据。百分之四十死于疾病, 百分之十六死于其它原因。

结论：我们的研究支持放疗作为可选治疗方法。但对于小肿瘤, 单独使用手术或与放射治疗组合也可考虑。

Keywords:

• Extramedullary plasmacytoma
• sinonasal
• nasopharynx
• multiple myeloma
• radiotherapy

Disclosure statement

No conflict of interest to declare.

Additional information

Funding

This study was funded by the Helsinki University Hospital Research Fund (Grant number of TYH2015204) and the Viipuri Tuberculosis Foundation.