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髓外浆细胞瘤:中国单中心的长期临床结果及文献复习。

Extramedullary Plasmacytoma: Long-Term Clinical Outcomes in a Single-Center in China and Literature Review.

机构信息

Department of Otolaryngology-Head and Neck Surgery, 34732Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing, China.

出版信息

Ear Nose Throat J. 2021 May;100(4):227-232. doi: 10.1177/0145561320950587. Epub 2020 Sep 17.

Abstract

The aim is to analyze the clinical outcomes of patients with extramedullary plasmacytoma (EMP) and review the characteristics of this disease. We retrospectively reviewed the clinical data of a cohort of 22 patients diagnosed with EMP between 1983 and 2017. Baseline characteristics and progression data were collected to calculate the incidence of progression to multiple myeloma (MM) and to analyze survival rates and outcomes. Previous major cohort studies were reviewed and compared with this study. Extramedullary plasmacytomas were located in the head and neck in 17 (77%) of the 22 patients. The median time between onset and diagnosis was 7 months, and the median age at diagnosis was 52.5 years (range 15-72 years). Extramedullary plasmacytoma occurred more frequently in men with an approximate ratio of 2.1:1. The majority of patients underwent surgery (13/22, 59.1%) and half received radiation (11/22, 50%). A small proportion of patients received chemotherapy (2/22, 9.1%). There was a median follow-up time of 98.5 months. The 5-year overall survival, progression-free survival, and MM-free survival rates were 84.2%, 67.3% and 75.9%, respectively. Complete resection without major functional damage is a promising option that can favorably improve prognosis in patients with resectable disease. Patients with regional lymph node metastasis or positive immunoelectrophoresis results require more aggressive treatment and may have poorer prognosis.

摘要

目的是分析髓外浆细胞瘤(EMP)患者的临床结局,并回顾该疾病的特征。我们回顾性分析了 1983 年至 2017 年间诊断为 EMP 的 22 例患者的临床资料。收集基线特征和进展数据,以计算进展为多发性骨髓瘤(MM)的发生率,并分析生存率和结局。回顾了以前的主要队列研究,并与本研究进行了比较。22 例患者中,17 例(77%)的 EMP 位于头颈部。发病至诊断的中位时间为 7 个月,诊断时的中位年龄为 52.5 岁(15-72 岁)。EMP 更常发生于男性,男女比例约为 2.1:1。大多数患者接受了手术(13/22,59.1%),一半患者接受了放疗(11/22,50%)。少数患者接受了化疗(2/22,9.1%)。中位随访时间为 98.5 个月。5 年总生存率、无进展生存率和无 MM 生存率分别为 84.2%、67.3%和 75.9%。无主要功能损害的完全切除是一种很有前途的选择,可以改善可切除疾病患者的预后。有区域淋巴结转移或免疫电泳结果阳性的患者需要更积极的治疗,可能预后较差。

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