儿童淋巴上皮瘤样癌病例特点及预后分析*

Characteristics and prognosis of lymphoepithelioma-like carcinoma in children

    摘要
  • 摘要:
    目的 探讨儿童淋巴上皮瘤样癌（lymphoepithelioma-like carcinoma，LELC）的发病特点、临床特征、治疗及预后情况。
    方法 回顾性分析2008年3月至2023年6月在中山大学肿瘤防治中心诊治的年龄<18岁LELC患儿的临床资料。
    结果 共19例儿童及青少年患儿纳入分析，其中男性10例（52.6%），女性9例（47.4%），中位年龄12.9（4.3～17.0）岁，14例患儿居住地为广东省（73.7%），其余散发分布。原发部位为纵隔11例（57.9%），腮腺4例（21%），颈部1例（5.3%），肺部1例（5.3%），涎腺1例（5.3%），颌下腺1例（5.3%）；其中15例（78.9%）在初诊时就已经出现至少1个部位远处转移，常见转移部位为颈部淋巴结。多因素Cox分析显示肿瘤体积≥801 cm3为总生存（overall survival，OS）较差的独立不良预后因素（P<0.01）。2年OS和PFS（progression-free survival，PFS）率分别为84.2%和57.9%。完成手术+化疗+放疗的患儿和仅完成部分治疗的患儿的2年OS率分别为100%和25%（P=0.007）。初诊时联用程序性死亡因子-1（programmed death-1，PD-1）抗体和不联用PD-1抗体的患儿的2年PFS率分别为100%和38.5%（P=0.020）。肿瘤体积≥801 cm3和肿瘤体积<801 cm3患儿的2年OS率分别为40.0%和100%（P<0.001）。放疗和未放疗患儿的2年OS率分别为100%和0（P<0.001）。
    结论 儿童LELC采用手术、化疗、放疗等多学科综合治疗预后相对较好，初诊时联用PD-1抗体有潜在获益，值得进一步探讨。

     

    Abstract:
    Objective  To explore the pathogenesis, clinical features, treatment strategies, and prognosis of pediatric lymphoepithelioma-like carcinoma (LELC).
    Methods A retrospective analysis was conducted on the clinical data of patients with LELC aged <18 years, treated at Sun Yat-sen University Cancer Center from March 2008 to June 2023.
    Results A total of 19 children and adolescents were included in the analysis, comprising 10 males (52.6%) and 9 females (47.4%), with a median age of 12.9 (4.3-17.0) years. Fourteen patients (73.7%) lived in Guangdong province, with the remainder scattered across other regions. Primary LELC sites were the mediastinum (11 cases, 57.9%), parotid glands (4 cases, 21%), neck (1 case, 5.3%), lungs (1 case, 5.3%), salivary glands (1 case, 5.3%), and submandibular glands (1 case, 5.3%). Among these, 15 patients (78.9%) had at least one distant metastasis at initial diagnosis, with common metastasis sites being cervical lymph nodes. Multivariate Cox regression analysis identified tumor volume ≥801 cm3 as an independent adverse prognostic factor of poor overall survival (OS) (P<0.01). The 2-year OS and progression-free survival (PFS) rates were 84.2% and 57.9%, respectively. The 2-year OS for patients who underwent surgery, chemotherapy, and radiotherapy was 100%, compared with 25% for those who received only partial treatment (P=0.007). The 2-year PFS rate was significantly higher in patients receiving first-line combination therapy with programmed death-1 (PD-1) antibodies (100%) compared with those not treated with PD-1 antibodies (38.5%) (P=0.020). For patients with tumor volume ≥801 cm3, the 2-year OS was 40.0%, whereas for those with a tumor volume <801 cm3, the 2-year OS was 100% (P<0.001). The 2-year OS for patients who underwent radiotherapy was 100%, while it was 0 for those who did not receive radiotherapy (P<0.001).
    Conclusions Pediatric LELC exhibits a relatively favorable prognosis with multidisciplinary treatment, including surgery, chemotherapy, and radiotherapy. The combined use of PD-1 antibodies at the time of initial diagnosis could offer potential benefits and warrants further exploration.

     

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