原发性鼻腔鼻窦骨外尤文肉瘤/外周原始神经外胚层肿瘤2例并文献复习

Primary extraskeletal Ewing sarcoma/peripheral primitive neuroectodermal tumor in the nasal cavity and paranasal sinus:a case report and literature review

目的分析原发性鼻腔鼻窦骨外尤文肉瘤/外周原始神经外胚层肿瘤的发病特点,为该病的诊疗提供参考。方法回顾性分析2014年3月至2019年7月收治入院的2例鼻腔鼻窦骨外尤文肉瘤/外周原始神经外胚层肿瘤患者的临床资料,并检索2006年至2021年国内外相关文献,共48例(含本文2例)患者纳入研究,总结临床表现、治疗方法及预后。结果本组患者7~83岁、中位数26岁,男女比例1∶1.5,以鼻部症状为主,鼻塞16/28(57.1%)、鼻出血/涕中带血12/28(42.9%)、头痛3/28(10.7%)、嗅觉减退3/28(10.7%)。病变侵犯部位:鼻腔32/48(66.7%)、上颌窦24/48(50.0%)、筛窦27/48(56.3%)、蝶窦8/48(16.7%)、额窦3/48(6.3%),侵袭眼眶17/48(35.4%)、颅底6/48(12.5%)。单因素分析:综合治疗(P=0.009)、放疗(P=0.014)可明显改善患者预后,病变侵犯蝶窦(P=0.027)、颅底(P=0.002)提示患者预后不良,多因素COX回归分析示侵犯颅底为预后的独立危险因素(P=0.008),与未侵犯颅底的患者相比,侵犯颅底的患者死亡风险更高,HR=8.940(95%CI:1.756~45.506)。结论鼻腔鼻窦骨外尤文肉瘤(Extra-skeletal Ewing Sarcoma,EES)好发于青年女性,症状以鼻塞、鼻出血/涕中带血多见,病变范围主要累及鼻腔、上颌窦、筛窦,可侵及眼眶、颅底,病变侵犯颅底为死亡的独立危险因素,治疗以化疗联合放疗的综合治疗为主。

Objective This study aimed to analyze the characteristics of primary extraskeletal Ewing sarcoma(EES)/peripheral primitive neuroectodermal tumor(pPNET)in the nasal cavity and paranasal sinus to provide reference for the diagnosis and treatment of the disease.Methods We conducted a retrospective study on the data of two patients with EES/pPNET in the nasal cavity and paranasal sinuses admitted to our hospital from March 2014 to July 2019.We also performed a literature review in Chinese and English from 2006 to 2021,including the two cases in this study.A total of 48 patients were included,and their clinical manifestations,diagnosis,treatment methods,and prognosis were summarized.Results The median age of the patients was 26 years,age range was 7-83 years,and ratio of men to women was 1.5.The main symptoms of the disease were nasal symptoms,including nasal congestion 16/28(57.1%),epistaxis/nasal discharge stained with blood 12/28(42.9%),headache 3/28(10.7%),and hyposmia 3/28(10.7%).The lesion invasion sites were the nasal cavity 32/48(66.7%),maxillary sinus 24/48(50.0%),ethmoid sinus 27/48(56.3%),sphenoid sinus 8/48(16.7%),frontal sinus 3/48(6.3%),orbital invasion 17/48(35.4%),and skull base 6/48(12.5%).Univariate analysis,such as comprehensive treatment(P=0.009)and radiotherapy(P=0.014),could significantly improve the prognosis of patients.Invasion in the sphenoid sinus(P=0.027)and skull base(P=0.002)showed poor prognosis.Multivariate Cox regression analysis showed that skull-base invasion was an independent risk factor for prognosis(P=0.008).Patients with skull-base invasion had a higher risk of death compared to those without skull-base invasion,HR=8.940(95%CI:1.756-45.506).Conclusions EES in the nasal cavity and paranasal sinus is mostly common in young women.Nasal congestion and epistaxis are common symptoms.It mainly involves the nasal cavity,maxillary sinus,and ethmoid sinus,which also could invade the orbit and skull base.Skull base invasion was an independent risk factor for prognosis.Chemotherapy combined with radiotherapy is the main treatment.