Journal of Otolaryngology and Ophthalmology of Shandong University ›› 2022, Vol. 36 ›› Issue (3): 290-296.doi: 10.6040/j.issn.1673-3770.0.2022.009

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

CHEN Jiahong, WANG Lin, JIANG Yan, YU Longgang, ZHANG Jisheng, ZHANG Zhiyong, KONG Jingwen   

  1. Department of Otorhinolaryngology & Head and Neck Surgery;
    Department of Skull Base Surgery, Affiliated Hospital of Qingdao University/Otorhinolaryngology Head and Neck Surgery, Key Laboratory of Shandong Province, Qingdao 266003, Shandong, China
  • Published:2022-06-15

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Abstract: 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.

Key words: Ewing's sarcoma, Primitive neuroectodermal tumor, Small round cell tumor, Cavidade nasal, Sinuses

CLC Number: 

  • R739.62
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