Primary adrenal Ewing sarcoma:A systematic review of the literature  

作　　者：Dimitrios K Manatakis Ioannis Tsouknidas Emmanouil Mylonakis Nikolaos Tasis Maria Ioanna Antonopoulou Vasileios Acheimastos Aikaterini Mastoropoulou Dimitrios P Korkolis 

机构地区：[1]2^(nd)Department of Surgery,Athens Naval and Veterans Hospital,Athens 11521,Greece [2]General Surgery,Lankenau Medical Center,Main Line Health,Wynnewood,PA 19096,United States [3]Department of Surgical Oncology,St Savvas Cancer Hospital,Athens 11522,Greece [4]Department of Surgery,Naval Hospital of Crete,Chania 73200,Greece [5]Department of Pediatrics,Stony Brook University Hospital,Stony Brook,NY 11794,United States

出　　处：《World Journal of Clinical Cases》2023年第28期6782-6791,共10页世界临床病例杂志

摘　　要：BACKGROUND Ewing sarcoma(ES)is a malignant neoplasm of neuroectodermal origin and is commonly observed in children and young adults.The musculoskeletal system is the main body system impacted and ES is rarely seen in the visceral organs particularly the adrenal gland.AIM To present a comprehensive review of primary adrenal ES,with emphasis on diagnosis,therapy and oncological outcomes.METHODS A systematic review of the literature was performed according to the Preferred Reporting Items for Systematic reviews and Meta-Analyses 2020.PubMed/MEDLINE,EMBASE and Google Scholar bibliographic databases were searched to identify articles from 1989 to 2022 and included patients with ES/primitive neuroectodermal tumor(PNET)of the adrenal gland.PubMed,Google Scholar and EMBASE medical databases were searched,combining the terms“adrenal”,“ES”and“PNET”.Demographic,clinical,pathological and oncological data of patients were analyzed by SPSS version 29.0.RESULTS A total of 52 studies were included for review(47 case reports and 5 case series)with 66 patients reported to have primary adrenal ES.Mean age at diagnosis was 26.4±15.4 years(37.9%males,57.6%females,sex not reported in 3 cases).The most frequent complaint was abdominal/flank pain or discomfort(46.4%)followed by a palpable mass(25.0%),and the average duration of symptoms was 2.6±3.1 mo.The imaging modality of choice was computed tomography scan(81.5%),followed by magnetic resonance imaging(20.4%).Preoperative staging revealed that 17 tumors(27.9%)were metastatic and 14 patients had inferior vena cava or renal vein neoplastic thrombus at initial diagnosis.Open adrenalectomy was performed in the majority of cases(80.0%),of which 27.9%required more extensive resection.Minimally invasive surgery was attempted in 8.2%of tumors.Complete surgical resection was achieved in 89.4%of the patients.Adjuvant therapy was administered to 32 patients,in the form of chemotherapy(62.5%),radiotherapy(3.1%)or combination(34.4%).Median overall survival was 15 mo and 24-mo overal

关 键 词：Ewing sarcoma Primary adrenal tumor ADRENALECTOMY 

分 类 号：R738.1[医药卫生—肿瘤]