Primary adrenal Ewing sarcoma:A systematic review of the literature

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 overall survival was 40.5%.Median disease-free survival was 10 mo and 24-mo disease-free survival was 33.3%.CONCLUSION The significant progress in molecular biology and genetics of ES does not reflect on patient outcomes.ES remains an aggressive tumor with a poor prognosis and high mortality.

Traumatic lumbar hernia: A systematic review of the literature

Purpose:Traumatic lumbar hernia(TLH)constitutes a protrusion of content through a defect in the posterior abdominal wall,as a result of injury.This rare entity has been described in limited number of cases.Methods:A systematic review of the literature was performed according to the meta-analysis of observational studies in epidemiology guidelines.The English literature from 1990 until 2021 was reviewed,using PubMed,EMBASE and Google Scholar bibliographic databases,to identify case reports and case series with patients that were diagnosed with TLH.For each eligible study,demographics,clinical presentation,hernia characteristics,preoperative imaging investigations,operation details,and postoperative data were extracted for assessment.Statistical analysis was performed on SPSS,version 20.0.Results:A total of 62 studies were included for review,with 164 patients with TLH.Mean age was(42.6±14.3)years(47.6%males,31.1%females,gender not specified in 35 cases).Mean diameter of hernia neck was(6.3±3.1)cm,while the triangles of Petit and Grynfeltt were affected in 74.5%and 14.6%,respectively.Patients diagnosed in the emergency setting account for 54.2%,with CT scan establishing diagnosis in all but one case(97.7%).A delayed diagnosis was made in 45.8%,at a mean 1 year following trauma.Flank bulging(82.8%)and chronic back pain(34.3%)were the most frequent symptoms.In both delayed and acute group,open surgery(63.6%and 92.3%,respectively)was the preferred surgical approach.Postoperative complications were reported in 11.4%of acute and 15.0%of delayed patients.Hernia recurrence was 7%.Conclusions:TLH is uncommon with 164 cases described since 1990.CT scan is the gold standard in diagnosis.Open surgery is generally the preferred approach,particularly in the emergency setting.Acute TLH can be treated either by primary suture repair or mesh,depending on the local conditions,whereas delayed cases usually require a mesh.