Ewing Sarcoma With Emphasis on Extra-skeletal Ewing Sarcoma: A Decade’s Experience From a Single Centre in India

Abstract

Introduction: The diagnosis of Ewing sarcoma family of tumours (ESFT) is challenging, especially in adults and in extra-skeletal or visceral location. Several morphologic mimics with varied treatment options and prognosis confer diagnostic dilemmas. Application of ancillary diagnostic modalities in surgical pathology in clinical routine has enabled accurate diagnosis of ESFT in bone, soft tissues, and viscera. Aim: The study aims to assess the clinicopathological features including molecular test results of ESFT with emphasis on sex, age, and location, especially extra-skeletal soft tissue and visceral location. Material and Methods: Data of clinicopathological, molecular tests (wherever performed), diagnosis rendered in 302 ESFT over a decade from our centre were reviewed. Statistical comparison of skeletal and extra-skeletal tumours with reference to age and sex was done using SPSS package. The P value of <.05 was considered significant. Results: The cohort included 302 ESFTs with 49% skeletal and 51% extra-skeletal tumours. Thigh was most common site among skeletal tumours; chest wall, paraspinal location, and retroperitoneum among soft tissues (39.4%); and kidney, ovary, and cervix among visceral tumours (11.3%). Fluorescence in situ hybridisation for EWSR1 gene rearrangement was positive in 54 patients and reverse-transcriptase polymerase chain reaction in 19 patients. Predominance of male sex, younger age and location in extremities among skeletal tumours and lack of gender predilection, higher age and axial location in extra-skeletal tumours were noted, which were statistically significant. Molecular tests were performed more frequently in extra-skeletal tumours, especially in visceral tumours to establish the diagnosis. Conclusions: The study showed statistically significant differences in the age, sex, and location between skeletal and extra-skeletal ESFT. The increased percentage of extra-skeletal tumours especially in viscera was attributed to the increased awareness and availability of ancillary techniques.

A primary Ewing's sarcoma of pleura: Case report and literature review

“…The immunohistochemical staining for S-100 protein, neurofilament protein, GFAP, desmin, F8, UEA-1, and keratin is negative. Finally, Ewing sarcoma can be diagnosed by detecting the rearrangement of EWS gene and related oncogene [ 15 ].…”

Neoadjuvant chemotherapy for Ewing’s sarcoma family tumors of the uterine cervix: A case report

“…ESFT of the cervix is rare and, to the best of our knowledge, only 28 cases were reported in the English literature between 1996 and 2020( Ahmad et al, 2017 , Hu et al, 2017 , Murthy et al, 2020 , Kyriazoglou et al, 2019 , Wang et al, 2017 ). We herein present a case of ESFT of the cervix and describe diagnostic and treatment procedures.…”

Primary EWS/PNET of the lung with TP53 germline and SKT11 somatic mutation: A case report and review of the literature

“…It accounts for 6%–8% of primary bone tumors in children and young adults, and its incidence peaks at 20, mostly in males 3 . Extra‐skeletal EWS/PNET is clinically rare with very low incidence, which accounts for about 1% of soft tissue sarcoma 2 . Primary pulmonary EWS/PNET (PPES) is extremely rare.…”

“…3 Extra-skeletal EWS/PNET is clinically rare with very low incidence, which accounts for about 1% of soft tissue sarcoma. 2 Primary pulmonary EWS/PNET (PPES) is extremely rare. Since Hammar et al reported the first case of PPES in 1989, 4 less than 40 cases have been reported in the literature.…”

“…Ewing's sarcoma is a rare and highly invasive mesenchymal tumor which belongs to the neuroectodermal tumor family, and includes Ewing's osteosarcoma, extra-skeletal Ewing's sarcoma, primitive neuroectodermal tumor (PNET), Askin tumor and atypical Ewing's sarcoma. 1,2 It accounts for 6%-8% of primary bone tumors in children and young adults, and its incidence peaks at 20, mostly in males. 3 Extra-skeletal EWS/PNET is clinically rare with very low incidence, which accounts for about 1% of soft tissue sarcoma.…”