Rhabdomyosarcoma and Extraosseous Ewing Sarcoma

Abstract

Rhabdomyosarcoma (RMS) is a malignant tumor that represents the most common form of pediatric soft tissue sarcoma. It arises from mesenchymal origin and forms part of the group of small round blue cell tumors of childhood. It has a constant annual incidence of 4.5 cases per 1,000,000 children. The known histological diagnosis of the two major subtypes (embryonal and alveolar) has been recently enhanced by tumor biological markers and molecular differentiation diagnostic tools that have improved not only the updated classification based on risk stratification, but also the treatment approach based on the clinical group. Ewing sarcoma (ES) is a round cell tumor, highly malignant and poorly differentiated that is currently the second most common malignant bone tumor in children. In rare instances, it develops from an extraskeletal origin, classified as extraosseous Ewing sarcoma (EES). We provide an updated, evidence-based and comprehensive review of the molecular diagnosis, clinical and diagnostic approach and a multidisciplinary medical and surgical management according to the latest standard of care for the treatment of pediatric RMS and EES.

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

“…Similar observations were made earlier. 12,13,16 The reported frequency of extra-skeletal location of tumours in various series ranged from 21% to 31% in cohort ranging from 120 to 2202 patients. 12,14,15 Reports from India described extra-skeletal tumours in small sample size of 51 to 58 patients and described the frequency to range from 23% to 29%.…”

“… 12 - 14 The clinicopathological features and prognosis were reported to be different in skeletal and extra-skeletal tumours. 12 - 21 This article aims to study the clinicopathological features and the role of ancillary techniques in the diagnosis of ESFT with particular emphasis on extra-skeletal soft tissue and visceral location.…”

Retargeting of NK-92 Cells against High-Risk Rhabdomyosarcomas by Means of an ERBB2 (HER2/Neu)-Specific Chimeric Antigen Receptor

“…Rhabdomyosarcoma (RMS) is the most common soft-tissue sarcoma in children and accounts for 5% of all pediatric cancers [ 1 , 2 ]. The five-year overall survival (OS) rates vary from 78% in low-risk patients to a dismal 8% in high-risk patients [ 3 ].…”

“…In addition to older age and unfavorable primary tumor site, alveolar histopathology also has a negative influence on patient outcome, as alveolar RMS (aRMS) has a higher risk of metastatic disease than other types of RMS, with 68% of patients already carrying metastases when they are first diagnosed with aRMS [ 3 , 6 , 7 ]. Other unfavorable prognostic factors are bone or bone marrow (BM) involvement, regional lymph node involvement, the presence of multiple metastases, large tumor size, invasion, and a positive fusion status by histopathology [ 2 , 3 , 8 , 9 , 10 ].…”

Primary pulmonary Ewing’s sarcoma: rare cause of massive hemothorax in a young girl-case report

“…Ewing’s sarcomas are relatively rare neuroectodermal tumors that primarily arise from the bone. Extraosseous Ewing’s sarcomas are a subset of primitive neuroectodermal tumors, which involve the soft tissues and organs such as the ovary, paravertebral soft tissue, testis, uterus, pancreas, renicapsule, and myocardium [ 1 ]. We have analyzed data from relevant studies published in PubMed Central and Excerpta Medica Database, and a total of 25 cases of primary pulmonary Ewing’s sarcoma have been reported since 2010 [ 2 – 8 ].…”