Adil Abdelhamed Abbas1* and Hatim Qasim Almaghraby2
1The Pediatric Hematology/Oncology Section, King Saud bin Abdulaziz University for Health Sciences, KSA
2Department of Laboratory Medicine, King Saud bin Abdulaziz University for Health Sciences, KSA
The Askin-Rosai (A-R) tumor merely represents specific histopathological appearance of Malignant Round Blue Cell Tumor (MRBCT) originating in the bones or the soft tissues of the thoracic cavity. On light microscopy, the same histopathological appearance of A-R tumor has been termed Ewing Sarcoma (ES), Primitive Neuroectodermal Tumor (PNET), by various other authors. The immunophenotypic profile is similar. The chromosomal translocation t (11; 22) (q24; q12) resulting in the EWSR1-FLI1 fusion gene is detected in nearly 90% of cases of ES, PNET and A-R tumor. Therefore, A-R tumor, ES and PNET are currently regarded as one entity grouped together under the Ewing Family Tumors (EFT) and are treated in an exactly identical way. Nearly a quarter of patients develop metastasis in the lungs, bone or Bone Marrow (BM). Because of rarity of the A-R tumor, there are no separate treatment trials and the disease is treated within the contest of the EFT. Optimal treatment requires the use of adjuvant and new adjuvant Chemotherapy (CTR), radical surgical resection and/or involved field Radiotherapy (RT). Without CTR 90% of the patient relapse and die as a result of disease. Effective radical surgical resection was always complicated by the tumor size and local invasiveness. Compared to other EFT at other locations the overall prognosis of A-R tumor remains poor. Variable Overall Survival (OS) and Disease-Free Survival (DFS) at 5-years results were reported for localized disease (45% to 60% and 60% to 85% respectively). The OS for metastatic disease remains very poor at around 15% at 3-years. Little progress has been made in the treatment over the last 3 decades. Improved CTR regimens, surgical and RT delivery techniques and the use of newer targeted medications represent the hope for improving the outcomes. Literature review revealed the presence of large number of case reports and case series which indicates the rarity of this late childhood tumor, its aggressive nature. Larger randomized controlled trials are needed. In this review article we provided detailed account of this group of tumors and discuss the most recent advances used in the diagnosis and treatment.
Abbas AA, Almaghraby HQ. The Askin- Rosai Tumor: A Clinicopathological Review. Clin Surg. 2020; 5: 2914..