Histology, fusion status, and outcome in metastatic rhabdomyosarcoma: A report from the Children's Oncology Group

Pediatric Blood & Cancer
Erin R RudzinskiD M Parham

Abstract

Distinguishing alveolar rhabdomyosarcoma (ARMS) from embryonal rhabdomyosarcoma (ERMS) has historically been of prognostic and therapeutic importance. However, classification has been complicated by shifting histologic criteria required for an ARMS diagnosis. Children's Oncology Group (COG) studies after IRS-IV, which included the height of this diagnostic shift, showed both an increased number of ARMS and an increase in the proportion of fusion-negative ARMS. Following diagnostic standardization and histologic re-review of ARMS cases enrolled during this era, analysis of low-risk (D9602) and intermediate-risk (D9803) rhabdomyosarcoma (RMS) studies showed that fusion status rather than histology best predicts prognosis for patients with RMS. This analysis remains to be completed for patients with high-risk RMS. We re-reviewed cases on high-risk COG studies D9802 and ARST0431 with an enrollment diagnosis of ARMS. We compared the event-free survival (EFS) and overall survival by histology, PAX-FOXO1 fusion, and clinical risk factors (Oberlin score) for patients with metastatic RMS using the log-rank test. Histology re-review resulted in reclassification as ERMS for 12% of D9802 cases and 5% of ARST0431 cases. Fusion-negative RMS ...Continue Reading

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Citations

Feb 23, 2018·Virchows Archiv : an International Journal of Pathology·Tobias M DantonelloUNKNOWN Cooperative Weichteilsarkom Studiengruppe (CWS)
Dec 14, 2018·Children·Juan P Gurria, Roshni Dasgupta
Jun 22, 2019·Pediatric Blood & Cancer·Marielle E YoheDavid M Langenau
Feb 27, 2020·Pediatric Blood & Cancer·Kayleen A Bailey, Leonard H Wexler
May 23, 2019·Skeletal Muscle·Narendra BharathyCharles Keller
Sep 15, 2017·Pediatric Blood & Cancer·Scott C BorinsteinLars M Wagner
Apr 3, 2020·Cold Spring Harbor Molecular Case Studies·Cora A RickerCharles Keller
Aug 5, 2020·Strahlentherapie und Onkologie : Organ der Deutschen Röntgengesellschaft ... [et al]·Maria-Elena A SalfelderStephanie E Combs
Jun 14, 2020·Cold Spring Harbor Molecular Case Studies·Kenneth A CrawfordCharles Keller
Nov 28, 2020·Pediatric Blood & Cancer·Soledad GallegoUNKNOWN European paediatric Soft tissue sarcoma Study Group and Children's Oncology Group
Jan 14, 2021·Cold Spring Harbor Molecular Case Studies·Cora A RickerNoah E Berlow
Mar 13, 2021·Surgical Oncology Clinics of North America·Timothy N Rogers, Roshni Dasgupta
Apr 2, 2021·CA: a Cancer Journal for Clinicians·Erin ButlerTheodore W Laetsch
Apr 16, 2021·Molecular Cancer Therapeutics·Megan M ClearyCharles Keller
Jun 12, 2021·Seminars in Cancer Biology·Mirjam Blattner-JohnsonElke Pfaff
Mar 17, 2021·Cold Spring Harbor Molecular Case Studies·Noah E BerlowCharles Keller
Aug 11, 2021·Surgical Pathology Clinics·Jonathan C Slack, Alanna J Church
Aug 7, 2021·Molecular Cancer Therapeutics·Avanthi Tayi ShahE Alejandro Sweet-Cordero

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