Management of undifferentiated embryonal sarcoma of the liver: A Pediatric Surgical Oncology Research Collaborative study.
| Autor: | Kastenberg ZJ; Primary Children's Hospital, University of Utah School of Medicine, Salt Lake City, Utah, USA., Short SS; Primary Children's Hospital, University of Utah School of Medicine, Salt Lake City, Utah, USA., Riehle KJ; Seattle Children's Hospital, University of Washington School of Medicine, Seattle, Washington, USA., Utria A; Seattle Children's Hospital, University of Washington School of Medicine, Seattle, Washington, USA., Lautz TB; Lurie Children's Hospital, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA., Ott KC; Lurie Children's Hospital, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA., Murphy AJ; St Jude Children's Research Hospital, Memphis, Tennessee, USA., Mansfield S; St Jude Children's Research Hospital, Memphis, Tennessee, USA., Lal DR; Children's Wisconsin, Medical College of Wisconsin, Milwaukee, Wisconsin, USA., Hallis B; Children's Wisconsin, Medical College of Wisconsin, Milwaukee, Wisconsin, USA., Murphy JT; Children's Health Specialty Center, University of Texas - Southwestern Medical Center, Dallas, Texas, USA., Roach JP; Children's Hospital Colorado, University of Colorado School of Medicine, Denver, Colorado, USA., Polites SF; Mayo Clinic, Rochester, Minnesota, USA., Beckhorn C; Duke Children's Health Center, Duke University School of Medicine, Durham, North Carolina, USA., Tracy ET; Duke Children's Health Center, Duke University School of Medicine, Durham, North Carolina, USA., Fialkowski EA; Doernbecher Children's Hospital, Oregon Health & Sciences University, Portland, Oregon, USA., Seemann NM; Children's Hospital London Health Sciences Centre, Western University, London, Ontario, Canada., Bütter AM; Children's Hospital London Health Sciences Centre, Western University, London, Ontario, Canada., Rich BS; Cohen Children's Medical Center, Donald and Barbara Zucker School of Medicine, New Hyde Park, New York, USA., Glick RD; Cohen Children's Medical Center, Donald and Barbara Zucker School of Medicine, New Hyde Park, New York, USA., Bondoc AJ; Cincinnati Children's Hospital Medical Center, University of Cincinnati School of Medicine, Cincinnati, Ohio, USA., Ofori-Atta BS; Department of Internal Medicine, University of Utah School of Medicine, Salt Lake City, Utah, USA., Presson AP; Department of Internal Medicine, University of Utah School of Medicine, Salt Lake City, Utah, USA., Chen SY; Children's Hospital Los Angeles, Keck School of Medicine, Los Angeles, California, USA., Zamora AK; Children's Hospital Los Angeles, Keck School of Medicine, Los Angeles, California, USA., Kim ES; Children's Hospital Los Angeles, Keck School of Medicine, Los Angeles, California, USA., Vasudevan S; Texas Children's Hospital, Baylor College of Medicine, Houston, Texas, USA., Rinehardt HN; UPMC Children's Hospital of Pittsburgh, University of Pittsburgh, Pittsburgh, Pennsylvania, USA., Malek MM; UPMC Children's Hospital of Pittsburgh, University of Pittsburgh, Pittsburgh, Pennsylvania, USA., Lapidus-Krol E; Department of Surgery, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada., Putra J; Department of Pathology, Boston Children's Hospital, Boston, Massachusetts, USA., Superina RA; Lurie Children's Hospital, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA., Langham MR; St Jude Children's Research Hospital, Memphis, Tennessee, USA., Meyers RL; Primary Children's Hospital, University of Utah School of Medicine, Salt Lake City, Utah, USA., Tiao G; Cincinnati Children's Hospital Medical Center, University of Cincinnati School of Medicine, Cincinnati, Ohio, USA., Dasgupta R; Cincinnati Children's Hospital Medical Center, University of Cincinnati School of Medicine, Cincinnati, Ohio, USA., Baertschiger R; Department of Surgery, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada. |
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| Jazyk: | angličtina |
| Zdroj: | Pediatric blood & cancer [Pediatr Blood Cancer] 2024 Jun; Vol. 71 (6), pp. e30975. Date of Electronic Publication: 2024 Mar 31. |
| DOI: | 10.1002/pbc.30975 |
| Abstrakt: | Background: Undifferentiated embryonal sarcoma of the liver (UESL) is a rare tumor for which there are few evidence-based guidelines. The aim of this study was to define current management strategies and outcomes for these patients using a multi-institutional dataset curated by the Pediatric Surgical Oncology Research Collaborative. Methods: Data were collected retrospectively for patients with UESL treated across 17 children's hospitals in North America from 1989 to 2019. Factors analyzed included patient and tumor characteristics, PRETEXT group, operative details, and neoadjuvant/adjuvant regimens. Event-free and overall survival (EFS, OS) were the primary and secondary outcomes, respectively. Results: Seventy-eight patients were identified with a median age of 9.9 years [interquartile range [IQR): 7-12]. Twenty-seven patients underwent resection at diagnosis, and 47 patients underwent delayed resection, including eight liver transplants. Neoadjuvant chemotherapy led to a median change in maximum tumor diameter of 1.6 cm [IQR: 0.0-4.4] and greater than 90% tumor necrosis in 79% of the patients undergoing delayed resection. R Conclusion: Complete resection is associated with improved survival for patients with UESL. Neoadjuvant chemotherapy causes minimal radiographic response, but significant tumor necrosis. (© 2024 Wiley Periodicals LLC.) |
| Databáze: | MEDLINE |
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