Outcome of patients with undifferentiated embryonal sarcoma of the liver treated according to European soft tissue sarcoma protocols.

Autor: Guérin F; Department of Pediatric Surgery, Université Paris-Saclay, Assistance Publique Hôpitaux de Paris (AP-HP), Bicêtre Hospital, Le Kremlin Bicêtre, France., Martelli H; Department of Pediatric Surgery, Université Paris-Saclay, Assistance Publique Hôpitaux de Paris (AP-HP), Bicêtre Hospital, Le Kremlin Bicêtre, France., Rogers T; Department of Pediatric Surgery, University Hospitals Bristol and Weston NHS foundation trust, Bristol, UK., Zanetti I; Department of Women's and Children's Health, Hematology Oncology Division, University of Padova, Padua, Italy., van Scheltinga ST; Princess Máxima Center for Pediatric Oncology, Utrecht, The Netherlands., De Corti F; Department of Women's and Children's Health, Pediatric Surgery Unit, University of Padova, Padua, Italy., Burrieza GG; Department of Pediatric Surgery, Vall d'Hebron University Hospital, Barcelona, Spain., Minard-Colin V; Department of Pediatric and Adolescent Oncology, INSERM U1015, Gustave Roussy, Université Paris-Saclay, Villejuif, France., Orbach D; SIREDO Oncology Center (Care, Innovation and Research for Children, Adolescents and Young Adults with Cancer), Paris Science et Lettres University, Institut Curie, Paris, France., van Noesel MM; Princess Máxima Center for Pediatric Oncology, Utrecht, The Netherlands., Karanian M; Department of Biopathology, UNICANCER, Centre Leon Bérard, Lyon, France., Fajardo RD; Department of Radiation Oncology, University Medical Center Utrecht, Utrecht, The Netherlands., Merks JHM; Princess Máxima Center for Pediatric Oncology, Utrecht, The Netherlands., Ferrari A; Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy., Bisogno G; Department of Women's and Children's Health, Hematology Oncology Division, University of Padova, Padua, Italy.
Jazyk: angličtina
Zdroj: Pediatric blood & cancer [Pediatr Blood Cancer] 2023 Jul; Vol. 70 (7), pp. e30374. Date of Electronic Publication: 2023 Apr 21.
DOI: 10.1002/pbc.30374
Abstrakt: Background: To assess the outcomes of pediatric patients with undifferentiated embryonal sarcoma of the liver (UESL) and treatment including at least surgery and systemic chemotherapy.
Methods: This study included patients aged up to 21 years with a pathological diagnosis of UESL prospectively enrolled from 1995 to 2016 in three European trials focusing on the effects of surgical margins, preoperative chemotherapy, use of radiotherapy (RT), and chemotherapy.
Results: Out of 65 patients with a median age at diagnosis of 8.7 years (0.6-20.8), 15 had T2 tumors, and one had lymph node spread, 14 were Intergroup Rhabdomyosarcoma Study (IRS) I, nine IRS II, 38 IRS III, and four IRS IV. Twenty-eight upfront surgeries resulted in five operative spillages and 11 infiltrated surgical margins, whereas 37 delayed surgeries resulted in no spillages (p = .0119) and three infiltrated margins (p = .0238). All patients received chemotherapy, including anthracyclines in 47. RT was administered in 15 patients. With a median follow-up of 78.6 months, 5-year overall and event-free survivals (EFS) were 90.1% (95% confidence interval [CI]: 79.2-95.5) and 89.1% (95% CI: 78.4-94.6), respectively. Two out four local relapses had previous infiltrated margins and two out of three patients with metastatic relapses received reduced doses of alkylating agents. Infiltrated margins (p = .1607), T2 stage (p = .3870), use of RT (p = .8731), and anthracycline-based chemotherapy (p = .1181) were not correlated with EFS.
Conclusions: Multimodal therapy improved the outcome of UESL. Neoadjuvant chemotherapy for pediatric patients increases the probability of complete surgical resection. The role of anthracyclines and RT for localized disease remains unclear.
(© 2023 The Authors. Pediatric Blood & Cancer published by Wiley Periodicals LLC.)
Databáze: MEDLINE
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