Outcome of patients with undifferentiated embryonal sarcoma of the liver treated according to European soft tissue sarcoma protocols |
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| Authors: | Florent Guérin Hélène Martelli Timothy Rogers Ilaria Zanetti Sheila Terwisscha van Scheltinga Federica De Corti Gabriella Guillen Burrieza Véronique Minard-Colin Daniel Orbach Max M van Noesel Marie Karanian Raquel Dávila Fajardo Johannes H M Merks Andrea Ferrari Gianni Bisogno |
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| Institution: | 1. Department of Pediatric Surgery, Université Paris-Saclay, Assistance Publique Hôpitaux de Paris (AP-HP), Bicêtre Hospital, Le Kremlin Bicêtre, France;2. Department of Pediatric Surgery, University Hospitals Bristol and Weston NHS foundation trust, Bristol, UK;3. Department of Women's and Children's Health, Hematology Oncology Division, University of Padova, Padua, Italy;4. Princess Máxima Center for Pediatric Oncology, Utrecht, The Netherlands;5. Department of Women's and Children's Health, Pediatric Surgery Unit, University of Padova, Padua, Italy;6. Department of Pediatric Surgery, Vall d'Hebron University Hospital, Barcelona, Spain;7. Department of Pediatric and Adolescent Oncology, INSERM U1015, Gustave Roussy, Université Paris-Saclay, Villejuif, France;8. SIREDO Oncology Center (Care, Innovation and Research for Children, Adolescents and Young Adults with Cancer), Paris Science et Lettres University, Institut Curie, Paris, France;9. Department of Biopathology, UNICANCER, Centre Leon Bérard, Lyon, France;10. Department of Radiation Oncology, University Medical Center Utrecht, Utrecht, The Netherlands;11. Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy |
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| Abstract: | 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. |
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| Keywords: | antineoplasic agents liver pediatrics radiotherapy sarcoma surgical procedures |
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