Morgane Pondrom1, Daniele Pariente2, Brenda Mallon1, Sophie Taque3, Sophie Branchereau2, Christophe Chardot4, Véronique Laithier5, Marie-Dominique Tabone6, Julien Lejeune7, Cécile Faure-Conter8, Laure Saumet9, Cécile Vérité10, Isabelle Aerts11, Laurence Brugières1, Brice Fresneau1,12. 1. Gustave Roussy, Department of Children and Adolescents Oncology, Université Paris-Saclay, Villejuif, France. 2. Centre hospitalier universitaire de Bicêtre, APHP, Department of Pediatric Radiology, Le Kremlin-Bicêtre, France. 3. Department of Pediatric Onco-hematology, Centre hospitalier universitaire, Rennes, France. 4. Department of Pediatric Surgery, Hôpital Necker-Enfants malades, Paris, France. 5. Department of Pediatric Onco-hematology, Centre hospitalier universitaire, Besançon, France. 6. Department of Pediatric Onco-hematology, Hôpital Trousseau, Paris, France. 7. Department of Pediatric Onco-hematology, Centre hospitalier universitaire, Tours, France. 8. IHOPe (Institut d'Hématologie et d'Oncologie Pédiatrique), Lyon, France. 9. Department of Pediatric Onco-hematology, Centre hospitalier universitaire, Montpellier, France. 10. Department of Pediatric Onco-hematology, Centre hospitalier universitaire, Bordeaux, France. 11. SIREDO Oncology Center (Care, Innovation and Research for Children, Adolescents and Young Adults with Cancer), Paris, France. 12. Paris-Saclay University, CESP, INSERM, Paris-Sud University, Villejuif, France.
Abstract
BACKGROUND: Hepatoblastoma tumor rupture is a high-risk criterion in the SIOPEL 3/4 protocol. Little is known about the outcome of these children. METHODS: Radiological signs of possible tumor rupture, defined as peritoneal effusion, peritoneal nodules, or hepatic subcapsular hematoma, were reported in 24 of 150 patients treated for hepatoblastoma in France from January 2000 to December 2014 after central radiological expert review. RESULTS: Twenty-two patients with available clinical data were included (nine PRETEXT-I/II, six PRETEXT-III, seven PRETEXT-IV, and five had lung metastases). Five patients had a subcapsular hematoma only, and 17 patients had intraperitoneal rupture (subcapsular hematoma and peritoneal effusion). A hepatic biopsy was performed in 19 patients. Intraperitoneal rupture occurred before biopsy in 12 and after biopsy in three (including one with prebiopsy subcapsular hematoma) (missing data: two). All patients were treated with chemotherapy, with high-risk regimens including cisplatin or carboplatin and doxorubicin in 19 and cisplatin or carboplatin alone in three. Liver surgery was performed in 20 patients (including three liver transplants). Fifteen patients (68%) achieved complete remission. With a median follow-up of 5.5 years, 11 events occurred (six progressions and three relapses, including three peritoneal progressions/relapses, one surgical complication, and one second cancer) and eight patients died. One of eight patients with no other high-risk criterion had a relapse. The three-year event-free survival and overall survival rates were 49.6% (95% CI = 30-69) and 68.2% (40-84), respectively. CONCLUSIONS: Tumor rupture is predictive of poor prognosis with risk of peritoneal progression/relapse. However, it should not be a contraindication for liver transplantation.
BACKGROUND:Hepatoblastoma tumor rupture is a high-risk criterion in the SIOPEL 3/4 protocol. Little is known about the outcome of these children. METHODS: Radiological signs of possible tumor rupture, defined as peritoneal effusion, peritoneal nodules, or hepatic subcapsular hematoma, were reported in 24 of 150 patients treated for hepatoblastoma in France from January 2000 to December 2014 after central radiological expert review. RESULTS: Twenty-two patients with available clinical data were included (nine PRETEXT-I/II, six PRETEXT-III, seven PRETEXT-IV, and five had lung metastases). Five patients had a subcapsular hematoma only, and 17 patients had intraperitoneal rupture (subcapsular hematoma and peritoneal effusion). A hepatic biopsy was performed in 19 patients. Intraperitoneal rupture occurred before biopsy in 12 and after biopsy in three (including one with prebiopsy subcapsular hematoma) (missing data: two). All patients were treated with chemotherapy, with high-risk regimens including cisplatin or carboplatin and doxorubicin in 19 and cisplatin or carboplatin alone in three. Liver surgery was performed in 20 patients (including three liver transplants). Fifteen patients (68%) achieved complete remission. With a median follow-up of 5.5 years, 11 events occurred (six progressions and three relapses, including three peritoneal progressions/relapses, one surgical complication, and one second cancer) and eight patientsdied. One of eight patients with no other high-risk criterion had a relapse. The three-year event-free survival and overall survival rates were 49.6% (95% CI = 30-69) and 68.2% (40-84), respectively. CONCLUSIONS:Tumor rupture is predictive of poor prognosis with risk of peritoneal progression/relapse. However, it should not be a contraindication for liver transplantation.