Premium
Liver metastasis at diagnosis in children with nephroblastoma enrolled in SIOP2001 protocol: A French multicentric study
Author(s) -
Liné Antoine,
SudourBonnange Hélène,
LanguillatFouquet Virginie,
Brisse Hervé,
Irtan Sabine,
Verschuur Arnauld,
Sarnacki Sabine,
Thébaud Estelle,
CoulombL'Hermine Aurore,
NotzCarrère Anne,
Michon Jean,
Tabone MarieDominique,
Boulanger Cécile,
Pellier Isabelle,
Freycon Claire,
Audry Georges,
Dijoud Frédérique,
Morelle Magali,
Bergeron Christophe,
Pasqualini Claudia
Publication year - 2020
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.28201
Subject(s) - medicine , anaplasia , metastasectomy , wilms' tumor , chemotherapy , metastasis , gastroenterology , radiation therapy , stage (stratigraphy) , cohort , histology , surgery , pathology , cancer , paleontology , biology
Background Liver metastases are rare in children with Wilms tumor (WT), and their impact on the outcome is unclear. Patients and methods The French cohort of patients with WT presenting liver metastases at diagnosis and enrolled in the International Society of Pediatric Oncology (SIOP) 2001 study was reviewed. Results From 2002 to 2012, 906 French patients were enrolled in the SIOP2001 trial. Among them, 131 (14%) presented with stage IV WT and 18 (1.9%) had liver metastases at diagnosis. Isolated liver metastases were displayed in four of them. After preoperative chemotherapy, persistent liver disease was reported in 14/18 patients, and 13 of them underwent metastasectomy after nephrectomy. In resected liver lesions, the same histology of the primary tumor was reported for three patients, blastemal cells without anaplasia were identified in one patient with DA‐WT, and post‐chemotherapy necrosis/fibrosis was identified for the other 10 patients. For the four patients who had liver and lung surgery, both sites had nonviable cells with post‐chemotherapy necrosis/fibrosis. Six patients had hepatic radiotherapy. Sixteen patients achieved primary complete remission and were alive at the last follow‐up (median follow‐up: 6.4 years). The only two deceased patients presented diffuse anaplasia histology. The five‐year EFS and OS were 83% (60%‐94%) and 88% (66%‐97%), respectively. Conclusion Liver involvement does not appear to be an adverse prognostic factor in metastatic WT. The role of hepatic surgery and radiotherapy remains unclear, and should be carefully considered in case of persistent liver metastases, according to histology and radiological response to other metastatic sites.