Infographic: Survival in children with hepatoblastoma and pulmonary metastatic disease at diagnosis based on extent of metastases and need for surgical clearance
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Stephanie F Polites, Robert A Vierkant, Jennifer H Aldrink, Timothy B Lautz, Mecklin V Ragan, Audra Reiter, Stephanie Y Chen, Eugene S Kim, Hannah N Rinehardt, Marcus Malek, Andrew M Fleming, Andrew J Murphy, Jonathan P Roach, Sridharan Radhakrishnan, Nelson Piche, Yasmin Osman, Harold N Lovvorn 3rd, Elisabeth T Tracy, Juan Favela, Hau D Le, John Marquart, Brian Craig, Dave R Lal, Natashia Seemann, Robin Petroze, Barrie S Rich, Richard D Glick, Leigh Selesner, Ashley Yoo, Elizabeth Fialkowski, Erin G Brown, Chloe Boehmer, Roshni Dasgupta, Max R Langham Jr, Gregory M Tiao, Zachary J Kastenberg
Purpose: This study analyzed the effect of pulmonary metastatic burden at diagnosis and response to chemotherapy on disease-free and overall survival in patients with hepatoblastoma (HB), hypothesizing that long-term survival can be achieved in the setting of extensive pulmonary metastatic disease requiring metastasectomy.
Methods: An analysis of a multicenter dataset including children with HB and pulmonary metastatic disease at diagnosis was performed. Patients who presented with >10 pulmonary metastases were defined as having extensive metastatic disease. Kaplan Meier and univariate Cox Proportional Hazards analyses assessed disease-free survival (DFS) and overall survival (OS), analyzing the effect of pulmonary metastatic disease extent and modality of clearance.
Results: Among 315 patients with HB, pulmonary metastatic disease at diagnosis (n = 62) was associated with poorer DFS and OS (both p > 0.05). Of patients with pulmonary metastases, 23 (37 %) had extensive disease. Clearance of metastases occurred in 59 (95 %) patients: 32 (54 %) with chemotherapy alone and 27 (46 %) with chemotherapy and metastasectomy. Survival was similar between patients with >10 vs. ≤10 lung metastases and between those who had clearance of lung metastases by chemotherapy alone vs. chemotherapy and metastasectomy (all p > 0.05). Among 16 patients who required liver transplantation, all patients who underwent surgical clearance of metastatic disease were alive at last follow-up.
Conclusion: Among patients with HB who had pulmonary metastatic disease at diagnosis, long-term survival is possible regardless of the extent of pulmonary disease. A combination of neoadjuvant chemotherapy and metastasectomy can be utilized to achieve lung clearance.
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