{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,21]],"date-time":"2026-05-21T16:05:21Z","timestamp":1779379521325,"version":"3.53.1"},"reference-count":32,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,5,21]],"date-time":"2026-05-21T00:00:00Z","timestamp":1779321600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Surg."],"abstract":"<jats:p>\n                    Bilateral Wilms tumor (BWT) accounts for 5%\u20137% of all Wilms tumors. BWT presents with an increased complexity and poorer prognosis than unilateral tumors. Currently, the standard treatment involves neoadjuvant chemotherapy combined with nephron-sparing surgery to preserve renal function. While regimens established by leading cooperative groups have improved survival rates, patients with high-risk molecular alterations, such as TP53 mutations, often exhibit intrinsic chemoresistance, complicating their management. We report the case of a 3-year-old girl who presented with intermittent abdominal pain. Imaging demonstrated bilateral stage V WT, with multiple hepatic and pulmonary metastases and extensive lymphadenopathy. She was managed according to the Chinese Children Cancer Group (CCCG)-WT-2019 protocol, receiving neoadjuvant VAD (vincristine, actinomycin D, and doxorubicin) chemotherapy, followed by staged bilateral nephron-sparing surgery. Histopathological examination demonstrated a high-risk, blastemal-predominant WT. Molecular analysis identified a\n                    <jats:italic>TP53<\/jats:italic>\n                    p.R273H missense mutation. Fluorescence\n                    <jats:italic>in situ<\/jats:italic>\n                    hybridization (FISH) analysis further demonstrated 1q gain and 16q deletion. Postoperative treatments included targeted oral therapy, multiple chemotherapy regimens, and radiotherapy; however, the disease progressed. Ultimately, the tumor advanced to end-stage disease, and the patient died. This highlights the limitations of current histology- and stage-based approaches for specific molecular subtypes. Current evidence supports more aggressive surgical intervention for high-risk cases, but it is essential to balance the need for renal function preservation with the goal of achieving oncological control.\n                  <\/jats:p>","DOI":"10.3389\/fsurg.2026.1759786","type":"journal-article","created":{"date-parts":[[2026,5,21]],"date-time":"2026-05-21T15:12:45Z","timestamp":1779376365000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Case Report: Bilateral Wilms tumor with TP53 mutation: a case-based review of clinical challenges"],"prefix":"10.3389","volume":"13","author":[{"given":"Xin","family":"Zhang","sequence":"first","affiliation":[{"name":"Department of Pediatric Hematology\/Oncology, School of Medicine, Xinhua Hospital, Shanghai Jiao Tong University","place":["Shanghai, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qianqian","family":"Cheng","sequence":"additional","affiliation":[{"name":"Pediatric Surgery, School of Medicine, Xinhua Hospital, Shanghai Jiao Tong University","place":["Shanghai, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pinxiu","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Pediatric Hematology\/Oncology, School of Medicine, Xinhua Hospital, Shanghai Jiao Tong University","place":["Shanghai, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhen","family":"Tan","sequence":"additional","affiliation":[{"name":"Department of Pediatric Hematology\/Oncology, School of Medicine, Xinhua Hospital, Shanghai Jiao Tong University","place":["Shanghai, 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