A journey full of surprises: surgical insights from pediatric undifferentiated embryonal liver sarcomas

Abstract

Aim: Undifferentiated embryonal sarcoma of the liver (UESL) presents significant diagnostic and therapeutic challenges due to its rarity and unpredictable clinical course. This study emphasizes the importance of individualized, case-based assessment by highlighting the distinctive preoperative, intraoperative, and postoperative challenges encountered in pediatric UESL patients.

Material and methods: We performed a retrospective review of nine pediatric patients treated for UESL at our institution from 2012 to 2022. We systematically evaluated clinical presentations, radiological assessments, surgical techniques, perioperative findings, treatment protocols, and follow-up outcomes.

Results: Each patient presented unique diagnostic and therapeutic challenges at various management stages. Preoperatively, overlapping radiologic and immunohistochemical features led to diagnostic uncertainty; notably, weak β-catenin positivity and low AFP levels initially resulted in misdiagnosis and incorrect treatment protocol application in one case. Significant intraoperative events included unexpected pulmonary embolism, while another patient developed posterior reversible encephalopathy syndrome (PRES) immediately preoperatively, delaying surgical intervention. Excluding one patient who experienced an unexplained sudden death on postoperative day 5, no other major surgical complications (such as hemorrhage, infection, bile leakage, biliary complications, or sepsis) were recorded. At a median follow-up of 7.2 years (range: 13 months to 12 years), five patients remained disease-free, one patient continued with intermittent chemotherapy, two patients succumbed to progressive metastatic recurrence, and one patient died postoperatively on day 5 due to an unexplained event.

Conclusion: UESL requires individualized, case-based evaluation and multidisciplinary collaboration. Each patient may present unique diagnostic, surgical, and postoperative management challenges. Awareness of potential complications and the unpredictable nature of UESL highlights the importance of centralized, multidisciplinary care to optimize clinical outcomes.