Clinical characteristics, treatment, and prognosis of undifferentiated embryonal sarcoma of the liver in children: A retrospective study from four hospitals in China.
AI interpretation is pending for this paper.
Open original publication →What the AI sees
Not AI summarized yet.
Research significance
Pending deeper interpretation.
Source abstract
BACKGROUND: Undifferentiated embryonal sarcoma of the liver (UESL) is a rare and aggressive pediatric malignancy. This study aims to provide clinical evidence for the diagnosis and treatment of UESL. METHODS: Children diagnosed with UESL by pathology at four pediatric medical centers in China between January 2010 and December 2022 were included. Clinical characteristics and prognostic data were retrospectively collected and analyzed. RESULTS: A total of 37 children with UESL were included. 40.5 % (15/37) of patients underwent ultrasound-guided percutaneous biopsy and were diagnosed with UESL, 14 of whom had no complications. However, one patient died from tumor rupture and hemorrhage one week after the biopsy. Neoadjuvant chemotherapy (NAT) was administered to 45.9 % (17/37) of patients, with 47.1 % (8/17) achieving partial response. The median maximum tumor diameter decreased from 14.5 cm to 12.1 cm (p = 0.023), and tumors in the NAT group demonstrated a significantly higher necrosis rate (p = 0.02). Surgical resection followed by postoperative chemotherapy was performed in 94.6 % (35/37) of patients. Two patients died from massive hemorrhage caused by tumor rupture at initial diagnosis. A total of 11 patients (29.7 %) experienced tumor recurrence or progression. The 5-year overall survival (OS), event-free survival (EFS), and cumulative incidence of local progression (CILP) were 81.6 %, 61.1 %, and 21.6 %, respectively. Multivariate analysis identified positive surgical margins as a significant risk factor for EFS (p = 0.038). CONCLUSIONS: Ultrasound-guided percutaneous biopsy can be considered for diagnosing UESL in children; however, it may carry a risk of severe hemorrhage. NAT improves resectability by inducing tumor shrinkage and necrosis. A positive surgical margin is significantly associated with reduced EFS. LEVEL OF EVIDENCE: Level III (Retrospective comparative study).