Preoperative Interventional Therapy for Childhood Undifferentiated Embryonal Liver Sarcoma: Two Retrospective Cases from a Single Center

Abstract

Background Undifferentiated embryonal liver sarcoma (UELS) accounts for only 9 to 15% of all malignant liver tumors in children. Typically, UELS occurs in older children and presents as an abdominal mass. Most UELS are unresectable because of the later diagnosis. The outcome of UELS is very poor, with a 5-year overall survival of < 37.5%. Transarterial chemoembolization (TACE) has been reported to be an effective modality for unresectable liver tumors. To investigate the effects of TACE on UELS in children, we present two cases of children with UELS who underwent TACE and surgical resection in our center within the past 10 years. Methods In this study, two children with UELS were treated using TACE with cisplatin, doxorubicin, and iodized oil. The size of the tumors was measured before and after TACE using ultrasonography. Routine was also given before and after surgical resection. Side effects were recorded. Both patients had follow-up. Results After interventional therapy, both patients presented with vomiting, fever, and transient liver dysfunction without cardiac or renal dysfunction. One patient had bone marrow depression. The size of the tumors was reduced by 23% to 31% after TACE. The tumors were completely removed by surgical procedures after 4 weeks of TACE in both patients. One patient survived free of disease for 1 year, and the other survived free of disease for 9 years. Conclusion TACE yielded satisfactory results for unresectable UELS in children, with lower dosage of chemotherapy and fewer side effects. It may be applied as a preoperative therapy for children with unresectable UELS.

Neoadjuvant transcatheter arterial chemoembolization and systemic chemotherapy for the treatment of undifferentiated embryonal sarcoma of the liver in children

“…Since then, reports of TACE in pediatric cases of hepatoblastoma have gradually increased[ 6 , 14 , 15 ]. In previous studies at our center, preoperative TACE was effective in the treatment of UESL in two cases[ 5 ]. Also, neoadjuvant TACE and systemic chemotherapy have been administered in patients with Wilms tumor and clear cell sarcoma of the kidney and achieved satisfactory results[ 7 , 16 ].…”

“…A 5-F Pigtail catheter was introduced into the abdominal aorta to perform aortography and define the main feeding artery of the tumor, and then a 4-F or 5-F Cobra catheter was advanced toward the tumor-feeding arteries for chemoembolization. The chemoembolic emulsion consisted of pirarubicin 40 mg/m 2 , vindesine 3 mg/m 2 , cisplatin 90 mg/m 2 and iodized oil 0.5 mL per maximum tumor diameter (cm), which has been proved to have good efficacy and safety in the treatment of UESL and other tumors in our center[ 5 , 7 ]. The drugs were mixed and diluted in 120-180 mL of normal saline, and infused into the feeding artery at a rate of 120 mL/h.…”

“…Complete tumor excision plays the central role in the treatment of UESL[ 4 ]. Transcatheter arterial chemoembolization (TACE) is an effective therapeutic method and has been widely used in the treatment of patients with hepatic malignancy[ 5 , 6 ]. In an attempt to increase resection rate and improve the outcome of UESL, we performed preoperative TACE combined with systematic chemotherapy for the treatment of unresectable UESL in children and evaluated its feasibility and effectiveness.…”

Adult Embryonal Sarcoma of the Liver: Management of a Massive Liver Tumor

“…A report of two pediatric cases treated with TACE prior to resection reported a 23–31% decrease in tumor size [16]. In general, embolization techniques have been successful in the treatment of liver lesions with a response rate of 12% by RECIST criteria reported for transarterial embolization (TAE) and TACE independent of pretreatment lesion size and vascularity with a greater benefit seen with the addition of chemotherapy compared to bland embolization [17].…”

Surgical perspective on treatment of pediatric undifferentiated sarcoma of the liver

“…A case series dating from 2000, evaluating the role of transhepatic arterial chemoembolization (TACE) in liver tumors, showed that in two children with UESL TACE did not show any response to this type of therapy [15] . However, in a recent report of two cases, TACE combined with neoadjuvant chemotherapy with reduced dosage, allowed for significant reduction of tumor size.…”