Undifferentiated embryonal sarcoma of the liver (UESL) is the third most common liver malignancy of childhood. Intratumoral hemorrhage and rupture of UESL represents a complex clinical scenario for which local control requires deliberate preoperative planning and consideration for advanced neoadjuvant intervention. Interestingly, liver was the only anatomic site for which radiotherapy was not included in the Children’s Oncology Group ARST0332, soft tissue sarcoma unresectable at diagnosis, neoadjuvant treatment algorithm. Here we present our institutional experience with ruptured UESL in children and describe the use of neoadjuvant transarterial radioembolization (TARE) for initially unresectable tumors.
We reviewed all cases of ruptured UESL treated at our children’s hospital over the past five years. Descriptive analyses were performed to evaluate patient and tumor characteristics. Detailed review of neoadjuvant therapy, operative technique, and adjuvant regimens was performed.
Four patients presented with ruptured UESL during the study period. Ages ranged from four to 17 years at diagnosis. All four tumors emanated from the right liver; one of which had expanded to fill the entire abdomen. Sites of extrahepatic tumor at diagnosis included lung metastasis(1); IVC/RA tumor thrombus(1); diaphragm/pleural effusion(3); peritoneal cavity myxoid tumor/hematoma rupture(3). One patient underwent upfront resection. Of the three who received neoadjuvant chemotherapy, two also received neoadjuvant TARE and one received chemoembolization. Pathologically negative margins were achieved in all four patients. The two patients who received TARE experienced significant tumor regression prior to resection and had dramatic tumor necrosis on pathologic review. All four patients are alive with no evidence of disease at last follow-up (median 36.5 months).
Ruptured UESL is a complicated oncologic situation that requires individualized management. When upfront resection is not feasible, the use of neoadjuvant TARE provides an opportunity to enhance local control and, thus, facilitate long-term survival.