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Figure 1. Decisional algorithm for the management of undifferentiated embryonal sarcoma of the liver (UESL).
THE ROLE OF PREOPERATIVE CHEMOTHERAPY
Preoperative chemotherapy has shown to reduce tumor size and minimizes the risk of intraoperative
spillage or microscopic residue. Between 30% to 40% of the patients might not be amenable to a primary
tumor resection and are subjected to neoadjuvant chemotherapy to facilitate a complete surgical
resection [1,14] . In fact, after neoadjuvant chemotherapy, resectability has been reported to improve in up to
81% in recent series, with varied regimens that are basically used for other pediatric soft tissue
sarcomas [11,19] . Another, less studied effect of the neoadjuvant chemotherapy is the percentage of tumor
necrosis, which ranged between 70%-100% in a series of seven patients, even if the radiologic response was
[9]
not as obvious as the pathologic response .
Despite the commonly recognized credo that in case of resectable UESL, surgery could or should be done
upfront without neoadjuvant chemotherapy, some centers advocate preoperative chemotherapy regardless
of the stage of resectability. Resections seem to become easier after chemotherapy, and thus are associated
with a reduced morbidity. Nevertheless chemotherapy is also associated with multiple side effects: 5% and