Undifferentiated sarcoma of the liver in childhood

Abstract

BACKGROUND Undifferentiated (embryonal) sarcoma of the liver (UESL) is a rare childhood hepatic tumor, and it is generally considered an aggressive neoplasm with an unfavorable prognosis. METHODS The Soft Tissue Sarcoma Italian and German Cooperative Groups enrolled 17 children with UESL in studies conducted between 1979 and 1995. They were treated using the same multimodal approach as for patients with sarcomas including conservative surgery at diagnosis, multiagent chemotherapy, and second‐look operation in cases of residual disease. Radiotherapy was occasionally used (2 of 17 patients). RESULTS Twelve patients are alive with follow‐up ranging from 2.4 to 20 years. Eight underwent complete tumor resection either at diagnosis or after preoperative chemotherapy, and all are currently alive. After initial chemotherapy tumor reduction was evident in six of nine evaluable cases. Overall three patients died of disease and one of a surgical complication. One child died in second complete remission for a non‐disease‐related cause. CONCLUSIONS The current prognosis of UESL no longer should be regarded as poor. Modern multimodal treatment and supportive therapy have improved survival. Cancer 2002;94:252–7. © 2002 American Cancer Society.

Differential diagnosis and management of liver tumors in infants

“…Survival rates have significantly improved in the last decades and longterm survival cases have been reported [68,69] . In an ItalianGerman soft tissue sarcoma study [70] , 12 of 17 children with UESL achieved remission following treatment with chemoradiation and surgery. Patients whose tumor is not able to be resected or who have postoperative local recurrence of the tumor without distant metastasis may be candidates for liver transplantation.…”

Liver Tumors in Children

“…The third most common hepatic malignancy, after hepatoblastoma and hepatocellular carcinoma, is undifferentiated embryonal sarcoma [13,14]. These tumors occur in children 5-10 years of age and are mesenchymal in appearance.…”

The Complex Imaging Diagnosis of Undifferentiated Embryonal Sarcoma of the Liver in Children: The Own Experience and Literature Review

“…Благодаря нашим данным также была выяв-лена эта особенность. Необходимо отметить, что низкие показатели плотности опухолевой ткани при КТ, так же как и характерный сигнал для жид-костного содержимого на Т1-и Т2-взвешенных изображениях при МРТ, по-видимому, являются отображением миксоидной стромы, которая бога-та водой [1,10].…”

“…С другой стороны, при резистентных формах заболевания, в случаях нерезектабельной опухоли проводится трансплан-тация печени. Таким образом, за последние 25 лет данные исследований по лечению пациентов с НЭС демонстрируют улучшение выживаемости с 20 до 70% [10,11].…”