Treatment Options for UESL

UESL is rare. Only small series have been published regarding treatment.[20]

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Treatment options for UESL include the following:

Surgical resection and chemotherapy.

Liver transplant for unresectable tumors.

The generally accepted approach is resection of the primary tumor mass in the liver when possible.[18] Use of aggressive chemotherapy regimens seems to have improved the OS of patients with UESL. Neoadjuvant chemotherapy can be effective in decreasing the size of an unresectable primary tumor mass, resulting in resectability.[8-11] Most patients are treated with chemotherapy regimens used for pediatric rhabdomyosarcoma or Ewing sarcoma without cisplatin.[6]; [7-16,21][Level of evidence C1]

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Evidence (surgical resection and chemotherapy):

The Italian and German Soft Tissue Sarcoma Cooperative Groups prospectively studied patients with UESL. Patients were treated with conservative surgery or biopsy followed by neoadjuvant chemotherapy consisting of varying combinations of vincristine, cyclophosphamide, dactinomycin, doxorubicin, and ifosfamide. Disease evaluation, usually after four cycles of chemotherapy, was followed by second-look surgery when appropriate to try to remove residual primary tumor, followed by additional and/or adjuvant chemotherapy.[12]

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The Italian and German Soft Tissue Sarcoma Cooperative Groups prospectively studied patients with UESL. Patients were treated with conservative surgery or biopsy followed by neoadjuvant chemotherapy consisting of varying combinations of vincristine, cyclophosphamide, dactinomycin, doxorubicin, and ifosfamide. Disease evaluation, usually after four cycles of chemotherapy, was followed by second-look surgery when appropriate to try to remove residual primary tumor, followed by additional and/or adjuvant chemotherapy.[12]

Ten of 17 patients survived in first complete remission, and one patient survived in third complete remission.

In a subset analysis from the Children's Oncology Group ARST0332 (NCT00346164) study, 39 patients with embryonal sarcoma of the liver were available for analysis. Patients underwent upfront (n = 23) or delayed (n = 16) resection and received adjuvant or neoadjuvant chemotherapy (dose-intensive ifosfamide/doxorubicin). Eight patients received radiation therapy.[22]

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In a subset analysis from the Children's Oncology Group ARST0332 (NCT00346164) study, 39 patients with embryonal sarcoma of the liver were available for analysis. Patients underwent upfront (n = 23) or delayed (n = 16) resection and received adjuvant or neoadjuvant chemotherapy (dose-intensive ifosfamide/doxorubicin). Eight patients received radiation therapy.[22]

The 5-year EFS rate was 79% (95% CI, 65%–93%), and the 5-year OS rate was 95% (95% CI, 87%–100%).

In a single-center retrospective report, five patients with UESL were treated with surgery and adjuvant chemotherapy consisting of vincristine, doxorubicin, cyclophosphamide, ifosfamide, and etoposide. Four patients had stage I disease, and one patient had stage II disease. One patient received abdominal radiation for tumor rupture.[17][Level of evidence C1]

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In a single-center retrospective report, five patients with UESL were treated with surgery and adjuvant chemotherapy consisting of vincristine, doxorubicin, cyclophosphamide, ifosfamide, and etoposide. Four patients had stage I disease, and one patient had stage II disease. One patient received abdominal radiation for tumor rupture.[17][Level of evidence C1]

All patients were alive (range, 5–19 years), with EFS and OS rates of 100%.

Liver transplant has occasionally been used to successfully treat an otherwise unresectable primary tumor.[14,16,18,23]

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Studies cited in this source section

Publication references