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HEALTH PROFESSIONAL · SOURCE READING

Treatment Options for Osteosarcoma and UPS of Bone With Metastatic Disease at Diagnosis

Source: Osteosarcoma and Undifferentiated Pleomorphic Sarcoma of Bone Treatment (PDQ®)–Health Professional Version, National Cancer Institute.

Source updated: December 2, 2024 · Captured 2026-09-09.

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Context: Treatment of Osteosarcoma and UPS of Bone With Metastatic Disease at Diagnosis

Treatment options for patients with osteosarcoma or undifferentiated pleomorphic sarcoma (UPS) of bone with metastatic disease at diagnosis include the following:

Chemotherapy.

The chemotherapeutic agents used include high-dose methotrexate, doxorubicin, cisplatin, high-dose ifosfamide, etoposide, and, in some reports, carboplatin or cyclophosphamide.

Evidence (chemotherapy):

In a trial that investigated high-dose ifosfamide (17.5 g per course) in combination with etoposide for patients with newly diagnosed metastatic osteosarcoma, the following was observed:[4] However, similar to localized disease, there is no evidence that the addition of ifosfamide or etoposide contributes to improved event-free survival (EFS) or overall survival (OS) in patients with metastatic disease.

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In a trial that investigated high-dose ifosfamide (17.5 g per course) in combination with etoposide for patients with newly diagnosed metastatic osteosarcoma, the following was observed:[4] However, similar to localized disease, there is no evidence that the addition of ifosfamide or etoposide contributes to improved event-free survival (EFS) or overall survival (OS) in patients with metastatic disease.

The combination produced a complete response in 10% of the patients and a partial response in 49% of the patients.

A study using a factorial design in patients with metastatic osteosarcoma (n = 91) evaluated the addition of either muramyl tripeptide or ifosfamide to a standard chemotherapy regimen that included cisplatin, high-dose methotrexate, and doxorubicin.[5]

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A study using a factorial design in patients with metastatic osteosarcoma (n = 91) evaluated the addition of either muramyl tripeptide or ifosfamide to a standard chemotherapy regimen that included cisplatin, high-dose methotrexate, and doxorubicin.[5]

There was a nominal advantage for the addition of muramyl tripeptide (but not for ifosfamide) in terms of EFS and OS, but criteria for statistical significance were not met.

In the international European and American Osteosarcoma Study (EURAMOS) group consortium, 362 of 2,186 patients (17%) presented with metastasis at diagnosis. Patients were randomly assigned to receive either treatment with cisplatin, doxorubicin, and high-dose methotrexate or cisplatin, doxorubicin, high-dose methotrexate, and ifosfamide.[6][Level of evidence A1]

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In the international European and American Osteosarcoma Study (EURAMOS) group consortium, 362 of 2,186 patients (17%) presented with metastasis at diagnosis. Patients were randomly assigned to receive either treatment with cisplatin, doxorubicin, and high-dose methotrexate or cisplatin, doxorubicin, high-dose methotrexate, and ifosfamide.[6][Level of evidence A1]

At a median follow-up of 47 months, the 3-year EFS rate was 32% (95% confidence interval [CI], 27%–37%), and the 5-year EFS rate was 28% (95% CI, 23%–33%).

In the international European and American Osteosarcoma Study (EURAMOS) group consortium, 362 of 2,186 patients (17%) presented with metastasis at diagnosis. Patients were randomly assigned to receive either treatment with cisplatin, doxorubicin, and high-dose methotrexate or cisplatin, doxorubicin, high-dose methotrexate, and ifosfamide.[6][Level of evidence A1]

The 3-year OS rate was 56% (95% CI, 50%–61%), and the 5-year OS rate was 45% (95% CI, 39%–50%).

The treatment options for UPS of bone with metastasis at initial presentation are the same as the treatment for osteosarcoma with metastasis. Patients with unresectable or metastatic UPS have a very poor outcome.[7]

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