Outcome of rare primary malignant bone sarcoma treated with multimodal therapy : Results from the EUROpean Bone Over 40 Sarcoma Study (EURO-B.O.S.S.)
© 2023 The Authors. Cancer published by Wiley Periodicals LLC on behalf of American Cancer Society..
BACKGROUND: Rare primary malignant bone sarcomas (RPMBS) account for 5%-10% of primary high-grade bone tumors and represent a major treatment challenge. The outcome of patients with RPMBS enrolled in the EUROpean Bone Over 40 Sarcoma Study (EURO-B.O.S.S) is presented.
METHODS: Inclusion criteria were as follows: age from 41 to 65 years and a diagnosis of high-grade spindle cell, pleomorphic, or vascular RPMBS. The chemotherapy regimen included doxorubicin 60 mg/m2 , ifosfamide 9 g/m2 , and cisplatin 90 mg/m2 ; postoperative methotrexate 8 g/m2 was added in case of a poor histologic response. Version 2.0 of the Common Terminology Criteria for Adverse Events, Kaplan-Meier curves, log-rank tests, and univariate Cox regression models were used.
RESULTS: In total, 113 patients were evaluable for analysis. The median patient age was 52 years (range, 40-66 years), and 67 patients were men. Eighty-eight tumors were categorized as undifferentiated pleomorphic sarcomas (UPS), 20 were categorized as leiomyosarcomas, three were categorized as fibrosarcomas, and two were categorized as angiosarcomas. Eighty-three of 113 tumors were located in the extremities. Ninety-five of 113 patients presented with no evidence of metastases. After a median follow-up of 6.8 years (interquartile range [IQR], 3.5-9.8 years), the 5-year overall survival rate for patients with localized disease was 68.4% (IQR, 56.9%-77.5%), and it was 71.7% (IQR, 58.1%-81.6%) for patients with UPS and 54.9% (IQR, 29.5%-74.5%) for patients with leiomyosarcoma. Grade III-IV hematologic toxicity was reported in 81% patients; 23% had grade II-III neurotoxicity, and 37.5% had grade I-II nephrotoxicity. Five-year overall survival was significantly better for patients with localized disease, for patients who obtained surgical complete remission, and when the primary tumor was located in the extremities.
CONCLUSIONS: The survival of patients who had RPMBS in the current series was similar to that of age-matched patients who had high-grade osteosarcoma treated according to the same protocol. An osteosarcoma-like chemotherapy may be proposed in patients who have RPMBS.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2023 |
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| Erschienen: |
2023 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:129 |
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| Enthalten in: |
Cancer - 129(2023), 22 vom: 15. Nov., Seite 3564-3573 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Palmerini, Emanuela [VerfasserIn] |
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| Links: |
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| Anmerkungen: |
Date Completed 30.10.2023 Date Revised 02.11.2023 published: Print-Electronic Citation Status MEDLINE |
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| doi: |
10.1002/cncr.34964 |
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| 100 | 1 | |a Palmerini, Emanuela |e verfasserin |4 aut | |
| 245 | 1 | 0 | |a Outcome of rare primary malignant bone sarcoma treated with multimodal therapy |b Results from the EUROpean Bone Over 40 Sarcoma Study (EURO-B.O.S.S.) |
| 264 | 1 | |c 2023 | |
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| 500 | |a Date Completed 30.10.2023 | ||
| 500 | |a Date Revised 02.11.2023 | ||
| 500 | |a published: Print-Electronic | ||
| 500 | |a Citation Status MEDLINE | ||
| 520 | |a © 2023 The Authors. Cancer published by Wiley Periodicals LLC on behalf of American Cancer Society. | ||
| 520 | |a BACKGROUND: Rare primary malignant bone sarcomas (RPMBS) account for 5%-10% of primary high-grade bone tumors and represent a major treatment challenge. The outcome of patients with RPMBS enrolled in the EUROpean Bone Over 40 Sarcoma Study (EURO-B.O.S.S) is presented | ||
| 520 | |a METHODS: Inclusion criteria were as follows: age from 41 to 65 years and a diagnosis of high-grade spindle cell, pleomorphic, or vascular RPMBS. The chemotherapy regimen included doxorubicin 60 mg/m2 , ifosfamide 9 g/m2 , and cisplatin 90 mg/m2 ; postoperative methotrexate 8 g/m2 was added in case of a poor histologic response. Version 2.0 of the Common Terminology Criteria for Adverse Events, Kaplan-Meier curves, log-rank tests, and univariate Cox regression models were used | ||
| 520 | |a RESULTS: In total, 113 patients were evaluable for analysis. The median patient age was 52 years (range, 40-66 years), and 67 patients were men. Eighty-eight tumors were categorized as undifferentiated pleomorphic sarcomas (UPS), 20 were categorized as leiomyosarcomas, three were categorized as fibrosarcomas, and two were categorized as angiosarcomas. Eighty-three of 113 tumors were located in the extremities. Ninety-five of 113 patients presented with no evidence of metastases. After a median follow-up of 6.8 years (interquartile range [IQR], 3.5-9.8 years), the 5-year overall survival rate for patients with localized disease was 68.4% (IQR, 56.9%-77.5%), and it was 71.7% (IQR, 58.1%-81.6%) for patients with UPS and 54.9% (IQR, 29.5%-74.5%) for patients with leiomyosarcoma. Grade III-IV hematologic toxicity was reported in 81% patients; 23% had grade II-III neurotoxicity, and 37.5% had grade I-II nephrotoxicity. Five-year overall survival was significantly better for patients with localized disease, for patients who obtained surgical complete remission, and when the primary tumor was located in the extremities | ||
| 520 | |a CONCLUSIONS: The survival of patients who had RPMBS in the current series was similar to that of age-matched patients who had high-grade osteosarcoma treated according to the same protocol. An osteosarcoma-like chemotherapy may be proposed in patients who have RPMBS | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Research Support, Non-U.S. Gov't | |
| 650 | 4 | |a angiosarcoma | |
| 650 | 4 | |a bone sarcoma | |
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| 650 | 4 | |a fibrosarcoma | |
| 650 | 4 | |a leiomyosarcoma | |
| 650 | 4 | |a multimodality treatment | |
| 650 | 4 | |a nonosteosarcoma malignant bone tumors | |
| 650 | 4 | |a rare primary malignant bone sarcoma (RPMBS) | |
| 650 | 4 | |a ultra-rare sarcoma | |
| 650 | 4 | |a undifferentiated pleomorphic sarcoma | |
| 650 | 7 | |a Doxorubicin |2 NLM | |
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| 650 | 7 | |a Ifosfamide |2 NLM | |
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| 700 | 1 | |a Reichardt, Peter |e verfasserin |4 aut | |
| 700 | 1 | |a Hall, Kirsten Sundby |e verfasserin |4 aut | |
| 700 | 1 | |a Bertulli, Rossella |e verfasserin |4 aut | |
| 700 | 1 | |a Bielack, Stefan S |e verfasserin |4 aut | |
| 700 | 1 | |a Comandone, Alessandro |e verfasserin |4 aut | |
| 700 | 1 | |a Egerer, Gerlinde |e verfasserin |4 aut | |
| 700 | 1 | |a Hansmeier, Anna |e verfasserin |4 aut | |
| 700 | 1 | |a Kevric, Matthias |e verfasserin |4 aut | |
| 700 | 1 | |a Carretta, Elisa |e verfasserin |4 aut | |
| 700 | 1 | |a Hansson, Lina |e verfasserin |4 aut | |
| 700 | 1 | |a Jebsen, Nina |e verfasserin |4 aut | |
| 700 | 1 | |a Eriksson, Mikael |e verfasserin |4 aut | |
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| 700 | 1 | |a Donati, Davide Maria |e verfasserin |4 aut | |
| 700 | 1 | |a Ibrahim, Toni |e verfasserin |4 aut | |
| 700 | 1 | |a Smeland, Sigbjørn |e verfasserin |4 aut | |
| 700 | 1 | |a Ferrari, Stefano |e verfasserin |4 aut | |
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