Management of bone sarcoma: ESMO-EURACAN-GENTURIS-ERNPaedCan Guideline
Delhi: A recent clinical practice guideline published in the journal Annals of Oncology provides key recommendations on the management of bone sarcomas. The recommendations were agreed upon following a consensus meeting of representatives from ESMO, EURACAN, GENTURIS, and ERNPaedCan.
Primary bone sarcomas (BSs) constitute <0.2% of malignant neoplasms across all ages. The overall incidence rate lies between 0.8-0.9 cases per 100,000/year with single BS types having no more than 0.3 incident cases per 100,000/year. Osteosarcoma and Ewing sarcoma (ES) have a relatively high incidence in the second decade of life, whereas conventional chondrosarcomas are more common in older age.
Diagnosis, pathology and molecular biology
- The initial work-up of a suspected primary BS tumour should be carried out at a sarcoma reference centre, and should include medical history, physical examination, radiological assessment and biopsy.
- Pathological diagnosis should be made by a bone tumour expert dedicated pathologist according to the 2020 WHO classification and should be supported by ancillary investigations whenever relevant.
- For surgical specimens, tumour size and local extent of spread, site, status of surgical margins and percentage of pathological response to preoperative ChT should be described.
Staging and risk assessment
- General staging should be carried out to assess the extent of distant disease, including chest CT, bone scintigraphy and/or WB-MRI and and/or FDG-PET-CT/MRI as clinically indicated. Baseline serum analysis in ES and osteosarcoma should include AP and LDH.
- Low-grade central and parosteal osteosarcoma are malignancies with a low metastatic potential that should be treated by surgery alone.
- urative treatment of high-grade osteosarcoma consists of multimodal ChT and surgery.
- oxorubicin, cisplatin, HD-MTX and ifosfamide have anti-tumour activity in osteosarcoma [I, A]. In patients >40 years, preferred regimens combine doxorubicin, cisplatin and ifosfamide.
- igh-grade craniofacial osteosarcoma should be treated the same way as high-grade osteosarcoma of other sites [IV, B]. In this location, RT can be proposed when complete surgery is not feasible and in patients undergoing resection with positive margins.
- eavy-particle RT and IMRT can be considered, particularly for unresectable primary tumours.
- rimary metastatic osteosarcoma patients are treated with a curative intent following the same principles of non-metastatic osteosarcomas.
- he treatment of recurrent osteosarcoma is primarily surgical in the case of isolated lung metastases or local recurrence.
- RFA and stereotactic RT are potential alternative local treatment options in patients unfit for surgery and for small lung or bone metastases.
- Second-line ChT for recurrent osteosarcoma includes ifosfamide or cyclophosphamide, possibly in association with etoposide and/or carboplatin [III, B], and other active drugs including gemcitabine and docetaxel.
- Molecular confirmation is mandatory for the distinction between ES and other RCSs.
- The interval-compressed VDC/IE regimen is currently the preferred first-line treatment in ES.
- The use of BuMel could be considered for selected patients with poor response to VIDE induction ChT and/or tumour volume >200 ml.
- The role of high-dose ChT has not been evaluated with interval compressed VDC/IE. The selection of the most appropriate consolidation should take into account the ChT regimen received and the need for RT.
- Complete surgical excision, when feasible, rather than RT as a sole modality is regarded as the best modality of local tumour control.
- RT alone should be used if complete surgical excision is not possible and in primary sites where surgery will lead to unacceptable morbidity.
- Adjuvant RT (pre- or post-operative) is indicated where the originally involved tissues cannot be completely resected with adequate surgical margins, for large-volume tumors or poor histological response [IV, B]. It should be considered in patients with non-sacral pelvic ES regardless of surgical margins, tumour volume or histological response.
- Treatment of patients with extraskeletal ES follows the same principles as bone ES.
- For cutaneous/subcutaneous ES the number of ChT cycles should be discussed on an individual case basis.
- For patients with metastases at diagnosis, ChT is similar to that for localised disease.
- ChT regimens for relapsed disease include alkylating agents in combination with topoisomerase inhibitors, irinotecan with temozolomide, gemcitabine and docetaxel, high-dose ifosfamide or carboplatin with etoposide.
Source : Annals of Oncology