Management of bone sarcoma: ESMO-EURACAN-GENTURIS-ERNPaedCan Guideline - Page 2
RCS with EWSR1 non-ETS fusions, CIC-rearranged sarcoma and sarcoma with BCOR alterations
· There is no consensus on whether they should be treated with an ES-like approach or regarded as high-grade STS. Combination regimens including anthracycline and alkylating agents should be favored when feasible. Registration within clinical trials and prospective registries is recommended.
Chondrosarcoma
· Atypical cartilaginous tumours can be managed by curettage with or without local adjuvant therapy.
·High-grade chondrosarcomas and all chondrosarcomas of the pelvis or axial skeleton should be surgically excised with wide margins.
· RT can be considered for unresectable disease (primary or recurrent), after incomplete surgery and for symptoms palliation.
· High-dose RT is currently recommended for skull base chondrosarcomas.
· Localised MCSs are usually treated with neoadjuvant/adjuvant ChT combining anthracycline and alkylating agents.
· Neoadjuvant/adjuvant ChT can also be considered for localised DCS.
Chordoma
· The assessment of brachyury nuclear expression in conventional chordoma is highly recommended to confirm diagnosis.
· Surgery should be offered if the chordoma arises from S4 and below or discussed in the context of other alternatives for tumours originating above S3.
· R1–R2 surgery plus high-dose RT is the treatment of choice for skull base and upper cervical tract chordoma.
· Indications for definitive RT include disease for which R0 or R1 resection cannot be achieved according to an expert centre, inoperable patients and neurological impairment not accepted by the patient.
· For relapse, treatment includes surgery and/or RT and/or systemic therapies.
Giant cell tumor of bone
· Treatment options for GCTB include en-bloc excision [IV, A] and intralesional curettage with or without adjuvant therapy in carefully selected cases.
· Denosumab is standard treatment in unresectable or metastatic GCTB.
· Denosumab use in the preoperative setting for GCTB that are potentially resectable with high morbidity is debated and should be individualised and reserved for complex cases following multidisciplinary discussion.
Follow-up, long-term implications and survivorship
· Follow-up of high-grade BS could include physical examination, cross sectional imaging and plain radiograph of the primary site together with chest X-ray/CT scan.
· A recommended follow-up may foresee intervals of approximately every 3 months for the first 2 years; every 6 months for years 3-5; every 6-12 months for years 5-10, and thereafter every 0.5-1-2 years.
· For low-grade BS, the frequency of follow-up visits may be lower (e.g. 6 months for 2 years and then annually).
· Long-term toxic effects of ChT, surgery and RT should be evaluated, and monitoring for late effects should be continued for >10 years after treatment, depending on the protocol used.
Reference:
Bone sarcomas: ESMO–EURACAN–GENTURIS–ERNPaedCan Clinical Practice Guideline for diagnosis, treatment and follow-up, published in the Annals of Oncology.
DOI: https://www.annalsofoncology.org/article/S0923-7534(21)04280-0/fulltext
Source : Annals of Oncology
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