Guideline sources The following summarized guidelines for the evaluation and management of chordoma are prepared by our editorial team based on guidelines from the National Comprehensive Cancer Network (NCCN 2021) and the European Reference Network on Genetic Tumour Risk Syndromes (GENTURIS/ERN PaedCan/EURACAN/ESMO 2021). 1 2 Calculator TNM classification for bone can Guidelines 1. Diagnostic investigations History and physical examination: https://web.pathway.md/diseases/recigk2H522yLpOQM 1/6 6/29/23, 3:51 AM Chordoma Pathway As per ESMO 2021 guidelines, elicit medical history and perform physical examination in the initial work-up of patients with suspected primary bone sarcoma. B As per NCCN 2021 guidelines, elicit history and perform physical examination in the initial evaluation of patients with chordoma. B Imaging for staging: As per ESMO 2021 guidelines: Obtain radiological assessment in the initial work-up of patients with suspected primary bone sarcoma. B Obtain the following imaging modalities as clinically indicated for general staging to assess the extent of distant disease, to detect lung, bone and bone marrow metastases: chest CT bone scintigraphy whole body-MRI 18F-FDG positron emission tomography-CT/MRI. B As per NCCN 2021 guidelines: Obtain the following imaging studies in patients with chordoma: adequate imaging of primary site (such as X-ray, MRI with or without CT) screening imaging of spinal axis (MRI/CT with contrast) chest/abdominal/pelvic CT with contrast. B Consider obtaining positron emission tomography/CT from skull base to mid-thigh in patients with chordoma. Consider obtaining bone scan if positron emission tomography/CT is negative. C 2. Diagnostic procedures Biopsy and histopathology: perform biopsy in the initial work-op of patients with suspected primary bone sarcoma. B Show 4 more 3. Medical management Setting of care: As per ESMO 2021 guidelines, manage patients with bone sarcomas at reference centers and/or within reference networks able to provide access to the full spectrum of care and age- specific expertise. B As per NCCN 2021 guidelines, evaluate and manage all patients with chordoma by a multidisciplinary team with expertise in the management of chordoma. B Chemotherapy: Consider offering the following regimens in patients with chordoma: https://web.pathway.md/diseases/recigk2H522yLpOQM 2/6 6/29/23, 3:51 AM Chordoma Pathway imatinib sasatinib sunitinib. C Consider offering the following regimens in selected patients with chordoma: imatinib with cisplatin or sirolumus erlotinib lapatinib for EGFR-positive chordomas sorafenib. C Management of metastatic disease: Consider offering the following options in selected patients with oligometastatic disease: surgery radiofrequency ablation cryotherapy stereotactic radiotherapy. C Do not offer chemotherapy in patients with oligometastatic disease. D 4. Therapeutic procedures Radiotherapy: As per ESMO 2021 guidelines, offer definitive radiotherapy (without debulking) in the following cases: inoperable patients R0 or R1 resection cannot be achieved according to an expert center neurological impairment not accepted by the patient. B Show 2 more As per NCCN 2021 guidelines: Consider offering radiotherapy as the primary treatment in patients with unresectable conventional or chondroid chordomas. C Consider offering radiotherapy for positive surgical margins or for large extra compartmental tumors. C 5. Surgical interventions Surgical excision: As per ESMO 2021 guidelines, consider performing surgery in patients with a chordoma arising from S4 and below or in the context of other alternatives for tumors originating above S3. C Show 3 more As per NCCN 2021 guidelines: https://web.pathway.md/diseases/recigk2H522yLpOQM 3/6 6/29/23, 3:51 AM Chordoma Pathway Perform wide resection, if resectable, with or without radiotherapy as the primary treatment in patients with conventional or chondroid chordoma in the sacrococcygeal region or in the mobile spine. B Perform intralesional excision, if resectable, with or without radiotherapy as the primary treatment in patients with conventional or chondroid chordoma in the skull base or clival region. Obtain follow-up MRI with contrast of primary site to assess adequacy of resection. Consider performing re-resection if necessary. B 6. Follow-up and surveillance Surveillance: As per ESMO 2021 guidelines, consider including the following in the follow-up of patients with high-grade bone sarcomas: physical examination cross-sectional imaging plain radiograph of the primary site CXR/chest CT. C Show 2 more As per NCCN 2021 guidelines, include the following in the surveillance of patients with chordomas: physical examination imaging of surgical site, timing, and modality, as clinically indicated (X-ray, MRI with contrast with or without CT with contrast) for up to 10 years chest imaging every 6 months, consider obtaining CT annually for 5 years, then annually thereafter. B Management of recurrent disease: As per ESMO 2021 guidelines, offer the following options in patients with a relapse: surgery radiotherapy systemic therapies. B As per NCCN 2021 guidelines: Offer the following options in patients with local recurrence: surgical excision radiotherapy systemic therapy. B Offer the following options in patients with metastatic recurrence: systemic therapy surgical excision radiotherapy https://web.pathway.md/diseases/recigk2H522yLpOQM 4/6 6/29/23, 3:51 AM Chordoma Pathway best supportive care. B Clinical findings Symptoms Past medical history Anosmia Diabetes inspidus Back pain Hypopituitarism Blurred vision Dysphagia Fecal incontinence Gait disturbance Headache Hearing loss Occipital headache Retro-orbital pain Shortness of breath Urinary incontinence Vertigo Vision loss Neurological exam Ataxia Dysarthria Dysmetria Dysphonia Facial nerve palsy Hemiparesis Ptosis Radiculopathy Tetraparesis Visual field defect References 1. J Sybil Biermann, Angela Hirbe, Warren Chow et al. Bone Cancer, Version 2.2022, NCCN Clinical Practice Guidelines in Oncology. NCCN. 2021 Oct 8. Open 2. S J Strauss, A M Frezza, N Abecassis et al. Bone sarcomas: ESMO EURACAN GENTURIS ERN PaedCan Clinical Practice Guideline for diagnosis, treatment and follow-up. Ann Oncol. 2021 Dec;32 12 1520 1536. Open 3. Andr s Redondo, Silvia Bagu , Daniel Bernabeu et al. Malignant bone tumors (other than Ewing's): clinical practice guidelines for diagnosis, treatment and follow-up by Spanish Group for Research on https://web.pathway.md/diseases/recigk2H522yLpOQM 5/6 6/29/23, 3:51 AM Chordoma Pathway Sarcomas GEIS . Cancer Chemother Pharmacol. 2017 Dec;80 6 1113 1131. Open https://web.pathway.md/diseases/recigk2H522yLpOQM 6/6