Guideline sources The following summarized guidelines for the evaluation and management of giant cell tumor of bone (GCTB) 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/recNwQ8EKBfCQAmg3 1/5 7/1/23, 12:02 AM y Giant cell tumor of bone Pathway p y 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 GCTB. 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 GCTB: imaging of primary site (such as X-ray, MRI with contrast with or without CT) chest imaging. B Consider obtaining bone scan in patients with GCTB. C 2. Diagnostic procedures Biopsy and histopathology: As per ESMO 2021 guidelines, perform biopsy in the initial work-op of patients with suspected primary bone sarcoma. B Show 2 more As per NCCN 2021 guidelines, perform biopsy to confirm the diagnosis of GCTB. B 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 Denosumab: As per ESMO 2021 guidelines, initiate denosumab as standard treatment in patients with unresectable or metastatic GCTB. B Show 3 more As per NCCN 2021 guidelines: https://web.pathway.md/diseases/recNwQ8EKBfCQAmg3 2/5 7/1/23, 12:02 AM Giant cell tumor of bone Pathway Initiate denosumab as a preferred treatment option in patients with localized disease, if resectable with unacceptable morbidity or with unresectable axial lesions. B Consider offering denosumab as a treatment option in patients with unresectable metastatic disease at presentation. C Interferon alpha-2b: Offer interferon alpha-2b as a treatment option in patients with localized disease, if resectable with unacceptable morbidity or with unresectable axial lesions. B Consider offering interferon alpha-2b as a treatment option in patients with unresectable metastatic disease at presentation. C 4. Therapeutic procedures Serial arterial embolization: perform serial arterial embolization as a preferred treatment option in patients with localized disease, if resectable with unacceptable morbidity or with unresectable axial lesions. B Radiotherapy: Offer radiotherapy as a treatment option in patients with localized disease, if resectable with unacceptable morbidity or with unresectable axial lesions. B Consider offering radiotherapy as a treatment option in patients with unresectable metastatic disease at presentation. C 5. Surgical interventions Surgical excision: As per ESMO 2021 guidelines, perform en bloc excision B or intralesional curettage with or without adjuvant therapy in selected patients with GCTB. B As per NCCN 2021 guidelines: Perform excision in patients with localized GCTB, if resectable. B Perform excision of primary tumor in patients with metastatic disease at presentation, if resectable. Consider resecting metastatic sites. B 6. Specific circumstances Patients with malignant transformation: manage patients with malignant transformation as patients with osteosarcoma. B 7. Follow-up and surveillance https://web.pathway.md/diseases/recNwQ8EKBfCQAmg3 3/5 7/1/23, 12:02 AM Giant cell tumor of bone Pathway Assessment of treatment response: obtain follow-up imaging, plain radiographs and CT with or without MRI (both with contrast), to assess treatment response. B Show 2 more 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 GCTB: physical examination imaging of surgical site as clinically indicated (X-ray, MRI with contrast with or without CT with contrast) chest imaging every 6-12 months for 4 years, then annually thereafter. B Management of recurrent disease: consider obtaining chest imaging in patients with resectable local recurrence. C Show 3 more Clinical findings Symptoms Past medical history Joint swelling Lung metastasis Localized bone pain Pathological fracture Localized bone swelling Imaging findings Musculoskeletal exam Osteolytic lesion knee ROM spinal ROM wrist ROM 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 https://web.pathway.md/diseases/recNwQ8EKBfCQAmg3 4/5 7/1/23, 12:02 AM Giant cell tumor of bone Pathway 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 Sarcomas GEIS . Cancer Chemother Pharmacol. 2017 Dec;80 6 1113 1131. Open https://web.pathway.md/diseases/recNwQ8EKBfCQAmg3 5/5