Approach Considerations
Guidelines for treatment of chondrosarcoma have been developed by the National Comprehensive Cancer Network (NCCN) [25] ; jointly by the European Society for Medical Oncology (ESMO), the European Reference Network for Paediatric Cancers (PaedCan), and the European Network for Rare Adult Solid Cancer (EURACAN) [26] ; and by the Sarcoma European Latin-American Network (SELNET). [27] (See Guidelines.) For further information, see Bone Sarcoma Guidelines.
Radiotherapy and Chemotherapy
Radiotherapy and chemotherapy play limited roles in primary treatment. An exception is their use as adjuvant therapy or palliative treatment in surgically inaccessible areas.
Diffuse metastasis is usually an indication for systemic radiotherapy or chemotherapy. The results are generally poor, as they are in dedifferentiated chondrosarcoma, for which the 1-year survival rate is 10%. [14]
Chemotherapy and radiotherapy may be used in dedifferentiated chondrosarcoma because distant systemic metastasis may be present at the time of diagnosis. However, current evidence indicates that surgery with clear margins remain the primary treatment for dedifferentiated chondrosarcomas as well. Grimer et al recommended further use of chemotherapy in such cases only as a trial or treatment protocol. [28]
Surgical Therapy
Surgery is the primary treatment for any chondrosarcoma. Complete, wide surgical excision of the chondrosarcoma is the preferred method when it is feasible. Success depends on the stage of the disease, with low-grade intracompartmental lesions offering the best prognosis after complete surgical resection with surgically clear margins. Lesions with isolated pulmonary metastasis can still be surgically resected if metastasectomy of the isolated pulmonary lesion is feasible.
Chondrosarcomas in the appendicular skeleton are amenable to wide excision. Hence, these lesions tend to fare better with surgery than those occurring in the axial skeleton. Complete removal of most lesions in the axial skeleton is difficult. Complete en-bloc excision can cure clear cell chondrosarcomas. Surgery remains the primary treatment for mesenchymal chondrosarcomas as well.
A retrospective study by Park et al suggested that intralesional curettage with cryotherapy appears to be a safe and reasonable surgical option for patients with low-grade chondrosarcoma lesions confined to bone (Enneking stage I-A). [29]
Long-Term Monitoring
Regular follow-up is required to rule out local recurrences of chondrosarcoma and distant metastases. Regular follow-up clinical evaluations and radiologic investigations are required.
-
Plain radiograph shows low-grade chondrosarcoma in pelvis (B). Incidental finding is that proximal femur contains benign enchondroma (A).
-
T1-weighted MRI shows low-signal-intensity lesion in pelvis: chondrosarcoma.
-
T2-weighted MRI shows high-signal-intensity lesion in pubis: chondrosarcoma.
-
MRI of chondrosarcoma (B) shows contrast enhancement of lesion. Enchondroma (A) is also present.
-
Plain radiograph shows chondrosarcoma.
Tables
Chondrosarcoma |
Male-to-Female Ratio |
Age of Peak Incidence |
Conventional |
Almost 1:1 (slight male predominance) |
50-70 y (most common >50 y, gradual increase with age) |
Dedifferentiated |
Similar to the ratio above |
>50 y |
Clear cell |
2.4:1 |
20-40 y (common 10-90 y) |
Mesenchymal |
1:1 |
20-30 y (common in teenagers and young adults) |
Juxtacortical |
1:1 |
20-40 y |
