NCCN Clinical Practice Guidelines for Treatment of Chondrosarcoma
NCCN recommendations for treatment of chondrosarcoma are as follows [25] :
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Enrollment in a clinical trial should be considered when available; in addition, whenever possible patients should be referred to a tertiary care center with expertise in sarcoma, for treatment by a multidisciplinary team
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Wide excision or intralesional excision with or without adjuvant therapy for resectable low-grade and intracompartmental lesions
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Wide excision is preferred for pelvic low-grade tumors
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High-grade (grade II, III), clear cell, or extracompartmental lesions, if resectable, should be treated with wide excision
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Wide excision should provide negative surgical margins and may be achieved by either limb-sparing surgery or amputation
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Postoperative treatment with proton and/or photon beam radiation for tumors in unfavorable location
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Consider radiation therapy for unresectable tumors
No established chemotherapy regimens exist for grade I-III tumors; treatment of patients with dedifferentiated chondrosarcoma should follow osteosarcoma guidelines; treatment of patients with mesenchymal tumor should follow Ewing sarcoma guidelines; dasatinib is an option. Standard sarcoma chemotherapy agents can be tried to palliate metastatic disease.
NCCN recommendations for treatment of local recurrence of chondrosarcoma are as follows [25] :
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Wide excision if resectable
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Consider radiation therapy or re-resection to achieve negative surgical margins following wide excision with positive margins
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Radiation therapy for unresectable recurrences
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Surgical excision or participation in a clinical trial for patients with systemic recurrence of a high-grade chondrosarcoma
ESMO-PaedCan-EURACAN Clinical Practice Guidelines for Treatment of Chondrosarcoma
European Society for Medical Oncology, European Reference Network for Paediatric Cancers, and European Network for Rare Adult Solid Cancer (ESMO-PaedCan-EURACAN) guidelines recommend that low-grade central chondrosarcomas in the long bones of the limbs be managed by curettage, with or without adjuvant therapy (eg, phenol, cement, cryotherapy) and low-grade peripheral chondrosarcomas be surgically excised. [26] Higher-grade chondrosarcomas and all chondrosarcomas of the pelvis or axial skeleton should be surgically excised with wide margins. For dedifferentiated chondrosarcoma, if wide margins cannot be achieved with limb salvage, consideration of amputation is recommended.
The ESMO-PaedCan-EURACAN guidelines are generally in agreement with NCCN regarding chemotherapy regimens for mesenchymal chondrosarcoma and also note the following [26] :
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Dedifferentiated chondrosarcoma is often treated as a high-grade bone sarcoma, with systemic and local therapies that need to be adapted to the patient’s age
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Skull-base chondrosarcomas can be treated with high-dose radiation therapy, including proton or carbon ion beam radiation therapy
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Doxorubicin and ifosfamide may prove active in chondrosarcoma, especially in high-grade lesions, and gemcitabine in combination with docetaxel has also been reported to be effective
SELNET Guidelines for Treatment of Chondrosarcoma
Sarcoma European Latin-American Network (SELNET) guidelines state that atypical cartilaginous tumors/grade I chondrosarcoma of long bones can be managed with intralesional extended curettage (large cortical window, high-speed burr) with or without local adjuvant therapy (eg, phenol or cryotherapy) and reconstruction with cement or bone graft. [27]
Grade II and III chondrosarcoma, as well as chondrosarcomas of any grade that are located in the pelvis or axial skeleton, should be surgically excised with wide margins.
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Plain radiograph shows low-grade chondrosarcoma in pelvis (B). Incidental finding is that proximal femur contains benign enchondroma (A).
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T1-weighted MRI shows low-signal-intensity lesion in pelvis: chondrosarcoma.
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T2-weighted MRI shows high-signal-intensity lesion in pubis: chondrosarcoma.
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MRI of chondrosarcoma (B) shows contrast enhancement of lesion. Enchondroma (A) is also present.
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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 |
