From the Guidelines
Chondrosarcoma is a type of bone cancer that develops from cartilage cells, typically affecting adults over 40 years old, with the most common primary sites being the long bones, pelvis, and ribs 1.
Definition and Characteristics
Chondrosarcoma becomes more frequent with increasing age, with approximately 80% of cases diagnosed after 40 years 1. The majority of conventional chondrosarcomas are locally aggressive or low-grade, non-metastasising tumours, rather than high-grade chondrosarcoma 1.
Diagnosis and Treatment
The diagnosis of chondrosarcoma is based on morphology, and approximately 50% of central chondrosarcomas carry IDH1 or IDH2 mutations 1. Treatment primarily involves surgical removal of the tumor, as chondrosarcomas generally don't respond well to chemotherapy or radiation therapy 1.
Prognosis and Follow-up
The prognosis depends on several factors including the grade of the tumor, location, size, and whether it has spread 1. Higher-grade tumors have a worse prognosis than lower-grade ones. Regular follow-up care is essential after treatment to monitor for recurrence, which can happen even years later 1.
- Key factors that influence treatment and prognosis include:
- Metastatic disease at presentation
- Histological grade
- Axial primary site
- Size of the tumor
- Surgical removal with wide margins is the recommended treatment for higher-grade chondrosarcomas and all chondrosarcomas of the pelvis or axial skeleton 1.
From the Research
Definition and Characteristics of Chondrosarcoma
- Chondrosarcomas (CHS) represent a heterogeneous group of disorders ranging from indolent, low-grade tumors to aggressive, high-grade forms 2.
- Chondrosarcomas are a diverse group of malignant cartilaginous matrix-producing neoplasms, with conventional chondrosarcomas being a continuum of disease based on the biologic activity of the tumor 3.
- The tumors range from relatively biologically benign low-grade tumors or intermediate atypical cartilaginous tumors (ACTs), to malignant, aggressive high-grade tumors 3.
Clinical Presentation and Diagnosis
- Chondrosarcomas present a diagnostic dilemma, particularly in the differentiation between high- and intermediate-grade tumors and that of low-grade tumors from benign enchondromas 3.
- The clinical presentation, radiographic and pathologic findings, treatments and outcomes vary significantly based on the histologic grade of the tumor 3.
Treatment Options
- Surgical resection represents the primary and preferred treatment modality for individuals with localized disease 2.
- Radiation therapy is appropriate for the treatment of positive surgical margins or palliation of disease-related symptoms 2.
- Systemic chemotherapy may be considered in variant forms such as mesenchymal or dedifferentiated chondrosarcomas, but high-quality data supporting its use is limited 2.
- Advanced radiation therapies, such as high-dose and conventional RT modalities, may present a treatment option for certain patients, particularly those with positive surgical margins or tumors in surgically challenging locations 4.
- Selective agonists of nuclear retinoic acid receptor gamma, such as palovarotene, may have an anti-tumor effect on low-grade chondrosarcomas 5.