Chondrosarcoma is a type of cancer that forms in bone cartilage. The most frequent primary sites are the pelvis, shoulder, ribs, or at the ends of the arms and legs. Chondrosarcoma can occur at any age but is more common in people older than 40 years.Mesenchymal chondrosarcoma is aggressive variant of chondrosarcoma, which tend to occur in younger people. Extraskeletal chondrosarcoma is a rare type of chondrosarcomadoes, which forms in the soft tissues, rather than bone cartilage.
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MeSH term: Chondrosarcoma
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Novel therapeutic approaches in chondrosarcoma.
Future Oncol. 2017; 13(7):637-648 [PubMed] Related Publications
C1q/TNF-related protein-3 exerts the chondroprotective effects in IL-1β-treated SW1353 cells by regulating the FGFR1 signaling.
Biomed Pharmacother. 2017; 85:41-46 [PubMed] Related Publications
MRI appearances of atypical cartilaginous tumour/grade I chondrosarcoma after treatment by curettage, phenolisation and allografting: recommendations for follow-up.
Bone Joint J. 2016; 98-B(12):1674-1681 [PubMed] Related Publications
PATIENTS AND METHODS: We reviewed the case notes, radiology and histology of 75 patients, who had been treated for an ACT/grade I chondrosarcoma by curettage, phenolisation and bone allografting between 1994 and 2005. The first post-operative Gd-enhanced MRI scan was carried out within one year of surgery. Patients had a minimum of two scans and a mean follow-up of 72 months (13 to 169). Further surgery was undertaken in cases of suspected recurrence.
RESULTS: In 14 patients (18.6%) a second procedure was undertaken after a mean period of 59 months (8 to 114). Radio frequency ablation (RFA) was used in lesions of < 10 mm and curettage, phenolisation and bone grafting for those ≥ 10 mm. Only six of these (8% of total) had a histologically-proven recurrence. No increase in tumour grade was seen at time of recurrence.
CONCLUSION: Based on this study, we have been able to classify the post-operative MRI appearances into four groups. These groups differ in follow-up, and have a different risk of recurrence of the lesion. Follow-up and treatment vary for the patients in each group. We present a flow diagram for the appropriate and safe follow-up for this specific group of patients. Cite this article: Bone Joint J 2016;98-B:1674-81.
Clinical outcomes for patients after surgery and radiation therapy for mesenchymal chondrosarcomas.
J Surg Oncol. 2016; 114(8):982-986 [PubMed] Related Publications
PATIENTS AND METHODS: Twenty-three patients with mesenchymal chondrosarcomas were treated with surgery and radiation therapy (min. dose 44 Gy; max. dose 78 Gy; median dose 60 Gy; mean dose 61 Gy).
RESULTS: The median survival for the entire cohort of patients was 21.65 years (95% confidence interval ± 4.25). The 5- and 10-year OS was 78.6%. Median disease-free survival for the 23 patients was 7.2 years. Disease-free survival (DFS) at 3 and 5 years was 70.7% and 57.8%, respectively. The local control rate at 5 and 10 years was 89.5% (95%CI 64.1-97.3%). Only three patients experienced local failure, three patients had regional failure, and eight developed distant metastases.
CONCLUSIONS: In this cohort of patients local tumor control was high when using a combination of surgery and radiation. There was not a clear relationship between radiation dose and local tumor control. J. Surg. Oncol. 2016;114:982-986. © 2016 Wiley Periodicals, Inc.
Chordomas and chondrosarcomas-The role of radiation therapy.
J Surg Oncol. 2016; 114(5):564-569 [PubMed] Related Publications
Chondrosarcoma of the para-acetabulum: correlation of imaging features with histopathological grade.
Radiol Med. 2016; 121(12):897-904 [PubMed] Related Publications
MATERIALS AND METHODS: Thirty-one patients with histopathologically confirmed central para-acetabular CSs (6 LGCS and 25 HGCS) were retrospectively reviewed. Image features were evaluated for the following: cortical destruction, tumor border and pattern, calcification mode, soft-tissue mass, density/signal intensity, peritumoral edema, acetabular (cartilage) destruction, diffuse signal changes in acetabulum, mass inside hip joint, femoral head involvement, enhancement manifestations and the maximum length of the tumor. These image features between LGCS and HGCS were also assessed.
RESULTS: The most common CT and/or MR findings included cortical destruction, punctate, ring-and-arc and linear calcification, soft-tissue mass, lobulated border, high signal intensity with low signal septa on T2-weighted image, peritumoral edema, hip joint infiltration, peripheral and septal enhancement on post-enhanced MR image. Statistical analysis showed that the image features, such as cortical destruction, soft-tissue mass, hip joint infiltration and tumor size were significantly different between LGCS and HGCS (p < 0.05).
CONCLUSION: The characteristic radiological features of para-acetabular CSs are osteolytic lesions with cortical destruction, soft-tissue mass, lobulated border, calcification, and high signal intensity with low signal septa on T2-weighted MR image, peripheral and septal enhancement on post-enhanced MR image. Cortical destruction, soft-tissue mass, hip joint infiltration and tumor size can differentiate HGCS from LGCS.
TCF-1 participates in the occurrence of dedifferentiated chondrosarcoma.
Tumour Biol. 2016; 37(10):14129-14140 [PubMed] Free Access to Full Article Related Publications
Clinicopathologic and radiologic features of extraskeletal myxoid chondrosarcoma: a retrospective study of 40 Chinese cases with literature review.
Ann Diagn Pathol. 2016; 23:14-20 [PubMed] Related Publications
Management of tracheal chondrosarcoma almost completely obstructing the airway: a case report.
J Cardiothorac Surg. 2016; 11(1):101 [PubMed] Free Access to Full Article Related Publications
CASE PRESENTATION: We present the case of a 79-year old Caucasian man with long-lasting wheezing misdiagnosed as asthma and affected by a tracheal chondrosarcoma almost completely obstructing the airway. Videobronchoscopy and imaging investigations revealed a well-circumscribed mass arising from the cartilaginous rings of the cervical trachea with a posterior residual respiratory space of about 1 mm. Because of the mobility and flaccidity of the uninvolved pars membranacea, the tiny respiratory space slightly expanded during inspiration and expiration allowing the patient to be treated without an essential emergency procedure. Standard tracheal intubation was impossible. Rigid bronchoscopy enabled placement of a small tracheal tube distally to the tumor. Successful cervical tracheal resection and reconstruction was then performed, achieving complete tumor excision. Histologically, the mass was characterized as a low-grade tracheal chondrosarcoma. Videobronchoscopy performed 9 months after surgery showed a wide, well healed tracheal anastomosis. Ten months after surgery, the patient is alive and disease free.
CONCLUSION: Complete surgical resection is the treatment of choice for tracheal chondrosarcoma. Rigid bronchoscopy is an essential tool for diagnostic and therapeutic purposes. It allows the palliative maneuvers for obstruction relief but also, in resectable patients, the intraoperative safe and straightforward management of the obstructed airway.
Recurrent chondrosarcoma of the larynx: A case report and literature review.
Medicine (Baltimore). 2016; 95(27):e4118 [PubMed] Free Access to Full Article Related Publications
CASE SUMMARY: We reported here a case of a 63-year-old male patient with a 3-month history of progressive hoarseness and throat pain. The patient had undergone surgical resection of a laryngeal mass 2 years prior. A supracricoid partial laryngectomy was performed this time. Histological examination supported the diagnosis of low-grade chondrosarcoma. Three years later, the radiological and clinical findings showed no evidence of recurrence.
CONCLUSION: Currently, total laryngectomy is preferred for patients with recurrent low-grade LCS. However, the literature review and our case suggest that a second function-preserving procedure may be a reasonable choice for recurrent LCS.
En Bloc Resection of Primary Malignant Bone Tumor in the Cervical Spine Based on 3-Dimensional Printing Technology.
Orthop Surg. 2016; 8(2):171-8 [PubMed] Related Publications
METHODS: Five patients with primary malignant bone tumors of the cervical spine underwent en bloc resection via a one-stage combined anteroposterior approach in our hospital from March 2013 to June 2014. They comprised three men and two women of mean age 47.2 years (range, 26-67 years). Three of the tumors were chondrosarcomas and two chordomas. Preoperative 3-D printing models were created by 3-D printing technology. Sagittal en bloc resections were planned based on these models and successfully performed. A 360° reconstruction was performed by spinal instrumentation in all cases. Surgical margins, perioperative complications, local control rate and survival rate were assessed.
RESULTS: All patients underwent en bloc excision via a combined posterior and anterior approach in one stage. Mean operative time and estimated blood loss were 465 minutes and 1290 mL, respectively. Mean follow-up was 21 months. Wide surgical margins were achieved in two patients and marginal resection in three; these three patients underwent postoperative adjuvant radiation therapy. One vertebral artery was ligated and sacrificed in each of three patients. Nerve root involved by tumor was sacrificed in three patients with preoperative upper extremity weakness. One patient (Case 3) had significant transient radiculopathy with paresis postoperatively. Another (Case 4) with C 4 and C 5 chordoma had respiratory difficulties and pneumonia after surgery postoperatively. He recovered completely after 2 weeks' management with a tracheotomy tube and antibiotics in the intensive care unit. No cerebrovascular complications and wound infection were observed. No local recurrence or instrumentation failure were detected during follow-up.
CONCLUSION: Though technically challenging, it is feasible and safe to perform en bloc resection of cervical primary bone tumors. This is the most effective means of managing cervical spine tumors. Preoperative 3-D printing modelling enables better anatomical understanding of the relationship between the tumor and cervical spine and can assist in planning the surgical procedure.
LARYNGEAL CHONDROSARCOMA: SUCCESSFUL USE OF VIDEO LARYNGOSCOPE IN ANTICIPATED DIFFICULT AIRWAY MANAGEMENT.
Acta Clin Croat. 2016; 55 Suppl 1:108-11 [PubMed] Related Publications
Multifaceted impact of trichothecene metabolites on plant-microbe interactions and human health.
Appl Microbiol Biotechnol. 2016; 100(13):5759-71 [PubMed] Related Publications
A Prospective Outcomes Study of Proton Therapy for Chordomas and Chondrosarcomas of the Spine.
Int J Radiat Oncol Biol Phys. 2016; 95(1):297-303 [PubMed] Related Publications
METHODS AND MATERIALS: Between March 2007 and May 2013, 51 patients with a median age of 58 years (range, 22-83 years) with chordoma (n=34) or chondrosarcomas (n=17) of the sacrum (n=21), the cervical spine (n=20), and the thoracolumbar spine (n=10) were treated with external beam proton therapy to a median dose of 70.2 Gy(RBE) [range, 64.2-75.6 Gy(RBE)] at our institution. Distant metastases, overall survival, cause-specific survival, local control, and disease-free survival were calculated.
RESULTS: The mean follow-up time was 3.7 years (range, 0.3-7.7 years). Across all time points, 25 patients experienced disease recurrence: 18 local recurrences, 6 local and distant recurrences, and 1 distant metastasis. The 4-year rates of overall survival and cause-specific survival were 72%; disease-free survival was 57%, local control was 58%, and freedom from distant metastases was 86%. The median time to local progression was 1.7 years (range, 0.2-6.0 years), and the median time to distant progression was 1.6 years (range, 0.2-6.0 years). The risk factors for local recurrence were age ≤58 years (62% vs 26%; P=.04) and recurrence after prior surgery (29% vs 81%; P=.01). Secondary cancers developed in 2 patients: B-cell lymphoma 5.5 years after treatment and bladder cancer 2 years after treatment. We observed the following toxicities: sacral soft tissue necrosis requiring surgery (n=2), T1 vertebral fracture requiring fusion surgery (n=1), chronic urinary tract infections (n=1), surgery for necrotic bone cyst (n=1), and grade 2 bilateral radiation nephritis (n=1).
CONCLUSION: High-dose proton therapy controls more than half of spinal chordomas and chondrosarcomas and compares favorably with historic photon data. Local progression is the dominant mode of treatment failure and may be reduced by treating patients at the time of initial diagnosis. The impact of age is a novel finding of this study.
Rapamycin causes growth arrest and inhibition of invasion in human chondrosarcoma cells.
J BUON. 2016 Jan-Feb; 21(1):244-51 [PubMed] Related Publications
METHODS: We determined the effect of rapamycin on cell proliferation, cell cycle arrest and invasion by using MTS, flow cytometry and invasion assays in two human chondrosarcoma cell lines, SW1353 and JJ012. Cell cycle regulatory and invasion-related genes' expression analysis was performed by quantitative RT-PCR (qRT-PCR). We also evaluated the effect of rapamycin on tumor growth by using mice xenograph models.
RESULTS: Rapamycin significantly inhibited the cell proliferation, induced cell cycle arrest and decreased the invasion ability of human chondrosarcoma cells. Meanwhile, rapamycin modulated the cell cycle regulatory and invasion-related genes' expression. Furthermore, the tumor growth of mice xenograph models with human chondrosarcoma cells was significantly inhibited by rapamycin.
CONCLUSIONS: These results provided further insight into the role of rapamycin in chondrosarcoma. Therefore, rapamycin targeted therapy may be a potential treatment strategy for chondrosarcoma.
miRNA-497 Negatively Regulates the Growth and Motility of Chondrosarcoma Cells by Targeting Cdc25A.
Oncol Res. 2016; 23(4):155-63 [PubMed] Related Publications
miR-125b acts as a tumor suppressor in chondrosarcoma cells by the sensitization to doxorubicin through direct targeting the ErbB2-regulated glucose metabolism.
Drug Des Devel Ther. 2016; 10:571-83 [PubMed] Free Access to Full Article Related Publications
Expression of insulin-like growth factor II mRNA binding protein 3 (IMP3) in enchondroma and chondrosarcoma.
Pathol Res Pract. 2016; 212(4):335-9 [PubMed] Related Publications
Primary synovial chondromatosis: a reassessment of malignant potential in 155 cases.
Skeletal Radiol. 2016; 45(6):755-62 [PubMed] Related Publications
MATERIALS AND METHODS: We reviewed 155 cases of PSC and identified 4 cases (3 in the hip joint; 1 in the elbow joint) of aggressive behaviour and chondrosarcoma-like histology.
RESULTS: Radiologically, these cases were all reported as showing features consistent with PSC and aggressive extra-articular soft tissue/bone involvement. Histologically, in addition to typical features of PSC, there was morphological evidence of peri-articular soft tissue and, in 2 cases, bone involvement by an infiltrating cartilaginous tumour. These tumours all behaved as locally aggressive neoplasms and did not give rise to metastasis.
CONCLUSION: Our findings show that chondrosarcoma arises infrequently in PSC (approximately 2.5 %), and that this complication occurs most commonly in the hip joint (approximately 11 % of cases of hip PSC). These tumours behaved mainly as low-grade, locally aggressive tumours analogous to atypical cartilaginous tumour of bone/grade 1 chondrosarcoma of bone.
Local Treatment with Adjuvant Therapy for Central Atypical Cartilaginous Tumors in the Long Bones: Analysis of Outcome and Complications in One Hundred and Eight Patients with a Minimum Follow-up of Two Years.
J Bone Joint Surg Am. 2016; 98(4):303-13 [PubMed] Related Publications
METHODS: A retrospective study was designed to analyze data from 108 patients treated for central ACT/CS1 in the long bones between 2006 and 2012. All patients were treated with curettage and adjuvant phenolization, and defects were filled with polymethylmethacrylate, bone graft, or bone substitutes. The primary end point was local recurrence or residual tumor. Secondary end points included the type and rate of complications and reoperations.
RESULTS: All patients were free from local recurrence at a mean follow-up of 48.7 months (range, 24.3 to 97.5 months). Residual tumor was suspected in five patients, leading to a 95.4% disease-free survival rate. A fracture occurred in eleven patients (10.2%). Other complications were osseous penetration during the surgery (two patients), wound infection (one patient), arthrofibrosis (one patient), and skin necrosis (one patient). Tumor volume was related neither to the risk of fracture nor to the occurrence of residual tumor.
CONCLUSIONS: In our experience, curettage of ACT/CS1 in the long bones with adjuvant phenolization is safe, even with large tumors of up to 100 cm(3). Most worrisome is the risk of fracture, which occurred in 10.2% of our patients. Considering the relatively mild behavior of ACT/CS1, less aggressive treatment, by observation or by minimally invasive surgery, could be the next step that should be evaluated prospectively.
α-methylacyl-CoA racemase (AMACR) expression in chordomas differentiates them from chondrosarcomas.
Sci Rep. 2016; 6:21277 [PubMed] Free Access to Full Article Related Publications
METHODS: Archival sections of 18 chordomas, 19 chondrosarcomas and 10 mature cartilage samples were immunostained and scored for AMACR, β-catenin and E-cadherin and the relative differential capacity of each marker was calculated. In addition, AMACR mRNA level was assessed in 5 chordomas by RT-PCR and evaluated by comparative CT method.
RESULTS: AMACR and β-catenin stained 88.9% and 94.1% of the chordomas respectively, 21.1% and 10.5% of the chondrosarcomas correspondingly and none of the mature cartilage samples. E-cadherin stained positively 82.4% of the chordomas, 36.8% of the chondrosarcomas and 42.9% of the mature cartilage cases. Both AMACR and β-catenin showed statistically significant difference between chordomas and chondrosarcomas (p < 0.001 for both), unlike E-cadherin. AMACR was detected at the mRNA level.
CONCLUSIONS: AMACR is expressed in most of the chordomas but only in a minority of chondrosarcomas. AMACR may serve as IHC marker of chordoma with differentiating ability comparable to that of β-catenin.
Do Patients After Chondrosarcoma Treatment Have Age-appropriate Bone Mineral Density in the Long Term?
Clin Orthop Relat Res. 2016; 474(6):1508-15 [PubMed] Free Access to Full Article Related Publications
QUESTIONS/PURPOSES: The aim of our study was to address the following questions: (1) Do long-term survivors of chondrosarcoma have normal BMD and, if not, which factors contribute to low BMD? (2) Is there a greater risk of fracture and does the Fracture Risk Assessment Tool (FRAX(®)) score reflect fracture likelihood?
METHODS: All known patients with a history of chondrosarcoma treated at our institution before 2006 were identified. Of 127 patients believed to be alive at the time of this study, 30 agreed to participate in this study (11 females, 19 males; mean age at surgery, 39 ± 12 years; mean followup, 12 ± 5 years). With the data available, the 30 participants were not different from the 97 nonparticipants in terms of age, sex, BMI, tumor grade, tumor location (axial versus appendicular, lower extremity versus elsewhere), and use of any treatment known to influence osteopenia (chemotherapy, lower extremity surgery). BMD was measured and history of fractures was assessed using a questionnaire. The patients´ BMD measurements in this study were sex- and age-matched with a normative sex- and age-categorized reference population reported by Kudlacek et al. Associations were tested by univariate regressions and ANOVAs of all measures of BMD and eligible oncologic and demographic factors.
RESULTS: Eighteen of 30 (60%) patients had a pathologic BMD according to the WHO dual-energy x-ray absorptiometry definition, 15 (50%) had osteopenia, and three (10%) had osteoporosis. T-scores in the study cohort were lower than reference values for the femur neck (mean difference, 0.64; 95% CI, 0.27-1.01; p < 0.0015), but not for the spine (mean difference, 0.39; 95% CI, -0.06 to 0.84; p = 0.09). Thirteen patients (45%) reported a history of fractures not distinguishing between low and high impact. The incidence of fractures was 2.8 greater than expected from a comparison with a published microcensus survey of the Austrian population. No effect of the FRAX(®) score on fracture risk could be identified (p = 0.057).
CONCLUSIONS: Long-term survivors of chondrosarcoma appear to be at greater risk for having low BMD develop than the healthy population. Although these results are preliminary and based on a very small sampling of patients, if they can be confirmed in larger studies, BMD assessment by dual-energy x-ray absorptiometry might be considered as these patients are followed posttreatment by sarcoma care units. The reasons for low BMD still must be elucidated.
LEVEL OF EVIDENCE: Level IV, prognostic study.
Efficacy and Safety of Adjuvant Proton Therapy Combined With Surgery for Chondrosarcoma of the Skull Base: A Retrospective, Population-Based Study.
Int J Radiat Oncol Biol Phys. 2016; 95(1):312-21 [PubMed] Related Publications
METHODS AND MATERIALS: From 1996 to 2013, 159 patients (median age 40 years, range 12-83) were treated with either protons alone or a combination of protons and photons. The median total dose delivered was 70.2 Gy (relative biologic effectiveness [RBE]; range 67-71). Debulking and biopsy were performed in 133 and 13 patients, respectively.
RESULTS: With a median follow-up of 77 months (range 2-214), 5 tumors relapsed based on the initial gross tumor volume. The 5- and 10-year LC rates were 96.4% and 93.5%, respectively, and the 5- and 10-year OS rates were 94.9% and 87%, respectively. A total of 16 patients died (13 of intercurrent disease, 3 of disease progression). On multivariate analysis, age <40 years and primary disease status were independent favorable prognostic factors for progression-free survival and OS, and local tumor control was an independent favorable predictor of OS. In contrast, the extent of surgery, dosimetric parameters, and adjacent organs at risk were not prognostic factors for LC or OS.
CONCLUSIONS: Systematic high-dose postoperative proton therapy for skull base chondrosarcoma can achieve a high LC rate with a low toxicity profile. Maximal safe surgery, followed by high-dose conformal proton therapy, is therefore recommended.
Primary juxtacortical chondrosarcoma of mandibular symphysis: Unique and rare case report.
J Cancer Res Ther. 2015 Oct-Dec; 11(4):1025 [PubMed] Related Publications
Reconstruction of periacetabular tumours with saddle prosthesis or custom-made prosthesis, functional results and complications.
Hip Int. 2016; 26(2):e14-8 [PubMed] Related Publications
In vitro cytotoxic screening of 31 crude extracts of Thai herbs on a chondrosarcoma cell line and primary chondrocytes and apoptotic effects of selected extracts.
In Vitro Cell Dev Biol Anim. 2016; 52(4):434-44 [PubMed] Related Publications
Surgical Management of Spinal Chondrosarcomas.
Spine (Phila Pa 1976). 2016; 41(8):678-85 [PubMed] Related Publications
OBJECTIVE: The aim of this study was to determine whether the application of the Enneking classification in the management of spinal chondrosarcomas influences local recurrence and survival.
SUMMARY OF BACKGROUND DATA: Primary spinal chondrosarcomas are rare. Best available evidence is based on small case series, thus making it difficult to determine optimal management and risk factors for local recurrence and survival.
METHODS: The AOSpine Knowledge Forum Tumor developed a multicenter ambispective database of surgically treated patients with spinal chondrosarcoma. Patient data pertaining to demographics, diagnosis, treatment, cross-sectional survival, and local recurrence were collected. Tumors were classified according to the Enneking classification. Patients were divided into two cohorts: Enneking appropriate (EA) and Enneking inappropriate (EI). They were categorized as EA when the final pathological assessment of the margin matched the Enneking recommendation, and otherwise, they were categorized as EI.
RESULTS: Between 1987 and 2011, 111 patients (37 female; 74 male) received surgical treatment for a primary spinal chondrosarcoma at a mean age of 47.4 ± 15.8 years. Patients were followed for a median period of 3.1 years (range = 203 d-18.7 yrs). Median survival for the entire cohort was 8.4 years postoperative. After 10 years postoperative, 36 (32%) patients died and 37 (35%) patients suffered a local recurrence. Twenty-three of these 37 patients who suffered a local recurrence died. Sixty (58%) patients received an EA procedure while 44 (42%) received an EI procedure. EI patients had a higher hazard ratio for local recurrence than those who received an EA procedure (P = 0.052). Local recurrence was strongly associated with chondrosarcoma-related death (risk ratio = 3.6, P < 0.010).
CONCLUSION: This is the largest multicenter cohort of spinal chondrosarcomas. EA surgical management appeared to correlate with a decreased risk of local recurrence, yet no relationship with survival was found. Where possible, surgeons should strive to achieve EA margins to minimize the risk of local recurrence.
LEVEL OF EVIDENCE: 4.
Clinicopathological features of five unusual cases of intraosseous myoepithelial carcinomas, mimicking conventional primary bone tumours, including EWSR1 rearrangement in one case.
APMIS. 2016; 124(4):278-90 [PubMed] Related Publications
Skull base chondrosarcoma.
J Clin Neurosci. 2016; 24:1-5 [PubMed] Related Publications
Correlation between survival and tumour characteristics in patients with chondrosarcoma.
J Orthop Surg (Hong Kong). 2015; 23(3):365-9 [PubMed] Related Publications
METHODS: Records of 15 men and 8 women aged 14 to 66 (mean, 37) years who were diagnosed with primary (n=19) or secondary (n=4) chondrosarcoma of the axial skeleton (n=8), proximal extremity (n=9), or distal extremity (n=6) were reviewed. The tumour diameter was <10 cm in 4 patients, 10-19 cm in 12, and 20-30 cm in 7. The tumour involved the intramedullary in 17 and the periosteum in 6 patients; tumour extension was intracompartmental in 5 and extracompartmental in 18 patients. The Evans histological grade for the tumours was grade 1 in 6 patients, grade 2 in 10, and grade 3 in 7. The mean tumour size was 12.3 cm for grade 1 tumours, 18.2 cm for grade 2 tumours, and 18.3 cm for grade 3 tumours. 13 patients had no metastasis and 3 of 10 patients with grade 2 tumours and all 7 patients with grade 3 tumours had metastasis to the lung at presentation. 17 patients underwent surgery, one underwent adjuvant treatment only, and 5 declined treatment.
RESULTS: The mean follow-up period for the 23 patients was 3.1 years (range, 3 weeks to 9 years). The 5-year survival rate was 43% overall, 83.3% for grade 1 tumours, 50% for grade 2 tumours, and 0% for grade 3 tumours. The median survival duration was 20 (95% confidence interval, 11-29) months. Two patients had local recurrence and 16 did not, and the 5 patients who declined treatment died. Survival correlated with Evans histological grading (p=0.004), the presence of metastasis at presentation (p=0.026) and local recurrence (p=0.004).
CONCLUSION: The survival rate was lower in patients with higher Evan grading, metastasis, or local recurrence.