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The research retrospectively analyzed cases of spinal chordoma and chondrosarcoma involving patients who received treatment at the two hospitals between 2001 and 2023. Among the 48 patients studied (39 chordoma and 9 chondrosarcoma cases), the average age was 53.9 ± 15.8 years, with a range of 17 to 86 years. Out of these patients, 43 underwent excision surgery and were categorized based on tumor margin into negative (R0) or microscopically positive (R1) margin ( n  = 14) and macroscopically positive (R2) margin ( n  = 29) groups. The mean overall survival (OS) for R0/R1 and R2 groups was 156.5 ± 19.3 and 79.2 ± 11.9 months, respectively ( p value = 0.012). The mean progression-free survival (PFS) for R0/R1 and R2 was 112.9 ± 24.4 and 25.5 ± 5.5 months ( p value < 0.001). The study showed that regardless of whether patients in the R0/R1 or R2 groups received radiation therapy (RT) or not, there was no significant improvement in OS or PFS. Specifically, the OS and PFS for the RT only group were 75.9 ± 16.6 and 73.3 ± 18.0 months. In conclusion, the recommended treatment approach for spinal chordoma and chondrosarcoma remains en bloc resection surgery with an appropriate margin. Patients who are unsuitable for or decline surgery may find a beneficial disease control rate with traditional external beam photon/proton therapy.

IntroductionSpinal chondrosarcoma exhibits higher invasiveness and a worse prognosis compared to chondrosarcoma in the extremities. The prognosis and therapeutic plan vary greatly among different pathological subtypes of chondrosarcoma. This study aimed to analyze the differences in clinical characteristics, molecular features, therapeutic effects, and prognostic factors among the subtypes of chondrosarcoma in the spine.MethodsA retrospective review was conducted on 205 patients with spinal chondrosarcoma. The clinical features and immunohistochemical (IHC) markers were compared among the pathological subtypes of chondrosarcoma grade 1, grade 2, grade 3, mesenchymal chondrosarcoma (MCS), dedifferentiated chondrosarcoma (DCS), and clear cell chondrosarcoma (CCCS). Chondrosarcoma grade 1/2/3 are collectively referred to as conventional chondrosarcoma (CCS) for multivariate survival analysis. Univariate and multivariate analyses were performed to investigate independent prognostic factors for overall survival (OS) and recurrence-free survival (RFS) in patients with spinal chondrosarcoma. Furthermore, independent prognostic factors for OS and RFS were identified in CCS and MCS.ResultsMCS patients were younger than the other subtypes. Patients with chondrosarcoma grade 1/2 had better OS than those with chondrosarcoma grade 3, MCS and DCS, while only chondrosarcoma grade 1 patients showed better RFS than chondrosarcoma grade 2/3, MCS and DCS patients. Ki-67 index was higher in chondrosarcoma grade 3, MCS and DCS than chondrosarcoma grade 1/2. The comparison of IHC markers further highlighted the overexpression of P53/MDM2 in MCS and DCS. Gross total resection, including en-bloc and piecemeal resection, significantly improved OS and RFS for CCS patients, while only en-bloc resection significantly improved the prognosis of MCS patients. Chemotherapy appeared to be important for the OS of MCS patients.ConclusionP53/MDM2 pathway was upregulated in MCS and DCS compared to chondrosarcoma grade 1/2. Radical tumor resection is crucial for the treatment of spinal chondrosarcoma, while MCS patients require further comprehensive treatments perioperatively.

BackgroundExtraskeletal myxoid chondrosarcoma (EMC) is a rare sarcoma of uncertain origin, marked by specific chromosomal translocations involving the NR4A3 gene, and usually characterized by an indolent course. Surgery (with or without radiotherapy) is the treatment of choice in localized disease. The treatment for advanced disease remains uncertain. In order to better evaluate prognostic factors and outcome, a retrospective pooled analysis of patients with EMC treated at three Italian Sarcoma Group (ISG) referral centers was carried out.MethodsAll patients with localized EMC surgically treated from 1989 to 2016 were identified. Diagnosis was centrally reviewed according to WHO 2013. Only patients with NR4A3 rearrangement were included.ResultsSixty-seven patients were identified: 13 (20%) female, 54 (80%) male. Median age was 56 years (range 18–84). Numbers and type of translocation were: 50 (80%) NR4A3-EWS, 10 (16%) NR4A3-TAF15, 1 (2%) NR4A3-TCF12, and 1 (2%) NR4A3-TFG. Median follow-up was 55 months (range 2–312). Five- and ten-year overall survival rates were 94% (86–100 95%CI) and 84% (69–98 95%CI). Thirty-five (52%) patients relapsed: 9 had local recurrence (LR) and 26 had distant metastasis (5 with concomitant LR). The 5- and 10-year disease-free survival rates (DFS) were 51% (38–65 95%CI) and 20% (7–33 95%CI). Size of the primary tumor was significantly related to distant metastasis-free survival (DMFS) (p = 0.004). Patients carrying the NR4A3-EWS translocation had a trend in favor of better DFS (p = 0.08) and DMFS (p = 0.09) compared with the patients with NR4A3-TAF15.ConclusionsProlonged survival can be expected in patients with EMC, in spite of a high rate of recurrence. Size is significantly associated with distant relapse. The type of NR4A3 translocation could influence outcome.

There is discrepancy in the histologic concordance of pre-operative biopsy grading and post-surgical resection grading of periacetabular chondrosarcoma. This discrepancy poses a question as to whether the chosen treatment should depend upon on the initial assessment of the tumor grade. This is a retrospective review of patients with periacetabular cartilaginous tumors who were treated between the years 2000 and 2022 at a single institution. All patients included in the analysis underwent surgical resection. The pre-operative biopsy and post-surgical resection histological grades were compared. Concordance and treatment implications were evaluated. Twenty-two pre-operative Image Guided Core Needle Biopsies (IGCNB) were performed. There was concordance between pre-operative and surgical resection histological grade in only 9 cases, giving a (9/22) 40.9% accuracy rate. In total there were 13 cases of discrepancy between pre-operative biopsy and surgical resection regarding histological grade. In 12 cases of discrepancy, surgical resection histopathology revealed a higher grade than that of pre-operative biopsy histopathology. In one case of discrepancy, pre-operative IGCNB biopsy histology revealed a high-grade chondrosarcoma while the surgical resection histology revealed an intermediate-grade. There were no situations in which the plan made based on a preoperative biopsy resulted in an inappropriate treatment decision, even when there was not concordance between preoperative diagnosis and diagnosis after definitive resection. Significant discrepancy in grading was observed with pre-operative IGCNB of periacetabular cartilaginous lesions. However, while 12 out of the 13 discordant cases (92.3%) underestimated the true grade of the tumor, there was no impact on appropriateness of the selected treatment, suggesting that accuracy of grade is not an important factor to consider in this location.

Skull base chondrosarcoma is a rare tumour usually treated by surgery and proton therapy. However, as mortality rate is very low and treatment complications are frequent, a less aggressive therapeutic strategy could be considered. The objective of this study was to compare the results of surgery only vs surgery and adjuvant proton therapy, in terms of survival and treatment adverse effects, based on a retrospective series. Monocentric retrospective study at a tertiary care centre. All patients treated for a skull base grade I and II chondrosarcoma were included. We collected data concerning surgical and proton therapy treatment and up-to-date follow-up, including Common Terminology Criteria for Adverse Events (CTCAE) scores. 47 patients (23M/24F) were operated on between 2002 and 2015; mean age at diagnosis was 47 years-old (10-85). Petroclival and anterior skull base locations were found in 34 and 13 patients, respectively. Gross total resection was achieved in 17 cases (36%) and partial in 30 (64%). Adjuvant proton therapy (mean total dose 70 GyRBE,1.8 GyRBE/day) was administered in 23 cases. Overall mean follow-up was 91 months (7-182). Of the patients treated by surgery only, 8 (34%) experienced residual tumour progression (mean delay 51 months) and 5 received second-line proton therapy. Adjuvant proton therapy was associated with a significantly lower rate of relapse (11%; p = 0.01). There was no significant difference in 10-year disease specific survival between patients initially treated with or without adjuvant proton therapy (100% vs 89.8%, p = 0.14). Difference in high-grade toxicity was not statistically significant between patients in both groups (25% (7) vs 11% (5), p = 0.10). The most frequent adverse effect of proton therapy was sensorineural hearing loss (39%). Long-term disease specific survival was not significantly lower in patients without adjuvant proton therapy, but they experienced less adverse effects. We believe a surgery only strategy could be discussed, delaying as much as possible proton therapy in cases of relapse. Further prospective studies are needed to validate this more conservative strategy in skull base chondrosarcoma.

Background The surgical treatment of low-grade chondrosarcoma in the extremities remains a topic of controversy. This study aims to evaluate the clinical efficacy of microwave ablation combined with curettage in treating low-grade chondrosarcoma of the limbs. Methods We analyzed data from 17 patients who underwent microwave ablation for low-grade chondrosarcoma of the extremities over the past decade. Results were assessed based on general condition, surgical complications, local disease control, and functional outcomes as measured by the Musculoskeletal Tumor Society (MSTS) 93 scoring system. Results Among the 17 cases of low-grade chondrosarcoma, the distribution included 8 in the humerus, 7 in the femur, and 2 in the fibula. The median maximum diameter of the tumors was 5.5 cm (range: 4.0–8.2 cm), with a median follow-up of 53 months (range: 19–128 months). No patients required blood transfusions or experienced perioperative complications. The median surgical duration was 130 min (range: 60–150 min), and the average hospital stay was 7.9 days (range: 6–11 days). No local tumor recurrence or distant metastasis was observed during follow-up. Most patients reported minimal postoperative impact on limb functionality, with a median MSTS score of 28 (range: 20–30). Two patients sustained fractures; one underwent secondary internal fixation surgery and healed, while the other, with an asymptomatic microfracture, was monitored conservatively. Conclusions Microwave ablation combined with curettage is a viable option for local control in the treatment of low-grade chondrosarcoma in the extremities.

Abstract Chondrosarcomas are slow-growing malignant tumors of cartilaginous origin. The tumor can be locally aggressive with a risk of recurrence. Less than 10% of these tumors occur in the head and neck region, with most involving the maxilla, skull base, or larynx. Chondrosarcoma arising from the nasal septum is extremely rare. We report a case of a 64-year-old woman who presented with nasal obstruction and was subsequently diagnosed with nasal septal chondrosarcoma. She was treated with wide endoscopic surgical excision and regular follow-up, resulting in a good outcome.

Purpose The management of chordoma or chondrosarcoma involving the spine is often challenging due to adjacent critical structures and tumor radioresistance. Spine stereotactic radiosurgery (SSRS) has radiobiologic advantages compared with conventional radiotherapy, though there is limited evidence on SSRS in this population. We sought to characterize the long-term local control (LC) of patients treated with SSRS. Methods We retrospectively reviewed patients with chordoma or chondrosarcoma treated with dose-escalated SSRS, defined as 24 Gy in 1 fraction to the gross tumor volume. Overall survival (OS) was calculated by Kaplan-Meier functions. Competing risk analysis using the cause-specific hazard function estimated LC time. Results Fifteen patients, including 12 with chordoma and 3 with chondrosarcoma, with 22 lesions were included. SSRS intent was definitive, single-modality in 95% of cases (N = 21) and post-operative in 1 case (5%). After a median censored follow-up time of 5 years (IQR 4 to 8 years), median LC time was not reached (IQR 8 years to not reached), with LC rates of 100%, 100%, and 90% at 1 year, 2 years, and 5 years. The median OS was 8 years (IQR 3 years to not reached). Late grade 3 toxicity occurred after 23% of treatments (N = 5, fracture), all of which were managed successfully with stabilization. Conclusion Definitive dose-escalated SSRS to 24 Gy in 1 fraction appears to be a safe and effective treatment for achieving durable local control in chordoma or chondrosarcoma involving the spine, and may hold particular importance as a low-morbidity alternative to surgery in selected cases.

Spinal chondrosarcomas are uncommon malignant bone tumors that are rare in children. The location and imaging features of the lesion help in formulating a differential diagnosis, though in certain instances the lack of typical imaging findings may elude the differential of a chondrosarcoma. There are only a few reported cases in literature and owing to its rarity and lack of specific management protocols in this age group, making treatment decisions can be complex. We report a case of spinal chondrosarcoma in a young girl, where the treatment of choice was primary surgical resection. Transarterial embolization was performed to reduce tumor vascularity and aid the surgery.

Background Secondary peripheral chondrosarcomas arising in solitary osteochondromas is an unusual complication, reported in small series. In this study, we aimed to present our experience with this rare variant of chondrosarcoma and compare results with already published data in order to determine prognostic factors for overall and disease-free survival. Methods The case study includes retrospective data from patients diagnosed at a single institution from 1943 to 2019. Clinical data were collected reviewing all available medical records from first to last follow-up visits. To exclude the presence of the Multiple Osteochondroma Hereditary Syndrome, few patients, with a suspect of a familial form of the disease, were evaluated for the presence of germline heterozygous variants in EXT1 and EXT2 genes. Results were summarized using descriptive statistics and statistical analysis were performed to reveal associations between variables. Results Two hundred and fourteen secondary peripheral chondrosarcomas that arose exclusively from solitary osteochondromas diagnosed in a multidisciplinary setting at the IRCCS Istituto Ortopedico Rizzoli were retrospectively identified, 66.4% males and 33.6% females with a median age at diagnosis of 38 years. The local recurrence rate was 17.3%, while the metastases one was 5.1%. Besides age, a high histologic grade is the only factor associated with worse 5-year and 10-year overall survival (log-rank p  = 0.0005, HR = 3.74; 95% CI 1.69–8.26). Moreover, high histological grade (HR = 3.75; 95% CI = 1.69–8.34; p  = 0.001) and surgical debulking (HR = 3.71; 95% CI = 1.57–8.79; p  = 0.003) were associated with a significantly worse disease-free survival. Conclusions Our study confirm the low-grade behavior of secondary peripheral chondrosarcomas and demonstrate that the best choice of treatment for those arising in solitary osteochondromas is the wide surgical excision, when possible. Location per se is not a factor that affects prognosis, while the accurate histological grade assessment is correlated with the tumor aggressiveness and a long term follow up is necessary for this rare variant of chondrosarcoma.