Chondrosarcoma Of Spine Research Articles

Spinal chondrosarcoma in a young girl: radiological diagnosis and treatment perspectives.

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.

Comparisons of clinical characteristics, treatments, and outcomes among different pathological subtypes of chondrosarcoma in the spine.

Spinal 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. A 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. MCS 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. P53/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.

84. Development and validation of nomograms predicting overall and cancer-specific survival of spinal chondrosarcoma patients

84. Development and validation of nomograms predicting overall and cancer-specific survival of spinal chondrosarcoma patients

Anti-tumor effect of zartoprofen, a non-steroidal anti-inflammatory drug, targeting PPARγ (peroxisome proliferator-activated receptor gamma) on chondrosarcoma.

232 Background: Peroxisome proliferator-activated receptor gamma (PPARγ) plays a central role in the differentiation of adipocytes. Anti-tumor effects by activating PPARγ have been reported on chondrosarcoma. We revealed that zaltoprofen, a non-steroidal anti-inflammatory drug (NSAID), could activate and induce PPARγ in chondrosarcoma cells. We report antitumor effects of zaltoprofen targeting PPARγ on chondrosarcoma. Methods: PPARγ activation by zaltoprofen was assessed with luciferase assay. Human chondrosarcoma cells and PPARγ-silencing cells established with shRNA were subjected to the anti-tumor analyses, western blotting, RT-PCR, and zymography. For in vivo study, chondrosarcoma cells were subcutaneously inoculated into nude mice. Zaltoprofen was administered to mice (30 mg/kg/day). Tumors were pathologically assessed. We obtained tumor tissue specimens from a patient who underwent several surgeries for recurrent spinal chondrosarcoma. The patient was administered zaltoprofen as a therapeutic agent for algia the tumor remained stable for more than 2 years. Pathological analyses were performed on the specimen before and after administration of zaltoprofen). Results: Zaltoprofen significantly increased PPARg activation (EC50 47.3 uM). Treatment with 200–400 uM zaltoprofen significantly inhibited cell viability and induced PPARγ protein and mRNA expression in chondrosarcoma cells. Zaltoprofen (400 uM) significantly decreased the cell proliferation, migration, invasion, and MMP2 expression in control cells, whereas these were significantly increased in PPARγ-silencing cells. Also, zaltoprofen (400 uM) induced p21, p27, and p53 expression in control cells, whereas these effects were canceled in PPARg-silencing cells. Upregulation of KROX20 followed by CEBP-α and –β, all of which were reported to induce PPARγ in normal adipose tissue, were detected after zaltoprofen administration. Tumor growth was significantly inhibited in mice administered zaltoprofen. Apoptotic indices in the TUNEL-labeled area were significantly larger and Ki-67 positive cells were significantly lower in tumor specimens from mice administered zaltoprofen. Patient’s specimens revealed that PPARγ expression was stronger and MMP2 expression was weaker in post-administration specimens. Conclusions: Zaltoprofen suppressed tumor progression by inhibiting cell proliferation, migration, and invasion with induction of PPARγ expression in chondrosarcoma cells. The suppressing effect on tumor progression by zaltoprofen was also observed in vivo. Zaltoprofen induced PPARγ expression and MMP2 downregulation in clinical specimens with expected antitumor effects. This is the first study demonstrating that one of the NSAIDs, zaltoprofen, may act as an antitumor agent by targeting PPARγ for chondrosarcoma.

Chondrosarcoma of the Mobile Spine in the Elderly: A National Cancer Database Study

BackgroundThe current research on geriatric patients with spinal chondrosarcoma is limited. This study aimed to investigate the demographics, patterns of care, and survival of geriatric patients with chondrosarcoma of the mobile spine. MethodsThe National Cancer Database was queried from 2008 to 2018 for geriatric patients (60-89 years) with chondrosarcoma of the mobile spine. The primary outcome of this study was overall survival (OS). The secondary outcome was treatment utilization patterns. Survival analyses were conducted using log-rank tests and Cox proportional hazards regressions. Logistic regression models were utilized to assess correlations between baseline variables and treatment utilization. ResultsThe database retrieved 122 patients. While 43.7% of the patients presented with tumors exceeding 5cm in size, the incidence of regional lymph node involvement or distant metastases was relatively low, affecting only 5% of the patients. Furthermore, 22.3% of the patients had tumors graded as 3-4. The five-year OS rate was 52.9% (95% confidence interval 42-66.6). The mortality risk was significantly associated with age, tumor grade and stage, and treatment plan. Most patients (79.5%) underwent surgery, while 35.9% and 4.2% were treated with radiotherapy and chemotherapy, respectively. Age, race, comorbidities, geographical region, tumor stage, and healthcare facility type significantly correlated with treatment utilization. ConclusionSurgical resection significantly lowered the mortality risk in geriatric patients with spinal chondrosarcomas. Demographic and geographical factors significantly dictated treatment plans. Further studies are required to assess the role of radiotherapy and chemotherapy in treating these patients in the modern era.

Chondrosarcoma of the Mobile Spine: An Update on Patients Treated at a Single Institution.

Retrospective study. The objective of this study was to report the clinical data for patients treated with mobile spine chondrosarcoma. Chondrosarcoma of the mobile spine is a rare and challenging entity. A handful of case series have been published that report the clinical results of treatment, largely influenced by chondrosarcoma of the appendicular skeleton and pelvis. The clinical results of patients treated for chondrosarcoma of the mobile spine from our institution were published over 10 years ago and this represents and update since that publication. Inclusion criteria were adults patients treated for chondrosarcoma of the mobile spine at Massachusetts General Hospital between 2007 and 2020. Patients with large sacral tumors extending into the lumbar spine were excluded. Furthermore, we excluded patients with metastatic chondrosarcoma undergoing palliative decompressions for neurological instability or instrumented procedures for biomechanical instability. Therefore, only patients undergoing definitive surgery at the primary site of disease in the mobile spine were included. A total of 24 patients were included for review in this series. Seventeen of the 24 patients had their tumors excised with negative (R0) margins. Three of these 17 patients (18%) were dead of disease at final follow-up. There were two patients with R1 resections and five patients with R2 resections. Three of the seven patients (43%) with positive margins were dead of disease at final follow-up. A Cox proportional hazard analysis indicated total radiation dose was a significant covariate (HR=1.18, 95% CI: 1.01-1.39, P =0.03). We found higher percentages of overall survival with R0 tumor resection and lower histologic grade, whereas development of metastatic disease was closely associated with local recurrence and poor survival. Despite the improvements in treatment paradigms, it is sobering that our findings largely mirror those of previous work considering patients treated between 1984 and 2006.

Computed Tomography and Magnetic Resonance Imaging Features of Spinal Chondrosarcoma in a Cat

Computed Tomography and Magnetic Resonance Imaging Features of Spinal Chondrosarcoma in a Cat

Clinical diagnostic and radiographic features of a primary intradural spinal chondrosarcoma in a young adult: illustrative case.

Mesenchymal chondrosarcoma (MCS) is an aggressive subtype of chondrosarcoma that occurs extremely rarely in the central nervous system. Patients often present with pain or sensorimotor deficits, and resection is considered the gold standard. The role of adjuvant radiation and/or chemotherapy is largely unknown. A 22-year-old male presented with a 4-month history of progressive back and bilateral leg pain. He underwent imaging workup with magnetic resonance imaging of the lumbar spine and was found to have an intradural, extramedullary, heterogeneously enhancing mass spanning the L4-5 vertebral levels. Intraoperatively, a lobular, partially calcified mass with a ventral dural attachment displacing the nerve roots laterally was observed. The mass was removed en bloc, and the patient later underwent adjuvant radiotherapy, with no evidence of recurrence 2 years following surgery. Spinal MCS is extremely rare and often presents with a more aggressive course than conventional chondrosarcoma. Radiological diagnosis is challenging, as the tumor mimics different pathologies. The presence of calcifications, heterogeneous enhancement, and a more rapid clinical course as well as the presence of HEY1::NCOA2 gene fusion, which can be detected by surrogate immunohistochemistry, aids in diagnosis. Resection is the standard of care, and adjuvant radiation may be considered to reduce local recurrence, although further studies are warranted.

Gross total resection of spinal chondrosarcoma is associated with improved locoregional relapse-free survival and overall survival.

Spinal chondrosarcomas are rare malignant osseous tumors. The low incidence of spinal chondrosarcomas and the complexity of spine anatomy have led to heterogeneous treatment strategies with varying curative and survival rates. The goal of this study is to investigate prognostic factors for locoregional recurrence-free survival (LRFS) and overall survival (OS) comparing en bloc vs. piecemeal resection for the management of spinal chondrosarcoma. We retrospectively identified patients who underwent curative-intent resection of primary and metastatic spinal chondrosarcoma over a 25-year period. Univariate and multivariate survival analyses were conducted with LRFS as primary endpoint and OS as secondary endpoint. LRFS and OS were modeled using the Kaplan-Meier method and assessed using Cox regression analysis. For 72 patients who underwent first resection, the median follow-up time was 5.1years (95% CI 2.2-7.0). Thirty-three patients (45.8%) had en bloc resection, and 39 (54.2%) had piecemeal resection. Of the 68 patients for whom extent of resection was known, 44 patients had gross total resection (GTR) and 24 patients had subtotal resection. In survival analyses, both LRFS and OS showed statistically significant difference based on the extent of resection (p = 0.001; p = 0.04, respectively). However, only LRFS showed statistically significant difference when assessing the type of resection (p = 0.02). In addition, higher tumor grade and more invasive disease were associated with worse LRFS and OS rates. Although in our study en bloc and GTR were associated with improved survival, heterogenous and complex spinal presentations may limit total resection. Therefore, the surgical management should be tailored individually to ensure the best local control and maximum preservation of function.

One-stage En bloc resection of thoracic spinal chondrosarcoma with huge paravertebral mass through the single posterior approach by dissociate longissimus thoracis.

Study designRetrospective case series.ObjectiveTo describe the technique details and therapeutic outcomes of 3-D printing model-guided en bloc resection of chondrosarcoma (CHS) with huge paravertebral mass via the combined posterior median and Wiltse approach.Summary of background dataTotal en bloc spondylectomy (TES) technique is conventionally based on the single posterior approach or combined anterior-posterior approach. However, the single posterior approach imposes a high technical demand on the surgeon due to the narrow field of vision, limited surgical space and the delicate spinal cord, while the combined anterior-posterior approach not only requires greater patient tolerance but is time consuming and runs the risk of more blood loss and injury to the visceral pleura and large blood vessels during surgery. In addition, it is difficult to completely remove the thoracic CHS with paravertebral mass through simple en bloc resection when it involves the aorta, vena cava, costa and lung.Material and methodsBetween August 2010 and January 2016, we performed a retrospective study to evaluate the clinical characteristics and outcomes of en bloc resection of thoracic spinal CHS with paravertebral mass through the combined posterior median and Wiltse approach. Postoperative recurrence-free survival (RFS) and overall survival (OS) were estimated by the Kaplan-Meier method. P values less than 0.05 were considered statistically significant.ResultsAltogether 15 patients received en bloc resection of thoracic spinal CHS with paravertebral mass through the combined posterior median and Wiltse approach. The mean age of these patients was 37.0 ± 12.8 years (median 36; range 15–64). This combination approach provided more extensive exposure and wider marginal resection of the tumor within a mean operation duration of 288 ± 96 min (median 280; range 140–480) and mean intraoperative blood loss of 1,966 ± 830 ml (median 2,000; range 300–3,000). Of the 15 patients, 5 experienced local recurrence of the disease; the mean time from surgery to recurrence was 22 ± 9.85 months (median 17, range 13–35). RFS in patients with recurrent CHS was significantly lower than that in patients with primary CHS on admission (p = 0.05).ConclusionsThe combined posterior median and Wiltse approach is a technically viable option for en bloc resection of thoracic spinal CHS with huge paravertebral mass, and can give a favorable local control of CHS.Level of evidenceLevel V.

Mice with Trp53 and Rb1 deficiency in chondrocytes spontaneously develop chondrosarcoma via overactivation of YAP signaling

Chondrosarcoma (CHS) is a rare type of soft sarcoma with increased production of cartilage matrix arising from soft bone tissues. Currently, surgical resection is the primary clinical treatment for chondrosarcoma due to the poor response to radiotherapy and chemotherapy. However, the therapeutic effect is not satisfactory due to the higher local recurrence rate. Thus, management and elucidation of the pathological mechanism of chondrosarcoma remain an ongoing challenge, and the development of effective chondrosarcoma mouse models and treatment options are urgently needed. Here, we generated a new transgenic chondrosarcoma model by double conditional deletions of Trp53 and Rb1 in chondrocyte lineage which spontaneously caused spinal chondrosarcoma and lung metastasis. Bioinformatic analysis of the human soft sarcoma database showed that Trp53 and Rb1 genes had higher mutations, reaching up to approximately 33.5% and 8.7%, respectively. Additionally, Trp53 and Rb1 signatures were decreased in the human and mouse chondrosarcoma tissues. Mechanistically, we found that YAP expression and activity were significantly increased in mouse Col2-Cre;Trp53f/f/Rb1f/f chondrosarcoma tissues compared to the adjacent normal cartilage. Knockdown of YAP in primary chondrosarcoma cells significantly inhibited chondrosarcoma proliferation, invasion, and tumorsphere formation. Chondrocyte lineage ablation of YAP delayed chondrosarcoma progression and lung metastasis in Col2-Cre;Trp53f/f/Rb1f/f mice. Moreover, we found that metformin served as a YAP inhibitor, which bound to the activity area of YAP protein, and inhibited chondrosarcoma cell proliferation, migration, invasion, and progression in vitro and significantly suppressed chondrosarcoma formation in vivo. Collectively, this study identifies the inhibition of YAP may be an effective therapeutic strategy for the treatment of chondrosarcoma.

Refractory recurrent spinal chondrosarcoma: What is the role of salvage surgery?

BackgroundThe spinal chondrosarcoma has high risk of recurrence if the initial surgery is not performed in an en bloc fashion. It remains technically demanding to surgically manage the refractory recurrent spinal chondrosarcoma (RRSC). This study is to assess the clinical features and investigate the prognostic factors for patients with RRSCs. Methodsforty-nine patients with RRSCs underwent salvage surgeries in our institution, and the clinical characteristics were collected and recorded by two independent reviewers. Univariate and multivariate analyses were performed to investigate the independent prognostic factors of recurrence-free survival (RFS) and overall survival (OS) for patients with RRSCs. ResultsDuring the mean follow-up of 31.7 ± 21.04 months (Range 9–93), the 3-year RFS and OS rate was 24.5% and 34.5%, respectively. According to the Cox proportional hazards regression model, wide excision with tumor-free margin (>4 mm) was associated with both better RFS and OS for patients with RRSCs. Meanwhile, the number of recurrences ≤2 was beneficial to RFS, while high pathological grade was correlated with worse OS. ConclusionsWide excision with tumor-free margin (>4 mm) is recommendable if appropriate in the salvage surgery for patients with RRSCs. Patients with number of recurrences ≤ 2 and lower pathological grade may have better RFS and OS, respectively.

Anaesthesia management of a case of huge neck mass due to cervical spine chondrosarcoma with distorted anatomy

Chondrosarcoma is the tumor which affects bone and soft tissue with only 2% spinal involvement. Anesthetic management becomes challenging in patients with cervical chondrosarcoma. Here, we are presenting a case of huge neck mass due to cervical spine chondrosarcoma in 70 year old male hypertensive patient. The patient has distorted anatomy with mucosal edema with left tracheal deviation and compression from right side. Awake Nasal Fiberoptic intubation was done with cuffed ETT no 8. The neck mass was removed and Anterior Cervical Discectomy and Fusion (ACDF) with bone grafting. The case was managed with adequate analgesia, replacement of fluids and Blood and Blood products. Considering complex cervical spine surgery and airway edema the patient was shifted to Surgical Intensive Care Unit (ICU) for elective ventilation and advanced monitoring. After serial ABG and proper weaning the patient was extubated next morning smoothly. Extensive preoperative evaluation, planning, clinical judgement and skilled experienced personale are essential for proper execution of difficult airway cases.

Mobile Spine Osseous Sarcoma: Descriptive Epidemiological Analysis Based on a National Population-Based Study

Primary osseous sarcomas of the mobile spine are rare bony tumors. Ewing sarcoma, chondrosarcoma, chordoma and osteosarcoma constitute the majority of primary bone sarcomas of the spine; however, other rare sarcoma tumors may also affect the spine. In order to perform an epidemiological study of theses tumors, national registries may help to evaluate data for populations with similar characteristics. A population-based study was designed based on data from the Iran National Cancer Registry (INCR). All morphology codes (M-Code) of primary osseous sarcomas of the mobile spine (C-code 41.2) were derived and analyzed. Among 186 patients with primary osseous sarcomas of the mobile spine, 67.2% were men and 32.8% were women. The median (IQR) age was 37.0 (20.0-59.0) years and the age-standardized incidence rate (ASIR) was 0.37 per million. The majority of cases of Ewing sarcoma (29.5%) were observed in the age group 20-25 years. Among male patients with chondrosarcoma, the median age was 39.0 (30.0-50.0), while females showed a median age of 56.0 (50.0-59.0). The median age of patients with chordoma was 54.0 (47.0-63.0) years. The crude incidence rate of mobile spine osteosarcoma was 0.04 per million. Ewing sarcoma was the most frequent primary osseous sarcoma of the mobile spine. A male predilection was observed among all major sarcomas of the mobile spine. Ewing sarcoma in Iran affects the mobile spine in slightly older ages compared to other studies. Myxoid chondrosarcoma is the most frequent subtype of the mobile spine chondrosarcoma. Chordoma affects male in older ages compared to females. In contrast with other studies which showed a bimodal distribution of osteosarcoma of the spine including young adult and older age groups, 86% of cases in Iran were in the age group of 10-40 years.

Incidence, Management, and Outcomes of Adult Patients with Spinal Chondrosarcoma in the United States

Spinal chondrosarcomas are rare primary malignant neoplasms composed of cartilage-producing cells. They are slow-growing but locally aggressive lesions that have high rates of recurrence and progression after treatment. We provide the largest comprehensive analysis of prognostic factors, treatment modalities, and survival outcomes in patients with spinal chondrosarcoma using a large, prospectively collected national database. Patients with diagnosis codes specific for chondrosarcoma of the spine, sacrum, and coccyx were queried from the National Cancer Database (NCDB) during 2004-2016. Outcomes were investigated using Cox univariate and multivariate regression analyses, and survival curves were generated for comparative visualization. A total of 1843 individuals were identified with a diagnosis of chondrosarcoma, 82.1% of which were at the sacrum or coccyx and 17.9% at the spine. The mean overall survival of patients in our cohort was 7.91 years. Increased age, larger tumor, dedifferentiated histology, and presence of metastases were associated with worsened overall survival. Regarding management, 77.7% of patients received surgical intervention and both partial and radical resection were associated with significantly improved overall survival (P < 0.001). Neither radiotherapy nor chemotherapy administration improved overall survival; however, among patients who received radiation, those who received higher-dose radiation had significantly improved overall survival compared with those who received lower-dose radiation. Surgical resection significantly improves overall survival in patients with spinal chondrosarcoma. In those patients receiving radiation, those who receive high doses have improved overall survival compared with those who receive lower doses. Further studies into optimal radiation modality and doses are required.

Use of Navigated Ultrasonic Bone Cutting Tool for En Bloc Resection of Thoracic Chondrosarcoma: Technical Report.

En bloc surgical resection with wide margins offers the best local control rates for chondrosarcoma of the spine. To describe the surgical technique for en bloc resection of a large thoracic chondrosarcoma using image guidance for a complex osteotomy with an ultrasonic bone cutting device (Misonix, Farmingdale, New York). A 2-stage procedure was performed for resection of a thoracic chondrosarcoma involving the T3-T7 vertebral bodies. During the first stage, a posterior approach, the ultrasonic bone cutter was precisely navigated to perform an intralaminar osteotomy as well as a multilevel split sagittal osteotomy through the vertebral bodies. In the second stage, a transthoracic approach was used to complete the en bloc resection of the specimen. Intraoperative frozen sections from the surgical margins were negative for tumor. The ultrasonic bone cutting device was navigated based on coregistration of the intraoperative computed tomography (CT) images and preoperative magnetic resonance imaging (MRI). Real-time navigation using coregistered images enabled identification of tumor margins within the bone and adjacent soft tissue allowing precise execution of the intralaminar and multilevel split sagittal vertebral osteotomies. Surgical video demonstrates the utility of real-time navigation to properly identify the tumor margins and guide the ultrasonic bone cutting tool during the osteotomies. We describe the use of image guidance to navigate an ultrasonic bone cutting tool for a complex en bloc resection of a multilevel thoracic spine chondrosarcoma.

Management of Incidental High-grade Dedifferentiated Chondrosarcoma by Triple Vertebrectomy and Anterior Reconstruction Using a Posterior-only Approach

Introduction: Chondrosarcomas of the spine most commonly present with pain and neurological involvement and surgical resection is the standard of care. However, the involvement of 3 contiguous vertebral bodies is rare and surgical en bloc resection may not be possible in all cases, particularly when there is an extra compartmental spread. Materials and Methods: We report a case of incidentally detected biopsy-proven chondrosarcoma who was treated by a triple vertebrectomy through a posterior-only approach. Results: Three nerve roots had to be sacrificed on one side and 1 nerve root on the other side. The radicular arteries were freed from the vertebral body and tumor mass. Since the pedicles and lamina were involved and there was an extra compartmental spread to the nearby vital structures, an en bloc resection could not be accomplished. Anterior reconstruction was performed through the posterior approach using a MESH cage. Posterior stabilization using pedicle screws and rods was done 3 levels above and below the vertebrectomy levels. Biopsy suggested a very rare aggressive variant of spinal chondrosarcoma labeled as a dedifferentiated chondrosarcoma. Postoperative Image-guided radiotherapy was given and the patient was doing well at 1-year follow-up. Discussion: Primary chondrosarcoma involving 3 or more vertebra has been rarely reported in the literature and no specific guidelines exist on the management of such tumors. The aim of this case report was to emphasize the feasibility of doing a triple vertebrectomy and anterior reconstruction using a posterior-only approach. Posterior-only approach allows for complete tumor removal and circumferential stabilization in a single setting.

Primary Tumor Resection Prolongs Survival in Spinal Chondrosarcoma Patients With Distant Metastasis.

Retrospective analysis. To investigate (1) whether resection of primary tumor improves survival of metastatic spinal chondrosarcoma patients and (2) which subgroups of metastatic spinal chondrosarcoma patients benefit more from primary tumor resection. Surgical resection is the mainstay of treatment for spinal chondrosarcoma, as chondrosarcoma is inherently resistant to radiotherapy and chemotherapy. However, evidence which justifies resection of the primary tumor for patients with metastatic spinal chondrosarcoma is still lacking. We retrospectively included 110 patients with metastatic spinal chondrosarcoma in the Surveillance, Epidemiology, and End Results database from 1983 to 2016. The association between primary tumor resection and survival was evaluated using Kaplan-Meier analyses, log-rank tests, and multivariable Cox analyses. The effect of primary tumor resection on survival was further assessed in subgroups stratified by histologic subtype, tumor grade, and age. Overall, 110 patients were divided into surgery group (n = 55, 50%) and nonsurgery group (n = 55, 50%). Primary tumor resection was associated with both prolonged overall survival (hazard ratio 0.262, 95% confidence interval 0.149-0.462, P < 0.001) and cancer-specific survival (hazard ratio 0.228, 95% confidence interval 0.127-0.409, P < 0.001). When we focused on surgical effects in subgroups, primary tumor resection conferred survival advantage on patients with conventional subtype, grade I to III malignancy, and an age younger than 70 years old (P < 0.001 for overall and cancer-specific survival). However, primary tumor resection brought limited survival benefit for patients with dedifferentiated subtype and patients over 70 years old. The present population-based study for the first time reports a clear association between primary tumor resection and prolonged survival in metastatic spinal chondrosarcoma patients. Specifically, primary tumor resection was associated with improved survival in patients with conventional subtype, grade I to III malignancy, and an age younger than 70 years old. 4.

Conditional Survival Analysis for Spinal Chondrosarcoma Patients After Surgical Resection.

Retrospective analysis. To evaluate conditional survival after surgical resection for spinal chondrosarcoma patients. Survival estimates are usually reported as survival from the time of surgery, but survival probabilities can change over time. Conditional survival, which is a measure of prognosis for patients who have survived a defined period of time, may be more clinically precise and relevant. However, data on conditional survival for spinal chondrosarcoma patients after surgical resection are still lacking. We used the Surveillance, Epidemiology, and End Results (SEER) database to identify 436 spinal chondrosarcoma patients who underwent surgical resection from 1994 and 2013. Kaplan-Meier analyses and Cox regression modeling were performed to evaluate prognostic factors associated with overall survival. Five-year conditional survival (i.e., probability of surviving an additional 5 years, given that a patient has already survived x years) was calculated as 5-CS(x) = OS(x+5)/OS(x). The effect of prognostic factors on conditional survival was also explored. Four hundred thirty six patients were included in the study cohort. Overall, 1-, 3-, and 5-year overall survival were 92.8%, 79.1%, and 70.3%, respectively. Five-year conditional survival at 1, 3, and 5 years after surgery were 72.9%, 79.0%, and 87.5%. The overall survival rates were lower in cases of age more than or equal to 60 years, male patient, dedifferentiated subtype, Grade III tumor, tumor size more than or equal to 10 cm, distant metastasis, and radiotherapy. Conditional survival improved over time in each subgroup divided by age, sex, race, year of diagnosis, grade, tumor size, extent of disease (EOD), and radiotherapy. In addition, patients with the least favorable prognosis at baseline experienced the greatest increase in 5-year conditional survival over time (e.g., Grade I/II: 78.0%-89.7%, Δ11.7% vs. Grade III: 36.5%-66.6%, Δ30.1%; Localized/Regional: 72.9%-88.1%, Δ15.2% vs. Distant: 43.5%-74.1%, Δ30.6%). Conditional survival for spinal chondrosarcoma patients after surgical resection improves over time, especially for patients with initial high-risk characteristics. Information derived from conditional survival analysis may provide individualized approaches to surveillance and treatment of spinal chondrosarcoma. 4.

Primary intradural extramedullary spinal mesenchymal chondrosarcoma: case report and literature review

BackgroundMesenchymal chondrosarcoma (MCS) is a rare malignant variant of chondrosarcoma with a high tendency of recurrence and metastasis. Intradural extramedullary spinal MCS is exceedingly rare and usually found in pediatric patients. Herein, we present an elderly patient with primary intradural extramedullary spinal MCS. Relevant literatures are reviewed to disclose characteristics of intradural extramedullary spinal MCS.Case presentationA 64-year-old female presented with urinary difficulty and tightness of upper back preceding progressive weakness of right lower extremity. Magnetic resonance imaging revealed an intradural extramedullary tumor at the level of 3rd thoracic vertebra. This patient underwent total tumor resection and then received adjuvant radiotherapy. Histopathological examination showed that the tumor composed of spindle and round cells with high nucleocytoplasmic ratio accompanied by scattered eosinophilic chondroid matrix. Along with immunohistochemical findings and the existence of HEY1-NCOA2 fusion transcript, the diagnosis of MCS was confirmed. Neurologic deficit recovered nearly completely after surgery. No evidence of local recurrence or distant metastasis was found 5 years after treatments. Including the current case, a total of 18 cases have been reported in the literature with only one case with local recurrence and one case of mortality. The current case was the eldest patient diagnosed with primary intraspinal MCS in the literature.ConclusionsMCS rarely appears in the intradural space of the spine. In contrast to classic MCS, treatment outcome of primary intradural extramedullary spinal MCS is usually excellent as total tumor resection is commonly achievable. Adjuvant radiotherapy may reduce local recurrence and chemotherapy may be associated with fewer recurrences especially for unresectable tumors.