A rare case of cutaneous metastasis of distal phalanx chondrosarcoma.

Chondrosarcoma is the second-most common primary malignant bone tumor but chondrosarcoma of small bones of the hand is extremely rare, representing less than 2% of all cases, with exceedingly rare skin metastases. Cutaneous metastases of chondrosarcoma represent less than 3% of all cutaneous metastases. According to PubMed, there are only four previous case reports of cutaneous metastases originating from chondrosarcoma of small bones of the hand. We present an additional case of cutaneous metastases of phalangeal chondrosarcoma with a unique immunophenotype.

Outcome in dedifferentiated chondrosarcoma for patients treated with multimodal therapy: Results from the EUROpean Bone Over 40 Sarcoma Study.

INTRODUCTION: The role of chemotherapy for patients with dedifferentiated chondrosarcoma (DDCS) is still under discussion. Here, we present the outcome in patients with DDCS treated with intensive chemotherapy from the EUROpean Bone Over 40 Sarcoma Study. MATERIALS AND METHODS: The chemotherapy regimen included doxorubicin, ifosfamide and cisplatin. Postoperative methotrexate was added in case of poor histological response. Toxicity was graded based on the National Cancer Institute expanded common toxicity criteria, version 2.0, and survival was analysed using Kaplan-Meier curves, log-rank tests and univariate Cox regression models. RESULTS: Fifty-seven patients with DDCS (localised, 34 [60%]; metastatic, 23 [40%]) aged 42-65 years were included. Surgical complete remission (SCR) was achieved in 36 (63%) patients. The median overall survival (OS) was 24 months (95% confidence interval, 22-25), and the 5-year OS was 39%. Patients with extremity localisation had a 5-year OS of 49% compared with 29% in patients with a central tumour (P = 0.08). Patients with localised disease had a 5-year OS of 46%, whereas patients with metastatic disease had a 5-year OS of 29% (P = 0.12). Patients in SCR had a 5-year OS of 49%, whereas patients not in SCR had a 5-year OS of 23% (P = 0.004). Chemotherapy toxicity was considerable but manageable. There was no treatment-related death, and 39 (70%) patients received ≥6 cycles of the planned nine chemotherapy cycles. CONCLUSIONS: Adding intensive chemotherapy to surgery for treatment of DDCS is feasible and shows favourable survival data compared with previous reports. With the limitations of data from a non-controlled trial, we conclude that chemotherapy could be considered in the management of patients aged >40 years.

Efficacy and safety of regorafenib in patients with metastatic or locally advanced chondrosarcoma: Results of a non-comparative, randomised, double-blind, placebo controlled, multicentre phase II study.

BACKGROUND: This multi-cohort trial explored the efficacy and safety of regorafenib for patients with advanced sarcomas of bone origin; this report details the cohort of patients with metastatic or locally advanced chondrosarcoma (CS), progressing after prior chemotherapy. PATIENTS AND METHODS: Patients with CS, progressing despite prior standard therapy, were randomised (2:1) to receive regorafenib or placebo. Patients on placebo could crossover to receive regorafenib after centrally confirmed progressive disease. The primary endpoint was progression-free rate (PFR) at 12 weeks. With one-sided α of 0.05, and 80% power, at least 16/24 progression-free patients at 12 weeks were needed for success (P0 = 50%, P1 = 75%). RESULTS: From September 2014 to February 2019, 46 patients were included in the CS cohort, and 40 patients were evaluable for efficacy: 16 on placebo and 24 on regorafenib. Thirteen patients (54.2%; 95% CI [35.8%-[) were non-progressive at 12 weeks on regorafenib versus 5 (31.3%; 95% CI [13.2%-[);) on placebo. Median PFS was 19.9 weeks on regorafenib, and 8.0 on placebo. Fourteen placebo patients crossed over to regorafenib after progression. The most common grade ≥3 treatment-related adverse events on regorafenib included hypertension (12%), asthenia (8%), thrombocytopenia (8%) and diarrhoea (8%). One episode of fatal liver dysfunction occurred on regorafenib. CONCLUSION: Although the primary endpoint was not met statistically in this small randomised cohort, there is modest evidence to suggest that regorafenib might slow disease progression in patients with metastatic CS after the failure of prior chemotherapy. CLINICAL TRIAL REGISTRATION: The trial is registered at ClinicalTrials.gov (NCT02389244).

Unexpected diagnosis of multiple sclerosing pneumocytomas in a patient with chondrosarcoma of the hand.

Sclerosing pneumocytomas are rare, benign pulmonary neoplasms that predominantly affect Asian female patients in the age category of 40-70 years, mostly non-smokers. We report on a 72-year-old Caucasian woman with chondrosarcoma of the hand who developed multiple bilateral progressive lung nodules suspicious of lung metastases. Staged lung resections were performed, and pathological diagnosis was confirmed by immunohistochemical analysis of the resected specimens. Next-generation sequencing (NGS) was used to detect gene mutations. Immunohistochemistry demonstrated sclerosing pneumocytomas, and NGS showed an IDH1 mutation. Eventually, the patient developed lung metastases for which rethoracotomy was performed. The differentiation of sclerosing pneumocytoma from lung cancer is a diagnostic challenge, and sclerosing pneumocytoma should be considered in the differential diagnosis of pulmonary nodules. Gene mutation analysis does not always show classical and common mutations, which should be kept in mind when interpreting its results.

Differences in clinical characteristics and tumor prognosis between primary and secondary conventional pelvic chondrosarcoma.

BACKGROUND: Chondrosarcoma (CS) most commonly involves the pelvis. This study aimed to analyze differences in clinical characteristics and prognostic factors between primary and secondary conventional pelvic CS, and provide reference for clinical diagnosis and treatment. METHODS: Eighty patients (54 primary cases and 26 secondary cases) with pelvic CS were included in this retrospective study. The tumor site, Enneking stage, soft tissue mass, margin, initial tumor grade, incidence of local recurrence and distant metastasis were evaluated. Kaplan-Meier method was used to calculate the overall survival rate. X2 test and log-rank test were used for univariate analysis, and Cox test was used in multivariate analysis. RESULTS: The average age of patients with secondary CS was significantly younger than that of patients with primary CS (P < 0.001). The soft tissue mass of patients with secondary CS was significantly larger than that of patients with primary CS (P = 0.002). There was a significant difference in initial tumor pathologic grade between the two groups (P = 0.002). No statistically significant difference was observed in the local recurrence rate between the two groups. The median recurrence time of patients with primary CS after the first treatment was significantly shorter than that of patients with secondary CS (P < 0.001). The overall survival rate of patients with secondary CS was much higher than that of patients with primary CS (P = 0.003). Cox regression analysis showed that the initial tumor grade was an independent factor in the overall survival rate of patients with CS. CONCLUSION: There were significant differences in age, soft tissue mass, initial tumor grade, and overall survival rate between the two groups. The overall survival rate of pelvic CS was related to the initial tumor grade of CS.

Laparoscopic liver resection of the Spiegel lobe by a left-sided approach preserving an accessory left hepatic artery.

BACKGROUND: Caudate lobe of the liver can be divided in three portions: Spiegel lobe, paracaval portion and caudate process [1]. The anatomy of the caudate lobe and its proximity to major vascular structures makes this operation a difficult resection, especially for laparoscopic liver surgery [2]. For that reason the Iwate criteria proposed to classify this surgery at the "2nd International Consensus Conference on Laparoscopic Liver Resection" as intermediate or advanced difficulty depending on the type of resection, and the size of the tumor [3]. For tumors located in the Spiegel lobe, the left side approach is preferred [4]. METHODS: We present a 69-year-old woman with liver metastasis from previously resected sternal condrosarcoma on 30/05/2017. A unique recurrence in the caudate lobe was found on computed tomography and magnetic resonance imaging on April 2019. RESULTS: A Spiegel lobe resection with left side approach preserving an accessory left hepatic artery was performed. A resection of the artery was planned if the preservation was not possible. The operative time was 180 min and the total Pringle time was 30 min. Patient was discharged at the 4 postoperative day and there were no postoperative complications CONCLUSIONS: Laparoscopic liver resection of the caudate lobe for condrosarcoma liver metastasis is feasible and safe. Left approach seems the best option for Spiegel lobe resections even in patients with accessory left hepatic artery.

Reconstruction of forearm support with ulnar translocation after resection of chondrosarcoma in the proximal radius.

This is a report of the reconstructive surgery of a patient with chondrosarcoma in the proximal radius. After extensive resection of the proximal radius that contained the tumor, the skeleton of the forearm was reconstructed by ulnar translocation. This patient was followed for 2 years, no recurrence of the tumor was found and the function of the forearm was nearly normal. This case is reported and discussed and a literature review is presented.

Exosomes in Bone Sarcomas: Key Players in Metastasis.

Bone sarcomas are rare cancers which often present with metastatic disease and are still associated with poor survival rates. Studies in the last decade have identified that exosomes, a type of extracellular vesicle released by cells, play an important role in tumour progression and dissemination. Through the transfer of their cargo (RNAs, proteins, and lipids) across cells, they are involved in cellular cross-talk and can induce changes in cellular behaviour. Exosomes have been shown to be important in metastasis organotropism, induction of angiogenesis and vascular permeability, the education of cells towards a pro-metastatic phenotype or the interaction between stromal and tumour cells. Due to the importance exosomes have in disease progression and the high incidence of metastasis in bone sarcomas, recent studies have evaluated the implications of these extracellular vesicles in bone sarcomas. In this review, we discuss the studies that evaluate the role of exosomes in osteosarcoma, Ewing sarcoma, and preliminary data on chondrosarcoma.

Lichen planus related to transforming growth factor beta inhibitor in a patient with metastatic chondrosarcoma: a case report.

Transforming growth factor-beta1 (TGF-ß1) is expressed in normal epidermis. TGF-ß1 potently inhibits keratinocyte proliferation and immunomodulatory properties, mainly by suppressing immune responses to self-antigens. Lichen planus (LP) is a form of dermatitis caused by cell-mediated immune dysfunction, but the exact pathogenic pathways are unknown, which poses therapeutic challenges. We report on a 68-year-old man who developed multiple pruritic, discrete, and well-demarcated, flat-topped red-purple papules and macules on the back and upper arms following 4 cycles of treatment with TGF-ß receptor I (TGFBR-I) inhibitor, ly3200882, for metastatic chondrosarcoma. The biopsy showed hyperkeratosis, wedge-shaped hypergranulosis, elongation of the rete ridges, and a dense band-like lymphohistiocytic infiltrate admixed with colloid bodies and pigment incontinence, consistent with LP. Temporal correlation suggested that the TGFBR-I inhibitor might be a trigger. Treatment with topical clobetasol and oral metronidazole led to partial resolution of the lesions with postinflammatory hyperpigmentation. We believe this is the first reported case of LP related to TGFBR-I inhibitor therapy. This report expands the list of cutaneous adverse events associated with this novel class of targeted therapy. More importantly, this report supports emerging evidence that failure of TGF-ß1 activation/signal transduction is an important mechanism in the pathogenesis of LP and suggests the TGF-ß1 pathway as a potential therapeutic target in this disease.

The diagnostic significance of pulmonary nodules on CT thorax in chondrosarcoma of bone.

AIM: To provide a diagnostic approach to pulmonary nodules in patients with chondrosarcoma. MATERIALS AND METHODS: A search of the oncology database at a specialist orthopaedic oncology referral centre was performed to identify all patients who were treated surgically for chondrosarcoma between January 2007 and December 2018. Reports from the computed tomography (CT) examinations of the thorax of these patients were reviewed. In patients who had pulmonary nodules/metastases identified on CT, data on the primary chondrosarcoma and pulmonary nodule characteristics were collected. RESULTS: Twenty point two percent of patients had a pulmonary nodule identified on either initial or follow-up staging CT of the thorax, of which 8.1% were pulmonary metastases. Patients with grade 3 and dedifferentiated chondrosarcoma were more likely to have pulmonary metastases than patients with grade 1/2 chondrosarcoma. The time interval to developing metastases was shorter in patients with grade 2/3 and dedifferentiated chondrosarcoma versus patients with grade 1 chondrosarcoma. A low proportion of patients with grade 1 chondrosarcoma developed metastases (12.5%), all of which were identified at the time of a local recurrence. Nodules ≥10mm, nodules with lobulate margins, nodules containing irregular or subtle calcification, and nodules seen bilaterally or both centrally and peripherally were more likely to represent pulmonary metastases than benign nodules. CONCLUSION: The diagnostic significance of pulmonary nodules (i.e., whether they represent pulmonary metastases or not) can be predicted by taking into account a number of factors, in particular, the histological grade of the patient's chondrosarcoma, the size and margins of the nodules, and the presence of subtle/irregular calcification.

Thyroid metastasis from chondrosarcoma.

For chondrosarcoma, metastasis to the thyroid gland is extremely rare. The diagnosis and treatment of thyroid metastasis from chondrosarcoma are discussed here.We found a case of thyroid malignancy occurring after treatment of chondrosarcoma. We reviewed patient characteristics, histological presentations on initial chondrosarcoma and thyroid metastasis, treatments, times of recurrence and death. In addition, we searched Embase, PubMed, and ISI Web of Science databases (1996-2018) for articles published in the English language using the key words "chondrosarcoma" and "thyroid" and we reviewed almost all the reports about thyroid metastasis from chondrosarcoma.Only 5 cases of chondrosarcoma metastases in the thyroid gland have been reported in the literature. We found that most patients are adults, with compression signs or pain, most of whom have poor prognoses. The main examinations are ultrasound, CT and fine needle aspiration biopsy, and primary treatment is surgery.These rare cases of chondrosarcoma presenting as a metastasis in the thyroid gland highlight the importance of close communication between radiologists, histopathologists, and clinicians to ensure that such exceptional cases are not missed.

Secondary chondrosarcoma arising from osteochondroma: outcomes and prognostic factors.

AIMS: The aim of this study was to report the outcomes of patients who underwent definitive surgery for secondary chondrosarcomas arising from osteochondromas. PATIENTS AND METHODS: A total of 51 patients with secondary chondrosarcomas occurring from osteochondromas were reviewed. Median age was 36 years (interquartile range (IQR) 15 to 82). Median follow-up was 6.9 years (IQR 2.8 to 10.6). The pelvis was the most commonly affected site (59%). Histological grades were grade I in 35 (69%), grade II in 13 (25%), and grade III in three patients (6%). RESULTS: Preoperative biopsy histology correctly predicted the final histological grade in 27% of patients. The ten-year disease-specific survival (DSS) for all patients was 89.4%. Local recurrence occurred in 15 patients (29%), more commonly in pelvic tumours (37%) compared with limb tumours (19%). Four patients with pelvic tumours died from progression of local recurrence. No patient with limb tumours died of disease. Wide/radical margin was associated with improved local recurrence-free survival (p = 0.032) and local recurrence was associated with worse DSS (p = 0.005). CONCLUSION: We recommend that a secondary chondrosarcoma arising from osteochondroma of the pelvis is resected with wide/radical resection margins. The balance between the morbidity of surgery and risk of local recurrence needs to be considered in patients with limb secondary chondrosarcomas. Cite this article: Bone Joint J 2019;101-B:1313-1320.

Electropermeabilization of metastatic chondrosarcoma cells from primary cell culture.

Primary cell cultures are challenging, but reliable model reflecting tumor response in vitro. The study was designed to examine if the increased electropermeabilization can overcame initial drug insensitivity in chondrosarcoma cells from lung metastasis. We established a primary cell culture and evaluated the cytotoxic impact of four drugs-cisplatin (CDDP), camptothecin, 2-methoxyestradiol, and leucovorin calcium (LeuCa). After determination of parameters allowing for electropermeabilization, we performed electrochemotherapy in vitro with the least toxic drugs-CDDP and LeuCa. Although combining CDDP and leucovorin together increased their toxicity and supported apoptosis, application of pulsed electric fields (PEFs) brought no advantage for their efficacy. The study emphasizes the need for introduction of primary cell cultures into studies on pulse electric fields as model frequently less sensitive to PEF-based treatments than continuous cell lines.

Regional Lymph Node Involvement Is Associated With Poorer Survivorship in Patients With Chondrosarcoma: A SEER Analysis.

BACKGROUND: Regional lymph node involvement is thought to be rare in patients with chondrosarcoma, but its actual prevalence is unclear. Additionally, it is often not considered when prognostic factors are analyzed in patients with chondrosarcoma. However, it has been well established that lymph node involvement is a poor prognostic marker in patients with many types of bone and soft tissue sarcoma, including rhabdomyosarcoma, osteosarcoma, and Ewing's sarcoma. Although lymph node metastases are rare among all sarcoma types, it is important to consider whether lymph node metastases should be assessed in patients with chondrosarcoma because these metastases may impact survival. QUESTIONS/PURPOSES: (1) What is the reported prevalence of regional lymph node involvement in patients with chondrosarcoma? (2) Do patients who have chondrosarcomas with regional lymph node involvement have different clinicopathologic presentations and survival than patients without regional lymph node involvement? (3) Is regional lymph node involvement independently associated with prognosis in patients with chondrosarcoma? METHODS: The data of patients with chondrosarcoma registered in the Surveillance Epidemiology and End Results database (SEER) (1988-2015) were analyzed for the reported prevalence of regional lymph node involvement and its relationship with clinicopathologic features and the 5-year overall survival rate. From 1988 to 2015, 5528 patients with chondrosarcoma were registered in the SEER database. After screening by the inclusion criterion-chondrosarcoma as the first primary tumor, diagnosis with histology confirmation, patients with active followup and available information about regional node status-3374 patients met the inclusion criteria and were analyzed. Demographics and clinicopathologic data were compared using chi-square or Fisher's exact tests. Logistic regression analysis was used to assess the adjusted odds ratio. The overall survival rate was estimated with Kaplan-Meier curves and log-rank tests. Univariate and multivariate analyses of overall survival were performed with Cox proportional hazard models. In addition, a series of sensitivity analyses were performed to assess the robustness of the final Cox proportional hazard model. RESULTS: Forty-four patients (1.3%) were recorded in the database as having regional lymph node involvement at the time of the primary diagnosis. Lymph node metastases were more likely to be reported in an extraskeletal primary site (3% [13 of 426] versus 1% [31 of 2948], adjusted odds ratio [OR] = 2.9, 95% CI, 1.5-5.8; p = 0.003) for bone primary sites and tumors with maximum diameter ≥ 8 cm (2% [26 of 1045] versus 1% [10 of 1075], adjusted OR = 2.9, 95% CI, 1.3-6.3; p = 0.008) and poorer differentiation (4% [24 of 608] versus 1% [14 of 2308], adjusted OR = 4.0, 95% CI, 2.0-8.2; p < 0.001), and in those with distant metastases (7% [14 of 203] versus 1% [30 of 3148], adjusted OR = 3.5, 95% CI, 1.7-7.1, p = 0.001). The 5-year overall survival rates of patients with and without regional lymph node involvement were 28% (95% CI, 15-42%) and 77% (95% CI, 75-78%), respectively (p < 0.001). After controlling for age, sex, race, grade, metastatic status, size, and histologic subtype, the presence of regional lymph node involvement was associated with poorer survival (hazard ratio, 2.20; 95% CI, 1.50-3.24; p < 0.001); this finding was confirmed in several sensitivity analyses. CONCLUSION: The prevalence of regional lymph node involvement in patients with chondrosarcoma was 1.3% in the SEER database. Although chondrosarcomas are rare, patients with chondrosarcomas who have regional node metastases have a poorer prognosis than those who have not reported to have them. This may underrepresent the true proportion of patients with lymph node metastases given the inaccuracies of reporting in this database, but we believe these findings indicate that clinicians should examine patients more carefully for chondrosarcoma with lymph node metastases. Future studies are needed to assess potential treatment strategies to improve the prognosis of these patients. LEVEL OF EVIDENCE: Level III, prognostic study.

Rare case of malignant transformation of a solitary spinal osteochondroma into recurrent metastatic chondrosarcoma.

Osteochondroma or osteocartilaginous exostosis is a commonly occurring primary tumor of the bone. Solitary spinal osteochondromas are, however, very rare, seen in only in 1-4% of all known cases and only few symptomatic cases have been reported in the literature so far. Further, while recurrence and malignant transformation are known to occur in osteochondroma, this is rare in the spine. We would like to report one such a case of an unusual presentation of recurrent, metastatic spinal chondrosarcoma in a patient with previous history of solitary cervical osteochondroma.

Chondrosarcoma of right big toe with metastases to left orbital and left infra temporal region.

To report a case of chondrosarcoma of right big toe with left orbital and left infra temporal metastases. Chondrosarcoma is the second most frequent primary malignant tumour of the bone. A 56 year old man had history of trauma on his right big toe, which was amputated and the biopsy in 2011 at Sindh Institute of Urology and Transplantation (SIUT) revealed chondrosarcoma with clear margins. Eventually the patient was presented with swelling of the left eye, pain and gradual loss of vision of that eye. Later a CT scan of his chest, brain and orbit showed pulmonary and pleural based nodule, with mediastinal and hilar lymphadenopathy representing metastatic deposit in left orbit, extending to left infra temporal region. A treatment of palliative chemotherapy was started with doxorubicin and ifosfamide, after which he was referred for radiotherapy. At that time he had loss of vision, pain and exopthalamus, and palliative radiotherapy was delivered to the left orbit with the prescribed dose of30 Gy/300cGy×10 fraction. Thereafter his case will be followed up at the oncology OPD after a 03 month interval.

An unusual presentation of metastatic chondrosarcoma on Tc99m MDP Bone scan.

Radionuclide bone scintigraphy with technetium-99m-methylene diphosphonate is the commonest procedure in nuclear medicine. It is an effective diagnostic tool in staging of primary bone tumours since long. It is not only helpful to confirm the radiologic features of malignant bone tumours but also demonstrates skeletal metastasis and soft tissue involvement. Computed tomography (CT) combined with single-photon emission computed tomography (SPECT/CT) give better insight to localize and categorize the lesions. We describe an interesting image of chondrosarcoma showing anatomical extent of primary tumour and multifocal uptake in the chest conforming to underlying widespread partially calcified pulmonary metastases.

Surgical management of primary malignant proximal fibular tumors: Functional and clinical outcomes of 23 patients.

OBJECTIVES: This study aims to evaluate the clinical characteristics and treatment outcomes of patients with primary malignant tumors located in the proximal fibula. PATIENTS AND METHODS: This retrospective study included 23 patients (15 males, 8 females; mean age 22.1 years; range, 9 to 63 years) with primary malignant tumors located in the proximal fibula between May 2007 and May 2017. The anamnesis or medical history, physical examination, plain chest radiography, lung computed tomography, direct radiograph, and magnetic resonance imaging of the affected extremity and routine laboratory tests of all patients were evaluated. RESULTS: Of the patients, 11 were diagnosed with osteosarcoma (47.8%), nine with Ewing's sarcoma (39.1%), two with chondrosarcoma (8.7%), and one was diagnosed with synovial sarcoma (4.3%). Pain and palpable mass were the most common symptoms. Six patients had lung metastases at the time of diagnosis. Of the patients, eight were performed Malawer type 1 resection (34.8%), nine type 2 resection (39.1%), four above knee amputation (17.4%), and two proximal tibia tumor resection prosthesis (8.6%). Mean follow-up duration was 36 months (range, 12 to 119 months). Local recurrence developed in three patients. Mean Musculoskeletal Tumor Society (MSTS) score of all patients was 62. CONCLUSION: Surgical treatment of primary malignant tumors of the proximal fibula is problematic. In appropriate indications, Malawer type 1 resection should be the treatment of choice due to lower local recurrence rates and higher MSTS scores.

Does Resection of the Primary Tumor Improve Survival in Patients With Metastatic Chondrosarcoma?

BACKGROUND: Although surgical resection or amputation has been the mainstay of localized chondrosarcoma management for many decades, its efficacy in patients with metastatic chondrosarcoma remains unknown, and likewise we do not know whether there are any tumor- or patient-related factors associated with better survival after surgery for metastatic chondrosarcoma. QUESTIONS/PURPOSES: (1) Is resection of the primary tumor associated with improved survival in patients with metastatic chondrosarcoma? (2) Which subgroups of patients with chondrosarcoma benefit more from resection in terms of survival? METHODS: We identified 200 of 222 patients with metastatic chondrosarcoma in the Surveillance, Epidemiology, and End Results (SEER) database between 1988 and 2014 based on the exclusion criteria. Among those patients, 107 (53.5%) underwent primary tumor resection or amputation. Patient information, including demographics (patient age, gender, race, year of diagnosis), tumor characteristics (primary site, histologic subtype, tumor grade, tumor size), and treatment (record of operation and radiation), was collected and included in the study. Kaplan-Meier analyses, log-rank tests, competing risks framework, multivariable Cox regression modeling, and interaction tests were conducted to assess the association of primary tumor resection and survival in the overall cohort and subgroups. RESULTS: Resection of the primary tumor was associated with improved overall survival (hazard ratio [HR], 0.481; 95% confidence interval [CI], 0.340-0.680; p < 0.001) and cancer-specific survival (HR, 0.493; 95% CI, 0.343-0.709; p < 0.001) after controlling for confounding variables. After controlling further for age, histologic subtype, and grade, primary tumor resection was associated with a survival advantage in patients with conventional subtype and Grade II chondrosarcoma (conventional subtype: HR, 0.403; 95% CI, 0.260-0.623 for overall survival and HR, 0.396; 95% CI, 0.250-0.627 for cancer-specific survival). However, primary tumor resection was not associated with increased survival in patients with metastatic chondrosarcoma who had the dedifferentiated subtype and Grade III malignancy. CONCLUSIONS: The present study demonstrates a possible favorable association between primary tumor resection and survival in some patients with metastatic chondrosarcoma at initial diagnosis. Specifically, patients with conventional subtypes and Grade II malignancies who underwent primary tumor resection had better survival compared with those patients who did not have primary tumor resection. Thus, there might be a benefit from primary tumor resection in these patients, but given the limitations of this database, further prospective studies or randomized trials are needed to confirm our findings. In the meantime, this information might be helpful to consider when discussing surgical options with patients who have conventional, Grade 2 metastatic chondrosarcoma at diagnosis. LEVEL OF EVIDENCE: Level III, therapeutic study.