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Study Design: Retrospective analysis., Objective: The aim of this study was to develop and validate a competing-risk-based prognostic model and a nomogram for predicting the three- and five-year probability of cancer-specific death (CSD) in patients with spinal and pelvic chondrosarcoma., Summary of Background Data: The issue of competing risk has rarely been addressed and discussed in survival analysis of bone sarcoma. In addition, the Fine and Gray model, a more accurate method for survival analysis in the context of competing risk, has also been less reported in prognostic study of chondrosarcoma., Methods: A total of 623 patients with spinal or pelvic chondrosarcoma were identified from the SEER database and were divided into a training and a validation cohort. These two cohorts were used to develop and validate a prognostic model to predict the 3- and 5-year probability of CSD, considering non-CSD as competing risk. The C-index, calibration plot, and decision curve analysis were used to assess the predictive performance and clinical utility of the model., Results: Older age (subdistribution hazards ratio [SHR]: 1.02, 95% confidence interval [CI]: 1.01∼1.03; P = 0.013), high grade (SHR: 2.68, 95% CI: 1.80∼3.99; P < 0.001), regional involvement (SHR: 1.66, 95% CI: 1.06∼2.58; P = 0.026), distant metastasis (SHR: 5.18, 95% CI: 3.11∼8.62; P < 0.001) and radical resection (SHR: 0.38, 95% CI: 0.24∼0.60; P < 0.001) were significantly associated with the incidence of CSD. These factors were used to build a competing-risk-based model and a nomogram to predict CSD. The C-index, calibration plot, and decision curve analysis indicated that the nomogram performs well in predicting CSD and is suitable for clinical use., Conclusion: A competing-risk based prognostic model is developed to predict the probability of CSD of patients with spinal and pelvic chondrosarcoma. This nomogram performs well and is suitable for clinical use.Level of Evidence: 4., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Nonepithelial tumors of the larynx are relatively rare neoplasms of the head and neck. The chondrosarcoma, which develops commonly from cricoid and thyroid cartilage, stands for 0.2% of the laryngeal malignant neoplasms. The rhabdomyoma is even more uncommon benign tumor developing from the laryngeal striated muscles. The clinical manifestation and the treatment options depend on the histopathological evaluation, tumor localization, and its size. In presented case, the simultaneous occurrence of benign and malignant tumors of mesenchymal origin in the patient's larynx was provoking hoarseness, globus sensation, and dysphagia. To the best of authors' knowledge, no other case of the concomitance of rhabdomyoma and chondrosarcoma of the larynx have been reported in the literature.

Chondrosarcomas are the second most common malignant bone tumor. Activating promoter mutations in telomerase reverse transcriptase (TERT) was recently described by us and others as a frequent mutation in high-grade chondrosarcoma. In this study, we investigate the prognostic significance of TERT promoter mutations in 241 chondrosarcomas from 190 patients collected over 24 years (1994-2017). The TERT promoter was sequenced after microdissection of 135 chondrosarcomas from 106 patients in addition to data from our previous cohort. The TERT promoter mutation at -124 C > T was found in 45% of all patients and was significantly associated (p > 0,001) with higher tumor grade, shorter metastasis-free survival, and disease-specific survival. Additionally, TERT promoter-mutated tumors were associated with a more aggressive metastatic pattern. Shorter survival was observed in patients with wild-type primary tumors who developed a mutated metastasis indicative of tumor progression. Primary tumor genetic heterogeneity and altering mutational status between nonsynchronous metastatic lesions suggests that chondrosarcoma is a multiclonal disease progressing through a branching evolution. Conclusion: TERT promoter mutation seems to be a central event in chondrosarcoma progression with association to metastatic disease and disease-related mortality. As an easily analyzed marker, there is future potential to utilize TERT promoter mutation status as a prognostic marker and investigate telomerase-targeted therapy in chondrosarcomas., (© 2021. The Author(s).)

Objectives: Chordomas are rare malignant tumors with a broad differential diagnosis, including chondrosarcomas and metastatic carcinomas. Recently, insulinoma-associated protein 1 (INSM1) has gained great interest regarding the diagnosis of neuroendocrine tumors but also extraskeletal myxoid chondrosarcomas. However, its expression in chordomas remains largely unknown., Methods: We retrospectively examined 57 chordomas for INSM1 expression., Results: INSM1 expression was found in only 5% of tumors., Conclusions: This marker is rarely expressed in this type of tumor, raising questions about neuroendocrine differentiation., (© American Society for Clinical Pathology, 2021. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Introduction: Chondromyxoid fibroma is a rare bone tumor characterized by immature myxoid mesenchymal tissue showing early primitive cartilaginous differentiation. There have been limited case reports describing the cytologic features of chondromyxoid fibroma. Herein, we reported cytologic features of chondromyxoid fibroma on fine-needle aspiration (FNA)., Materials and Methods: We performed a retrospective search in our cytopathology and surgical pathology database for cases diagnosed as chondromyxoid fibroma that had corresponding cytology specimens from three medical institutions. All available cytopathology specimens were reviewed., Results: Eight cases were retrieved from patients aged 16-77 years (mean, 51 years), and M:F ratio of 1.7:1. Seven tumors (88%) were primary, and most (62%) occurred in flat bones. Cytologic diagnoses were made in 6 cases with cytologic slides not available to review in 1 case. All cases showed metachromatic matrix in the background, while hyaline cartilage fragments were absent. All cases had two cell populations comprising oval to round cells and stellate to spindle cells. The spindle to stellate cells were more commonly embedded in matrix material. Moderate atypia (hyperchromasia and moderate anisonucleosis) was present in 4 cases (80%), while no mitotic figure was present in all cases., Conclusions: Our study highlights common cytologic features of chondromyxoid fibroma, including the presence of the spindle or stellate cells embedded in matrix material. Hyaline cartilage is uncommon and, if present, diagnostic considerations should include enchondroma or low-grade chondrosarcoma. A specific cytologic diagnosis primarily using FNA samples can be challenging but possible when evaluated in conjunction with clinical and radiologic data., (Copyright © 2021 American Society of Cytopathology. Published by Elsevier Inc. All rights reserved.)

Background: The prognosis of lung metastasis (LM) in patients with chondrosarcoma was poor. The aim of this study was to construct a prognostic nomogram to predict the risk of LM, which was imperative and helpful for clinical diagnosis and treatment., Methods: Data of all chondrosarcoma patients diagnosed between 2010 and 2016 was queried from the Surveillance, Epidemiology, and End Results (SEER) database. In this retrospective study, a total of 944 patients were enrolled and randomly splitting into training sets (n = 644) and validation cohorts(n = 280) at a ratio of 7:3. Univariate and multivariable logistic regression analyses were performed to identify the prognostic nomogram. The predictive ability of the nomogram model was assessed by calibration plots and receiver operating characteristics (ROCs) curve, while decision curve analysis (DCA) and clinical impact curve (CIC) were applied to measure predictive accuracy and clinical practice. Moreover, the nomogram was validated by the internal cohort., Results: Five independent risk factors including age, sex, marital, tumor size, and lymph node involvement were identified by univariate and multivariable logistic regression. Calibration plots indicated great discrimination power of nomogram, while DCA and CIC presented that the nomogram had great clinical utility. In addition, receiver operating characteristics (ROCs) curve provided a predictive ability in the training sets (AUC = 0.789, 95% confidence interval [CI] 0.789-0.808) and the validation cohorts (AUC = 0.796, 95% confidence interval [CI] 0.744-0.841)., Conclusion: In our study, the nomogram accurately predicted risk factors of LM in patients with chondrosarcoma, which may guide surgeons and oncologists to optimize individual treatment and make a better clinical decisions., Trial Registration: JOSR-D-20-02045, 29 Dec 2020.

Bone sarcomas, although rare, are associated with significant morbidity and mortality. The most frequent primary bone cancers include osteosarcoma, chondrosarcoma and Ewing sarcoma. The treatment approaches are heterogeneous and mainly chosen based on precise tumour staging. Unfortunately, clinical outcome has not changed significantly in over 30 years and tumour grade is still the best prognosticator of metastatic disease and survival. An option to improve this scenario is to identify molecular biomarkers in the early stage of the disease, or even before the disease onset. Blood-based liquid biopsies are a promising, non-invasive way to achieve this goal and there are an increasing number of studies which investigate their potential application in bone cancer diagnosis, prognosis and personalised therapy. This review summarises the interplay between clinical and molecular aspects of the three main bone sarcomas, alongside biomarker discovery and promising applications of liquid biopsy in each tumour context., (Copyright © 2021 Elsevier B.V. All rights reserved.)

Background: Chondrosarcomas of the skull base are rare intracranial tumors of chondroid origin. The rarity of these lesions has made it difficult to form a consensus on optimal treatment regimens. The aim of this study was to provide a comprehensive analysis of prognostic factors, treatment modalities, and survival outcomes in patients with chondrosarcoma of the skull base., Methods: Patients with diagnosis codes for chondrosarcoma of the skull base were queried from the National Cancer Database for the years 2004-2016. Outcomes were investigated using Cox univariate and multivariate regression analyses, and survival curves were generated for comparative visualization., Results: A total of 718 patients with chondrosarcoma of the skull base were identified. Mean overall survival (OS) in these patients was 10.7 years. Older age and presence of metastases were associated with worsened OS. Of patients, 83.3% received surgical intervention, and both partial resection and radical resection were associated with significantly improved OS (P < 0.001). Neither radiotherapy nor chemotherapy improved OS; however, patients who received proton-based radiation and patients who received high-dose radiation (≥6000 cGy) had significantly improved OS compared with patients who received traditional radiation., Conclusions: In the largest study to our knowledge of skull base chondrosarcoma to date, both partial resection and radical resection significantly improved OS, thus supporting the goal of maximal safe resection to preserve vital neurovascular structures without sacrificing outcome. In patients who received radiotherapy, proton-based modalities and high-dose radiation were associated with increased OS., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Introduction: Advances in the genetics of soft tissue neoplasia have allowed for the diagnostic recognition of specific tumor types from small biopsy specimens, including those procured using the fine needle aspiration (FNA) biopsy technique. Extraskeletal myxoid chondrosarcoma (EMC) is a malignant mesenchymal neoplasm characterized by NR4A3 and, less specifically, by EWSR1 gene rearrangements. A series of EMC cytologic specimens was examined to demonstrate the diagnostic value of incorporating fluorescence in situ hybridization (FISH) testing in cytologic cases of suspected EMC., Materials and Methods: A search was made of our cytopathology and surgical pathology databases for cases diagnosed as EMC. FNA biopsy cytology, exfoliative cytology, imprint cytology, and FISH analysis were performed and examined using standard techniques., Results: A total of 16 cases of EMC were retrieved from 15 patients (male/female ratio, 2.8:1; mean age, 62 years). Of the 15 patients, 10 were new patients with primary tumors, 2 had locally recurrent tumors, and 4 had metastases. The sites included the extremities in 10 cases, the trunk in 4, serous effusion in 1, and a mediastinal lymph node in 1 case. The specific cytologic diagnoses were EMC (14 cases; 88%), suspicious for EMC (n = 1), and malignant cells (n = 1). All cases for which FISH testing was successfully used were specifically recognized as EMC. Aspirates and imprint smears consisted of uniformly rounded cells set in an opaque myxoid/chondromyxoid stroma (less abundant and more diaphanous in the effusion sample), sometimes arranged in short anastomosing cords. FNA of 1 case of an EMC cellular variant mimicked a malignant small rounded cell tumor., Conclusion: EMC can be added to the growing list of soft tissue neoplasms that are specifically recognizable using cytopathology, coupled with judicious application of ancillary molecular testing., (Copyright © 2020 American Society of Cytopathology. Published by Elsevier Inc. All rights reserved.)

Introduction: The accurate diagnosis of musculoskeletal neoplasms is difficult but a pattern-based approach combined with ancillary testing has been shown to improve diagnostic accuracy. The pattern-based approach is particularly appropriate for myxoid lesions., Materials and Methods: The authors reviewed their personal experience of over 3 decades of diagnosing myxoid neoplasms of musculoskeletal lesions., Results: The authors found that myxoid lesions can be accurately classified based on cell type, nuclear atypia, presence of blood vessel fragments, as well as the results of immunohistochemical and molecular testing., Conclusions: Musculoskeletal lesions with a prominence of myxoid or chondroid material in the background can be accurately diagnosed using pattern analysis and ancillary testing., (Copyright © 2020 American Society of Cytopathology. Published by Elsevier Inc. All rights reserved.)

Objective: The aim of this study is to assess the association between clinical and radiological features as well as of isocitrate dehydrogenase 1 and 2 ( IDH 1,2) mutations with outcome in head and neck chondrosarcomas., Study Design: Retrospective study., Setting: Tertiary referral center., Methods: Clinical, histological, and molecular data of patients with head and neck chondrosarcomas treated by surgery were collected., Results: Forty-six patients were included. The mean age at diagnosis was 56 years (range, 17-78). The tumor originated from the skull base (52.2%), facial bones (28.2%), or laryngotracheal area (19.6%). At last follow-up (median 52.5 months), 38 patients were alive, 30 of which were disease free, whereas 8 had died, 4 of disease progression and 4 of other causes. Fourteen (30.4%) had local recurrence and 2 (4.3%) had lung metastasis. All cases were negative for cytokeratin AE1/AE3, brachyury, and IDH1 at immunohistochemistry, while Sanger sequencing identified IDH1/2 point mutations, typically IDH1 R132C, in 9 (37.5%) tumors arising from the skull base. Margin infiltration on the surgical specimen negatively affected the outcome, whereas no correlation was identified with IDH mutation status., Conclusions: An adequate margin positively affects survival. IDH mutation status does not affect patient outcome.

Bone tumors of the foot are an uncommon finding. Most tumors are found incidentally on imaging and are benign. Care must be taken although due to the aggressive nature of malignant bone tumors that can occur in the calcaneus. Malignant lesions will more commonly present with symptoms of pain and swelling. Often misdiagnosed as soft tissue injuries, it is critical to be able to diagnose and treat these lesions early. Imaging plays an important role with plain films and advanced imaging. Surgical treatments can range from curettage with grafting to amputation for more aggressive lesions., Competing Interests: Disclosure The authors have nothing to disclose., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Objectives: Survival in patients with chondrosarcomas has not improved over 40 years. Although emerging evidence has documented the efficacy of navigation-assisted surgery, the prognostic significance in chondrosarcomas remains unknown. We aimed to assess the clinical benefit of navigation-assisted surgery for pelvic chondrosarcomas involving the peri-acetabulum., Methods: We studied 50 patients who underwent limb-sparing surgery for periacetabular chondrosarcomas performed with navigation (n = 13) without it (n = 37) at a referral musculoskeletal oncology centre between 2000 and 2015., Results: The intralesional resection rates in the navigated and non-navigated groups were 8% (n = 1) and 19% (n = 7), respectively; all bone resection margins were clear in the navigated group. The 5-year cumulative incidence of local recurrence was 23% and 56% in the navigated and non-navigated groups, respectively (p = 0.035). There were no intra-operative complications related to use of navigation. There was a trend toward better functional outcomes in the navigated group (mean MSTS score, 67%) than the non-navigated group (mean MSTS score, 60%; p = 0.412). At a mean follow-up of 63 months, the 5-year disease-specific survival was 76% and 53% in the navigated and non-navigated group, respectively (p = 0.085), whilst the 5-year progression-free survival was 62% and 28% in the navigated and non-navigated group, respectively (p = 0.032)., Conclusion: This study confirmed improved local control and progression-free survival with the use of computer navigation in patients with limb-salvage surgery for periacetabular chondrosarcomas, although the advancement in other treatment modalities is required for improvement of disease-specific survival., Competing Interests: Declaration of competing interest The authors have no conflicts of interest to declare., (Copyright © 2020 Elsevier Ltd, BASO ~ The Association for Cancer Surgery, and the European Society of Surgical Oncology. All rights reserved.)

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., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Clear-cell chondrosarcomas (CCCSs) constitute a very rare subtype of chondrosarcoma. CCCS may radiologically mimic chondroblastoma, and given the difference in surgical approach, it is important to distinguish these two entities preoperatively., Design: Using the institutional digital records, we identified histologically verified CCCS between 1996 and 2013, where preoperative fine-needle aspiration (FNA) cytology was available. Clinical characteristics were categorized and described, and FNAs were reviewed by a panel of senior cytopathologists. In addition, corresponding radiological imaging was reviewed by senior radiologists, and a literature review on CCCS and chondroblastoma was conducted., Results: A total of seven CCCS FNAs were identified from six patients. The cytomorphology showed low to intermediate cellular smears of clusters and single round or oval tumor cells. Tumor cells had rounded (sometimes binucleated) nuclei with limited pleomorphism and rich vacuolated cytoplasm. Chondroid background matrix was always found. While CCCS patients had a significantly higher age at diagnosis compared to chondroblastoma, no age cut-off would distinctly separate the two., Conclusions: CCCS has distinguished cytomorphological features on FNA smears. CCCS should be considered as a possible differential diagnosis in adults (>25 years) with a radiological suspicion of chondroblastoma. Since radiology and patient age cannot conclusively distinguish CCCS from chondroblastoma, FNA may prove an important tool for correct preoperative diagnosis of CCCS., (© 2020 The Authors. Diagnostic Cytopathology published by Wiley Periodicals LLC.)

Introduction: Primary chest wall sarcoma is a rare entity. It can be classified based on its origin, as bone sarcomas or soft tissue sarcomas. Various prognostic factors have been studied in different case series like age, sex, tumor histology, grade, resection margin status, adjuvant treatment, and others. The present study aimed to analyze common histological types, their management by resection and reconstruction and prognosis, in cases presenting at a regional cancer center in western India., Material and Method: This was an observational study from a prospectively maintained database. 57 patients with chest wall sarcoma treated with curative intent between January 2016 till January 2019 with a minimum follow-up of 3 months were included in the study. The goals of surgical treatment were to obtain a wide resection margin of 3-4 cm, preserve the function of the chest wall and provide stability and rigidity to protect intrathoracic organs., Results: The median follow-up of the present patient's cohort was for 20.2 months. Overall two-year survival was 74.7%. Two-year OS and DFS of bone sarcoma were 62.3% and 35% and soft tissue sarcomas were 91% and 71.3%. Ewing's sarcoma had the worst two-year overall survival of 50.6% and chondrosarcoma and fibromatosis had 100% two-year overall survival., Conclusion: Chest wall sarcoma forms a heterogeneous group of tumors. In the present study, Ewing's sarcoma was the most common histology with the worst survival, since they presented in advanced stages. Management should be multidisciplinary and surgical resection should be aggressive to achieve an R0 resection. Reconstruction of chest wall should aim to provide structural and functional stability with minimal morbidity. Frozen section assessment should be utilized whenever in doubt., (Copyright © 2021. Published by Elsevier Ltd.)

Purpose: The spectrum of diagnoses and clinical features of hand tumors differ from those of tumors in other body parts. However, only a few reports have comprehensively referenced the diagnosis and clinical features of hand tumors. This study aimed to elucidate the diagnostic distribution and the clinical features of hand tumors undergone surgery in our institute., Patients and Methods: A total of 235 lesions in 186 patients diagnosed with hand tumors between 1978 and 2020 were reviewed. Age at surgery, gender, chief complaint, tumor location, and pathological diagnosis were analyzed., Results: There were 121 benign bone tumors, 98 benign soft tissue tumors, and 16 malignant tumors. Chondroma and tenosynovial giant cell tumor were common benign bone and soft tissue tumors at the proximal phalanx of the ring finger and the palm, respectively. Meanwhile, chondrosarcoma and synovial sarcoma were common malignant tumors at the dorsal part of the hand. Local pain and painless mass were the chief complaints in patients with benign bone and soft tissue tumors, respectively. Most patients with malignant tumors were referred after unplanned resection. When patients were classified into two categories by tumor size according to maximal diameter, tumors larger than 19 mm had a significantly higher risk of malignant ( p = 0.031) despite being smaller than other tumors in different body parts., Conclusion: When a tumor malignancy is suspected, the patient should be referred to a specialist to avoid unplanned resection or delayed diagnosis due to misdiagnosis. Knowing the distribution and clinical features should help in diagnosing hand tumors.

There has been an increasing interest and enormous applications in three-dimensional (3D) printing technology and its prosthesis, driving many orthopaedic surgeons to solve the difficult problem of bony defects and explore new ways in surgery approach. However, the most urgent problem is without an effective prosthesis and standard treatment strategy. In order to resolve these problems, this study was performed to explore the use of a 3D-printed anatomically conforming pelvic prosthesis for bony defect reconstruction following tumor resection and to describe a detailed treatment flowchart and the selection of a surgical approach. Six patients aged 48-69 years who had undergone pelvic tumor resection underwent reconstruction using 3D-printed anatomically conforming pelvic prostheses according to individualized bony defects between March 2016 and June 2018. According to the Enneking and Dunham classification, two patients with region I+II tumor involvement underwent reconstruction using the pubic tubercle-anterior superior iliac spine approach and the lateral auxiliary approach and one patient with region II+III and three patients with region I+II+III tumor involvement underwent reconstruction using the pubic tubercle-posterior superior iliac spine approach. The diagnoses were chondrosarcoma and massive osteolysis. After a mean follow-up duration of 30.33 ± 9.89 months (range, 18-42), all patients were alive, without evidence of local recurrence or distant metastases. The average blood loss and blood transfusion volumes during surgery were 2500.00 ± 1461.51 ml (range, 1200-5000) and 2220.00 ± 1277.62  ml (range, 800-4080), respectively. During follow-up, the mean visual analogue scale (VAS) score decreased, and the mean Harris hip score increased. There were no signs of hip dislocation, prosthetic loosening, delayed wound healing, or periprosthetic infection. This preliminary study suggests the clinical effectiveness of 3D-printed anatomically conforming pelvic prostheses to reconstruct bony defects and provide anatomical support for pelvic organs. A new surgical approach that can be used to expose and facilitate the installation of 3D-printed prostheses and a new treatment strategy are presented. Further studies with a longer follow-up duration and larger sample size are needed to confirm these encouraging results., Competing Interests: There are no conflicts of interest to declare., (Copyright © 2020 Wei Peng et al.)

Background and Objectives: Malignant tumors of the calcaneus are rare but pose a treatment challenge., Aims: (1) describe the demographics of calcaneal malignancies in a large cohort; (2) describe survival after amputation versus limb-salvage surgery for high-grade tumors., Methods: Study group: a "pooled" cohort of patients with primary calcaneal malignancies treated at two cancer centers (1984-2015) and systematic literature review. Kaplan-Meier analyses described survival across treatment and diagnostic groups; proportional hazards modeling assessed mortality after amputation versus limb salvage., Results: A total of 131 patients (11 treated at our centers and 120 patients from 53 published studies) with a median 36-month follow-up were included. Diagnoses included Ewing sarcoma (41%), osteosarcoma (30%), and chondrosarcoma (17%); 5-year survival rates were 43%, 73% (70%, high grade only), and 84% (60%, high grade only), respectively. Treatment involved amputation in 52%, limb salvage in 27%, and no surgery in 21%. There was no difference in mortality following limb salvage surgery (vs. amputation) for high-grade tumors (HR 0.38; 95% CI 0.14-1.05), after adjusting for Ewing sarcoma diagnosis (HR 5.15; 95% CI 1.55-17.14), metastatic disease at diagnosis (HR 3.88; 95% CI 1.29-11.64), and age (per-year HR 1.04; 95% CI 1.02-1.07)., Conclusions: Limb salvage is oncologically-feasible for calcaneal malignancies., (© 2020 Wiley Periodicals LLC.)

Background: Karyopherin α2 (KPNA2), a member of the karyopherin α family, has been studied in several cancers but has not yet been substantially investigated in malignant bone tumors. The purpose of the current study was to evaluate the KPNA2 expression level and its utility as a novel diagnostic biomarker in osteosarcomas and malignant bone tumor mimics, such as chondrosarcomas and Ewing sarcomas (ESs)., Method: We investigated the expression of KPNA2 protein by immunohistochemistry on paraffin-embedded surgical specimens from 223 patients with malignant and benign bone tumors, including 81 osteosarcomas, 42 chondrosarcomas, 15 ESs, 28 osteoid osteomas, 20 osteochondromas and 37 chondroblastomas. Immunoreactivity was scored semiquantitatively based on staining extent and intensity., Results: Sixty-seven of 81 (82.7%) osteosarcoma, zero of 42 (0%) chondrosarcoma and one of 15 (6.7%) ES samples showed immunoreactivity for KPNA2. Negative KPNA2 expression was observed in all benign bone tumors. The expression of KPNA2 in osteosarcoma samples was much higher than that in chondrosarcoma and ES samples (P < 0.001). The sensitivity and specificity of KPNA2 immunoexpression for detecting osteosarcoma were 82.7 and 100%, respectively. Several subtypes of osteosarcoma were analyzed, and immunostaining of KPNA2 was frequent in osteoblastic samples (90.9%), with 39 samples (70.9%) showing strong-intensity staining. KPNA2 positivity was observed in ten of 13 (76.9%) chondroblastic, two of 6 (33.3%) fibroblastic, three of 4 (75%) telangiectatic and two of 3 (66.7%) giant cell-rich osteosarcoma samples. The strongest intensity staining was observed in osteoblastic osteosarcoma., Conclusion: KPNA2 is frequently expressed in osteosarcomas, particularly in osteoblastic and chondroblastic tumors, but is rarely positive in chondrosarcomas and ESs. This feature may aid in distinguishing between osteosarcoma and other bone sarcoma mimics. This report supports KPNA2 as a novel marker for the diagnosis of osteosarcoma.

Acquired unilateral alacrima as a presenting sign of an intracranial tumor is exceptionally rare, and only described once previously in a case of nasopharyngeal carcinoma. The authors present a 32-year-old female patient who presents with a year-long history of alacrima and arhinorrhea. She was subsequently diagnosed with a petroclival chondrosarcoma extending into Meckel's cave and the cavernous sinus and underwent surgical debulking. To the authors' knowledge, this is the first reported case of acquired unilateral alacrima as a presenting feature of a skull base chondrosarcoma. This case serves to remind general ophthalmologists and oculoplastic surgeons alike that acquired alacrima may be the presenting feature of serious intracranial disease.

Background: Extraskeletal myxoid chondrosarcoma (EMCS) is a rare tumor that typically has an indolent course but high rate of recurrence. We queried the Surveillance, Epidemiology, and End Results (SEER) database to assess factors associated with metastasis, treatment, and survival., Methods: We queried the SEER 1973-2016 database for patients with myxoid chondrosarcoma (ICD-O-3: 9231/3). Kaplan-Meier analyses and Cox proportional hazard models assessed effects on overall survival (OS) of demographics and clinical characteristics. Logistic regression assessed associations between tumor location and distant disease. Primary analysis was a complete case analysis; multiple imputation (MI) was used in a sensitivity analysis., Results: Locoregional disease (LRD) was found in 373 (85%) of patients. In univariate analysis with LRD, surgery correlated with superior OS [HR = 0.27; 95% confidence interval (CI), 0.16-0.47]; chemotherapy and radiotherapy associated with inferior OS (HR = 1.90; 95% CI, 1.11-3.27 and HR = 1.45; 95% CI, 1.03-2.06, respectively). No treatment modality associated with OS in the adjusted, complete case model. In the adjusted sensitivity analysis, surgery associated with superior outcomes (HR = 0.36; 95% CI, 0.19-0.69). There was no OS difference by primary tumor site. 10-year OS with distant disease was 10% (95% CI, 2%-25%)., Conclusions: Surgery in LRD associated with improved OS in univariate analysis and adjusted models correcting for missing data. There was no OS benefit with chemotherapy or radiotherapy., Impact: This represents the largest report of EMCS with long-term follow-up. Despite the reputedly indolent nature of EMCS, outcomes with metastatic disease are poor. We provide OS benchmarks and guidance for stratification in future prospective trials., (©2020 American Association for Cancer Research.)

Ollier disease is a rare congenital disease in which multiple enchondromas occur. The tumors can transform to malignant chondrosarcomas of various histologic grades. The patient we described has been treated in our orthopedic department six times, always being referred on account of new lesions. The tumors were excised with margins of healthy tissue. Each tumor was subjected to a histological examination to determine its type and grade. Chondroid tumors should be diagnosed carefully, because the treatment depends on their histologic features. If surgery is performed, removal of the tumor with a margin of healthy tissue is crucial for the patient's well-being and good prognosis.

Aims: To evaluate the available diagnostic histological criteria for synovial chondrosarcoma and to screen for the presence of IDH1/IDH2 mutations in a series of cases of this malignant cartilaginous neoplasm., Methods and Results: Ten cases of synovial chondrosarcoma diagnosed at our institute were reviewed. At presentation, all tumours occurred in adults (median age, 62 years). The most common location was the knee joint (five cases), and the size at diagnosis ranged from 30 mm to 170 mm. Eight patients had secondary synovial chondrosarcomas associated with pre-existing/recurrent or concomitant synovial chondromatosis. Five patients had local recurrences and three had lung metastases. All patients with intralesional excisions developed local recurrences, whereas those who underwent wide resections did not. At last follow-up (mean, 91 months), available for nine patients, seven patients were alive and disease-free, one patient had died of disease, and one was alive with paravertebral metastases. Frequent histological features observed included loss of clustering of chondrocytes (nine cases), the presence of variable amounts of myxoid matrix (eight cases), peripheral hypercellularity (eight cases), tumour necrosis (six cases), and spindling of chondrocytes (four cases). Of the seven cases for which it was possible to evaluate bone permeation, six showed infiltration of bone marrow. All seven cases screened for mutations of exon 4 of IDH1 and IDH2 were found to be wild-type., Conclusions: Histological criteria in correlation with clinical and radiological features allow the recognition of synovial chondrosarcoma. IDH1/IDH2 mutations were not present in synovial chondrosarcoma. Adequate surgical margins are important for disease control., (© 2020 John Wiley & Sons Ltd.)

Aim: To retrospectively review a series of solitary central cartilage tumours (CCTs) of the proximal fibula and propose an imaging protocol for their management., Materials and Methods: Over 11 years, 48 patients were identified with a CCT of the proximal fibula. The initial magnetic resonance imaging (MRI) and follow-up imaging were assessed for tumour length, endosteal scalloping, or aggressive/malignant features. Each case was categorised according to the Birmingham Atypical Cartilage Tumour Imaging Protocol (BACTIP). The final diagnosis and outcome were reviewed to establish an appropriate imaging protocol for the management of similar cases in the future., Results: The final diagnosis based on imaging alone or imaging and histopathology was enchondroma in 46 cases (96%) and atypical cartilage tumour (ACT)/low-grade chondrosarcoma in two (4%). Using the BACTIP classification, the majority were type IC (48%) with type IA the second commonest (24%). Of 19 that underwent follow-up MRI, only two revealed any increase in length, neither of which was shown to be malignant. No cases re-presented later with evidence of malignant transformation., Conclusion: There has been a significant increase in the number of CCTs of the proximal fibula referred to Royal Orthopaedic Hospital, Birmingham, over the past decade. This can largely be attributed to the increased use of MRI. The majority were benign with only 4% classified as an ACT. A modified BACTIP, the Fibular Atypical Cartilage Tumour Imaging Protocol (FACTIP) is presented, indicating which cases would benefit from follow-up imaging and which require referral to a specialist orthopaedic oncology service., (Copyright © 2020 The Royal College of Radiologists. Published by Elsevier Ltd. All rights reserved.)

Undifferentiated/dedifferentiated carcinoma is an aggressive endometrial carcinoma which remains underrecognized but may account for up to 9% of all endometrial malignancies. We describe 3 cases in which the undifferentiated component was associated with sarcomatous differentiation, characterized by spindled cells in 2 cases and heterologous malignant cartilage in 1 case. Two of the 3 cases demonstrated mismatch repair deficiency by immunohistochemistry. This phenomenon has not previously been formally reported and increases the likelihood of misdiagnosis, especially within biopsy samples; differential diagnoses may include endometrial stromal sarcoma and grade 3 endometrioid adenocarcinoma with spindled morphology. We review the current literature and provide strategies for resolving the differential diagnoses, with a suggested panel of antibodies which includes EMA, E-cadherin, and mismatch repair proteins as approximately 50% of cases show loss of mismatch repair expression.

Chondrosarcoma is a malignant tumor which often affects the pelvic ring and its symptomathology is non-specific and insidious. We display a case of a right iliopubic branch chondrosarcoma in a 62-year-old male whose first sing was a four-year history of sporadic macroscopic haematuria, related to efforts. After being studied by the Urology Department by cystoscopy and biopsy of bladder tumor, chondral cells were appreciated. Further study with imaging tests diagnosed low-grade chondrosarcoma with bladder infringement. The case is assessed by Musculoskeletal Tumors Committee and a multidisciplinary approach is carried out through en bloc resection and pelvic floor reconstruction. Currently the patient remains asymptomatic.

Background and purpose - Adequate staging of chondroid tumors at diagnosis is important as it determines both treatment and outcome. This systematic review provides an overview of MRI criteria used to differentiate between atypical cartilaginous tumors (ACT) and high-grade chondrosarcoma (HGCS).Patients and methods - For this systematic review PubMed and Embase were searched, from inception of the databases to July 12, 2018. All original articles describing MRI characteristics of pathologically proven primary central chondrosarcoma and ACT were included. A quality appraisal of the included papers was performed. Data on MRI characteristics and histological grade were extracted by 2 reviewers. Meta-analysis was performed if possible. The study is registered with PROSPERO, CRD42018067959.Results - Our search identified 2,132 unique records, of which 14 studies were included. 239 ACT and 140 HGCS were identified. The quality assessment showed great variability in consensus criteria used for both pathologic and radiologic diagnosis. Due to substantial heterogeneity we refrained from pooling the results in a meta-analysis and reported non-statistical syntheses. Loss of entrapped fatty marrow, cortical breakthrough, and extraosseous soft tissue expansion appeared to be present more often in HGCS compared with ACT.Interpretation - This systematic review provides an overview of MRI characteristics used to differentiate between ACT and HGCS. Future studies are needed to develop and assess more reliable imaging methods and/or features to differentiate ACT from HGCS.

Purpose: Postoperative radiation therapy (poRT) of intracranial/skull base chondrosarcomas (CHSs) is standard treatment. However, consensus is lacking for poRT in extracranial CHS (eCHS) owing to their easier resectability and intrinsic radioresistance. We assessed the practice and efficacy of poRT in eCHS., Methods and Materials: This multicentric retrospective study of the French Sarcoma Group/Rare Cancer Network included patients with eCHS who were operated on between 1985 and 2015. Inverse propensity score weighting (IPTW) was used to minimize poRT allocation biases., Results: Of 182 patients, 60.4% had bone and 39.6% had soft-tissue eCHS. eCHS were of conventional (31.9%), myxoid (28.6%; 41 extraskeletal, 11 skeletal), mesenchymal (9.9%), or other subtypes. En-bloc surgery with complete resection was performed in 52.6% and poRT in 36.8% of patients (median dose, 54 Gy). Irradiated patients had unfavorable initial characteristics, with higher grade and incomplete resection. Median follow-up time was 61 months. Five-year incidence of local relapse was 10% with poRT versus 21.6% without (P = .050). Using the IPTW method, poRT reduced the local relapse risk (hazard ratio, 0.27; 95% confidence interval, 0.14-0.52; P < .001). Five-year disease-free survival (DFS) was 71.8% with poRT and 64.2% without (P = .680). Using the IPTW method, poRT improved DFS (hazard ratio, 0.51; 95% confidence interval, 0.30-0.85; P = .010). The benefit of poRT on local relapse and DFS was confirmed after exclusion of the extraskeletal subtype. There was no difference in overall survival. Prognostic factors of poorer DFS in multivariate analysis were deeper location, higher grade, incomplete resection, and no poRT., Conclusions: poRT should be offered in patients with eCHS and high-grade or incomplete resection, regardless of the histologic subtype., (Copyright © 2020 Elsevier Inc. All rights reserved.)

The three most common primary bone cancers are osteosarcoma, Ewing sarcoma, and chondrosarcoma. Osteosarcoma occurs most often in children and young adults, with a peak incidence at ages 10 to 14 years. It also can occur later in life due to malignant transformation of benign bone lesions. Osteosarcoma occurs most commonly around the knee, but can occur in other bones. Management varies depending on tumor characteristics and involves chemotherapy and surgery. Ewing sarcoma is most common in teenagers. It occurs most commonly in long bones but can occur in the pelvis and other bones. Management involves surgical resection when possible, along with chemotherapy and occasionally radiation therapy. Chondrosarcoma typically occurs in patients 40 years and older. It can occur as a primary tumor or from malignant transformation of benign bone tumors. Chondrosarcomas are relatively resistant to chemoradiation, so surgery is the standard therapy. When any of these tumors is suspected, patients should be instructed to avoid weight-bearing on the affected extremity to help prevent pathologic fracture while evaluation is completed. Imaging with x-rays and occasionally magnetic resonance imaging study are the initial diagnostic steps. If imaging suggests a primary bone cancer, prompt referral to an orthopedic oncology subspecialist is indicated., (Written permission from the American Academy of Family Physicians is required for reproduction of this material in whole or in part in any form or medium.)

Primary chondrosarcoma of the trachea is an extremely rare tumor. We report two cases of tracheal chondrosarcoma describing the role of surgical and conservative treatment. Endoscopic treatment with rigid bronchoscopy was performed in both patients to restore airway patency and obtain histological specimens for diagnosis. One of the patients subsequently underwent successful tracheal resection and reconstruction. The other patient, who had a contraindication to surgical treatment due to associated diseases underwent iterative endoscopic LASER treatment and is alive three years after the first diagnosis. Surgical treatment remains the treatment of choice of tracheal chondrosarcoma. When surgery is contraindicated endoscopic treatment may allow relatively longterm survival due to the slow growth of these tumors.

Synovial chondromatosis (SC) is a rare benign cartilaginous neoplasm in which recurrent fibronectin 1 (FN1) and activin receptor 2A (ACVR2A) gene rearrangements have been recently reported. Triggered by a case of malignant transformation in SC (synovial chondrosarcoma) showing a novel KMT2A-BCOR gene fusion by targeted RNA sequencing, we sought to evaluate the molecular abnormalities in a cohort of 27 SC cases using a combined methodology of fluorescence in situ hybridization (FISH) and/or targeted RNA sequencing. Results showed that FN1 and /or ACVR2A gene rearrangements were noted in 18 cases (67%), with an FN1-ACVR2A fusion being confirmed in 15 (56%) cases. Two cases showed only FN1 gene rearrangement, without other abnormalities. A novel FN1-NFATc2 gene fusion was noted in one case by RNA sequencing. The remaining nine cases showed no abnormalities in FN1 and ACVR2A genes. No additional cases showed BCOR gene alterations. In conclusion, this study confirms that FN1-ACVR2A fusion is the leading pathogenetic event in SC, at even higher frequency than previously reported. FISH methodology emerges as an appropriate tool in the identification of FN1 and ACVR2A gene abnormalities, which can be used in challenging cases. Further studies are needed to determine the recurrent potential of BCOR abnormalities in this disease., (© 2019 Wiley Periodicals, Inc.)

Background/aim: Chondrosarcomas (CS) of the chest wall are rare, but present an aggressive biological behavior compared to CS of the extremities. The aims of the present study were to determine factors associated with oncological outcomes as well as complications., Patients and Methods: We retrospectively analyzed 53 patients (42 primary, 11 recurrent tumors). In total, 39 central CS, 10 peripheral CS, 3 dedifferentiated CS and 1 mesenchymal CS were included. The ribs were most commonly affected (68%). Overall survival and disease-free survival were estimated with Kaplan-Meier analyses and compared with log-rank test., Results: Mean follow-up was 7 years. Negative margins were achieved in 87% of patients. Thirty patients (57%) remained continuously disease-free (NED), three (5%) NED after treatment of relapse, seven (13%) were alive with disease, twelve (23%) were dead with disease and one of other cause. The 10-year survival rate was 81% and 45% in primary and recurrent tumors, respectively. Survival was significantly affected by tumor stage (p<0.001), local recurrence (p=0.025) and metastases (p=0.002). Six complications (16%) were observed., Conclusion: The outcome is rather poor, especially in patients with local recurrence. Presumably due to a high biological aggressiveness, a stricter definition of surgical margins should be considered for this location., (Copyright© 2020, International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.)

Chondrosarcomas are rare tumors and, historically, investigation of these tumors has been limited to small series and single-institution studies. There have been no studies that evaluated the identification or comparison of differences in prognostic factors between the five known non-conventional chondrosarcoma subtypes (myxoid, juxtacortical, clear-cell, mesenchymal, and dedifferentiated). The purpose of this paper was to determine the demographic, clinical, incidence, and tumor characteristics of all five known non-conventional chondrosarcoma subtypes, determine the 1-, 5-year, and median survival differences between these subtypes, and to determine the demographic and clinical variables that are significant prognostic indicators for each chondrosarcoma subtypes. We retrospectively reviewed the SEER database for all patients with non-conventional chondrosarcoma. χ 2 testing was used for correlations between clinical variables. Kaplan-Meier and Cox proportional hazard analysis were used to compare survival of the subtypes, and to assess the prognostic value of age group, race, sex, grade, anatomic location, and metastatic involvement. Several demographic characteristics including gender, race, age, and grade varied between chondrosarcoma subtypes. The tumor characteristics showed marked differences in presence of metastasis on presentation between the subtypes with increasing order of rate of metastasis with juxtacortical (2.1%), clear cell (5.7%), myxoid (7.6%), mesenchymal (10.6%), and the highest in dedifferentiated (19.8%). One-, 5-year, and median survival differed significantly between chondrosarcomas subtypes. The highest median survival was found in the juxtacortical subtype (97 months), followed by clear cell (79 months), myxoid (60 months), mesenchymal (33.5 months), and lowest in dedifferentiated (11 months). The only prognostic variable that was shown to significantly impact the survival of each non-conventional chondrosarcoma subtype was a metastatic disease at diagnosis (p = 0.03 to p < 0.001). Subtyping classification of chondrosarcoma should be made whenever possible, given differences in survival and prognostic factors between chondrosarcoma subtypes. © 2019 Orthopaedic Research Society. Published by Wiley Periodicals, Inc. J Orthop Res 38:311-319, 2020., (© 2019 Orthopaedic Research Society. Published by Wiley Periodicals, Inc.)

Chordoma and chondrosarcoma are locally aggressive tumors occurring in one-third cases at the base of the skull. These tumors often recur locally with significant morbidity and mortality. The mainstay of treatment is maximal safe tumor debulking. However, in spite of gross total resection, these tumors are likely to recur. Hence, adjuvant radiation is provided to reduce the risk of local recurrence and to improve outcomes. These tumors are considered relatively radioresistant; hence, high doses of radiation are generally required during treatment. However, the presence of several important structures around the lesion poses a major challenge with respect to covering the target with the prescribed high dose. In this regard, protons, for their physical and dosimetric advantages, have become the accepted modality of treatment in these tumors. With the evolution of proton beam therapy (PBT) over the years, especially pencil beam scanning techniques; which result in an extremely high conformal intensity-modulated proton beam therapy (IMPT), robust and Monte Carlo optimization, computational algorithms, and biological modelling are the significant advances which have further enhanced the value of this technology and have improved outcomes. Herein, we would like to report our experience of two cases of skull base tumors treated with intensity-modulated proton therapy at our center along with a review of the literature., Competing Interests: None

Context.—: Cartilaginous tumors represent one of the most common tumors of bone. Management of these tumors includes observation, curettage, and surgical excision or resection, depending on their locations and whether they are benign or malignant. They can be diagnostically challenging, particularly in small biopsies. In rare cases, benign tumors may undergo malignant transformation., Objective.—: To review common cartilaginous tumors, including in patients with multiple hereditary exostosis, Ollier disease, and Maffucci syndrome, and to discuss problems in the interpretation of well-differentiated cartilaginous neoplasms of bone. Additionally, the concept of atypical cartilaginous tumor/chondrosarcoma grade 1 will be discussed and its use clarified., Data Sources.—: PubMed (US National Library of Medicine, Bethesda, Maryland) literature review, case review of archival cases at the Massachusetts General Hospital, and personal experience of the authors., Conclusions.—: This review has examined primary well-differentiated cartilaginous lesions of bone, including their differential diagnosis and approach to management. Because of the frequent overlap in histologic features, particularly between low-grade chondrosarcoma and enchondroma, evaluation of well-differentiated cartilaginous lesions should be undertaken in conjunction with thorough review of the imaging studies.

Primitive malignant sternal tumours are extremely rare and represent less than 1% of all chest wall tumours. A combination of radical surgery and accurate reconstruction is mandatory to ensure adequate stability and to reduce paradoxical movement. To date, this is one of the few reports on a successful sternal reconstruction using a rigid titanium mesh to restore the skeletal integrity, which was then covered by bilateral muscular flaps., (© The Author(s) 2019. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Cartilage-forming lesions include tumours that can vary in severity from benign enchondromas to high-grade malignant chondrosarcomas. Chondrosarcoma is the second most frequent malignant bone tumour, accounting for 20-30% of all malignant bone neoplasms. Surgery is the standard treatment for cartilage tumours (CTs); however, their incidental diagnosis and the difficult differentiation of low-grade lesions like chondrosarcoma grade I from benign entities like enchondroma are challenges for clinical management. In this sense, the search for circulating biomarkers for early detection and prognosis is an ongoing interest. Targeted metabolomics is a powerful tool that can propose potential biomarkers in biological fluids as well as help to discover disturbed metabolic pathways to reveal tumour pathogenesis. In this context, the aim of this study was to investigate the 1 H nuclear magnetic resonance metabolomic serum profile of patients with CTs contrasted with healthy controls. Forty-one metabolites were identified and quantified; the multivariate statistical methods principal component analysis and partial least squares discriminant analysis reveal a clear separation of the CT group, that is, the differential metabolites that were involved in two main metabolic pathways: the taurine and hypotaurine metabolism and synthesis and degradation of ketone bodies. Our results represent preliminary work for emergent serum-based diagnostics or prognostic methods for patients with chondrogenic tumours., (© 2019 John Wiley & Sons, Ltd.)

BACKGROUND The incidence and prognostic factors of chondrosarcoma patients have been reported in early studies. However, the association between risk factors and the incidence or prognosis of chondrosarcoma patients with pulmonary metastasis remains unclear. Therefore, we assessed these risk factors among chondrosarcoma patients with pulmonary metastasis. MATERIAL AND METHODS From 1365 chondrosarcoma patients in the Surveillance, Epidemiology, and End Results (SEER) database, we collected the information of 69 patients with pulmonary metastasis at the initial diagnosis of chondrosarcoma from 2010 to 2016. We investigated the incidence, risk factors, and prognostic factors for pulmonary metastasis patients by using multivariate logistic regression and multivariate Cox regression analyses. RESULTS Data from a total of 69 (6.8%) chondrosarcoma patients with pulmonary metastasis at initial diagnosis were extracted. Patients with the following characteristics were positively associated with higher risk of pulmonary metastasis: dedifferentiated subtype, high grade of malignancy, extracompartmental tumor (Enneking B), presence of regional lymph nodes, local recurrence, large tumor size (larger than 15 cm), and being married. Older patients (older than 67 years), and patients with clear cell chondrosarcoma or large tumor size (larger than 15 cm) exhibited the worse prognosis and survival (overall and cancer-specific). Resection of the primary tumor tended to be correlated with a better prognosis. CONCLUSIONS The incidence of pulmonary metastasis in chondrosarcoma was approximately 6.8%, with poor prognosis. Identifying risk factors and their associations with the incidence and prognosis in chondrosarcoma patients with pulmonary metastasis could provide a reference for clinical surveillance and guide the design of personalized treatment plans.

Rationale: Chondrosarcoma of the sacrum is a highly unusual disease without standard curative managements yet. The objective of this study is to report a very rare case of chondrosarcoma of the sacrum successfully operated by percutaneous vertebroplasty. The management of these unique cases has yet to be well-documented., Patient Concerns: A 45-year-old woman presented with a five-month history of continuous and progressive pain and numbness of left extremity. A lytic, expanding lesion of the sacrum and paraspinal region with severe epidural spinal cord compression was identified., Diagnosis: MRI of spine showed spinal cord compression secondary to the epidural componant of the giant mass, with increased marrow infiltration of the left S2 vertebral and paravertebral region, which presented as a solid tumor. Post-operative pathology confirmed the diagnosis of sacral well-differentiated chondrosarcoma (stage I B)., Interventions: The patient underwent percutaneous vertebroplasty and cement augmentation of sacrum via a posterior approach., Outcomes: The patient's neurological deficits improved significantly after the surgery, but the patient died of multiple systemic metastases at the 2-year follow-up visit. There were no complications associated with the operation during the follow-up period., Lessons: Taken together, the lesion's clinical features, imaging results, and pathological characteristics are unique. Combined efforts of specialists from orthopedics, radiology, neurosurgery, pathology, and medical oncology led to the successful diagnosis and management of this patient. Giant sacral chondrosarcoma, although rare, should be part of the differential diagnosis when the patient presents with back pain and radiculopathy. We recommend the posterior approach for spinal decompression of the sacral chondrosarcoma when the tumor has caused neurological deficits or other severe symptoms. Osteoplasty by cement augmentation is also a good choice for surgical treatment.

Objective: To identify prognostic factors and establish nomograms for predicting overall survival (OS) and cause specific survival (CSS) of patients with non-metastatic chondrosarcoma., Methods: We collected information on patients with non-metastatic chondrosarcoma from the Surveillance, Epidemiology, and End Results (SEER) database between 2005 and 2014, together with data from the First Affiliated Hospital of Zhengzhou University from 2011 to 2016. Variables including patients' baseline demographics (age, race, and gender), tumor characteristics (tumor size and extension, histology subtype, primary site, and American Joint Committee on Cancer [AJCC] stage), therapy (surgery, chemotherapy, and radiotherapy), and socioeconomic status (SES) were extracted for further analysis. OS and CSS were retrieved as our researching endpoints. Patients from the database were regarded as the training set, and univariate analysis, Lasso regression and multivariate analysis as well as the random forest were used to explore the predictors and establish nomograms. To validate nomograms internally and externally, we applied bootstrapped validation internally with the training dataset, while the dataset for external validation was obtained from the First Affiliated Hospital of Zhengzhou University. We estimated the discriminative ability of nomograms based on Cox proportional hazard regression models by means of calibration curves and the concordance index (C-index) of internal and external validation., Results: After the implementation of exclusion criteria, there were 1267 patients in the training set and 72 patients in the testing set with non-metastatic chondrosarcomas. Age, gender, grade, histological subtype, primary site, surgery, radiation, chemotherapy, being employed/unemployed, tumor size, and tumor extension were significantly associated with prognosis in the univariate analysis. Age, gender, tumor size and extension, primary site, surgery, radiotherapy, chemotherapy, histological grade, and subtype were independent prognostic factors in the Cox models. The C-index of nomograms (internal: OS, 0.787; CSS, 0.821; external: OS, 0.777; CSS, 0.821) were higher than following conventional systems: AJCC sixth (OS, 0.640; CSS, 0.673) and seventh edition (OS, 0.675; CSS, 0.711)., Conclusions: Age, gender, tumor size and extension, surgery, histological grade, and subtype were independent prognostic factors for both OS and CSS. In addition, we revealed that chondrosarcomas in the trunk, radiotherapy, and chemotherapy were correlated with poor prognosis. Our nomograms based on significant clinicopathologic features can well predict the 3-year and 5-year survival probability of patients with non-metastatic chondrosarcoma and assist oncologists in making accurate survival evaluation., (© 2019 The Authors. Orthopaedic Surgery published by Chinese Orthopaedic Association and John Wiley & Sons Australia, Ltd.)

Background: Temporal bone chondrosarcoma (TBC) are uncommon primary temporal bone malignancies, and clinicians lack experience in its diagnosis and treatment. The optimal management of patients with tumor of TBC also remains a topic of debate and controversy. Objectives: This article summarizes the experience of diagnosis and treatment of TBC, in order to improve the quality of life of cancer patients in the future. Material and methods: We conducted a retrospective analysis of 10 patients who were referred to our hospital from June 2009 to June 2018 for the treatment of TBC. Results: There were 3 males and 7 females. The most common presenting symptoms were facial paresis (50%) and hearing loss (40%), whereas otalgia (10%), vertigo (10%) and headache (10%) were less common. All tumors originated from the temporal bone, and 80% involved the jugular foramen area. All patients survived without evidence of disease at a median time of follow up of 28.8 months. Conclusions and significance: TBC mostly originated in the middle ear mastoid area, and easily extended to the jugular foramen area. An individualized surgical procedure that removes tumors integrally with minimal nerve and blood vessel damage provides long-term cancer control and minimal morbidity in most cases.

Introduction: The tissues of the laryngeal region only rarely harbor primary cartilaginous lesions, and squamous cell carcinoma remains the most frequently encountered malignant tumor in this area., Materials and Methods: We reviewed the salient histological features of cartilaginous laryngeal lesions to provide differential diagnostics and guidelines for distinguishing the benign from the malignant ones., Results: Cartilaginous neoplasms of the larynx include chondroma and chondrosarcoma. Among the overarching group of all forms of laryngeal sarcoma, chondrosarcoma forms the most common entity in the larynx, followed by rhabdomyosarcoma. Cartilaginous tumors comprise about 0.1%-1% of all laryngeal neoplasms with chondrosarcomas being more frequently encountered than chondromas. Several neoplasms earlier reported as giant-cell tumors of the larynx would most likely, using current terminology, be classified as cases of undifferentiated pleomorphic sarcoma (previously known as malignant fibrous histiocytoma, giant-cell variant) or aneurysmal bone cyst., Conclusion: When true laryngeal sarcomas do exist, they may prove to be challenging lesions both for the pathologist and the treating clinician. The diagnostic problems are mainly a result of the infrequent exposure of clinicians and diagnosticians to these lesions.

Background: As the management of ventral skull-base pathology has transitioned from open to endonasal treatment, there has been an increased focus on the prevention and endoscopic endonasal management of internal carotid artery (ICA) and major vascular injury. The use of adenosine to induce transient hypotension or flow arrest has been previously described during intracranial aneurysm surgery; however, there have been no reports of the technique being used during endonasal skull-base surgery to achieve hemostasis following major vascular injury., Methods: Case report (n = 1) and literature review., Results: A 25-year-old female underwent attempted endoscopic endonasal resection of an advanced right-sided chondrosarcoma. During resection of the tumor, brisk arterial bleeding was encountered consistent with focal injury to the right cavernous ICA. Stable vascular hemostasis could not be achieved with tamponade. An intravenous bolus dose of adenosine was administered to induce a transient decrease in systemic blood pressure and facilitate placement of the muscle patch over the direct site of vascular injury. The patient subsequently underwent endovascular deconstruction of the right ICA., Conclusion: This is the first reported use of adenosine to induce transient hypotension for a major vascular injury sustained during endonasal skull-base surgery. Based on well-established safety data from neurosurgical application, adenosine has the potential to be used as a safe and effective adjunctive technique in similar endonasal circumstances and may represent an additional tool in the armamentarium of the skull-base surgeon. Surgeons should consider having adenosine available when a risk of ICA injury is anticipated., (© 2019 ARS-AAOA, LLC.)

The morphological and clinico-biological spectrum of chondrogenic tumours is wide. Given the diversity of therapeutic approaches, the correctness of the histological diagnosis is of major importance. The aim of this review is to provide insights into the challenges and limitations of diagnostic approaches restricted to morphology and to underscore the importance of an interdisciplinary dialogue between surgeons, radiologists and pathologists. Beyond this, the value of molecular analyses introduced in bone tumor diagnostics in recent years is discussed.

Background: Despite the overall diagnostic utility of core needle biopsy (CNB) comparable to incisional biopsy, increased diagnostic errors have been suggested of CNB for myxoid soft tissue tumors. This study compared the diagnostic performance of CNB between myxoid and non-myxoid soft tissue tumors., Methods: 369 patients who underwent ultrasound-guided CNB prior to resection for soft tissue tumors were classified into two groups according to resection pathology; myxoid group (n = 75) and non-myxoid group (n = 294). One-hundred and ninety-three patients were male and the median age of the patients was 40 years. Two-hundred and sixty-three tumors were malignant., Results: CNB correctly diagnosed malignancy in 84% (58 of 69) for the myxoid group and 95% (184 of 194) for the non-myxoid group. For diagnosing histologic grade of soft tissue sarcoma, CNB correctly identified high grade in 78% (18 of 23) for the myxoid group and 74% (94 of 128) for the non-myxoid group. Correct diagnosis rate of histological type was significantly lower in the myxoid group (63% [47 of 75] in the myxoid group and 83% [242 of 294] in the non-myxoid group, p = 0.013)., Conclusion: Our study suggests that CNB is useful for myxoid soft tissue tumors of the extremity, with regard to diagnosing malignancy and histologic grade. However, CNB was less useful for identifying histologic subtype in myxoid tumors than in non-myxoid tumors., (Copyright © 2019 Elsevier Ltd, BASO ~ The Association for Cancer Surgery, and the European Society of Surgical Oncology. All rights reserved.)