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To clarify the epidemiology, treatment, and prognosis of sarcomas occurring in the bones and joints. The surveillance, epidemiology, and end results (SEER) 18 registries, comprising sarcoma diagnoses made between 2008 and 2014, were queried for sarcomas arising in bones or joints. Kaplan-Meier analysis, multivariate logistic regression analysis, Cox proportional hazards model, and nomograms were used to identify prognostic factors. 2794 patients aged from 1 to 99 (55.8% male) with microscopically confirmed diagnosed as sarcomas (including osteosarcoma, chondrosarcoma, Ewing sarcoma, and soft tissue sarcomas) which primary site limited to bone and joint were identified. Eight independent factors, including age, race, sex, tumor site, histology, pathology grade, tumor size, and total number of malignant tumors (TNOMT), were associated with tumor metastasis. Nine independent prognostic factors, including age (>=60 year, hazard ratio [HR] = 4.145, 95% confidence interval [CI], P < .001), sex (female, HR = 0.814, 95%CI, P = .007), tumor site (spine, HR = 2.527, 95%CI, P < .001), histology, pathology grade (undifferentiated, HR = 5.816, 95%CI, P < .001), tumor size (>=20 cm, HR = 3.043, 95%CI, P < .001), tumor extent (distant, HR = 4.145, 95%CI, P < .001), surgery (no performed, HR = 2.436, 95%CI, P < .001), and TNOMT (1, HR = 0.679, 95%CI, P < .001, were identified and incorporated to construct a nomogram for 2- and 5-year overall survival (OS). The calibration curve for the probability of survival showed good agreement between prediction by the nomogram and actual observation. The C-index of the nomogram for survival prediction was 0.814. Patients who received chemotherapy had a significantly decreased risk of death only for Ewing sarcoma, poorly differentiated tumors, undifferentiated tumors, and distant tumor invasion (P < .05). However, radiotherapy did not show significant differences in OS. This study presents population-based estimates of prognosis for patients with bone sarcomas and demonstrates the impact of age, race, sex, tumor site, histology, pathology grade, tumor size, tumor extent, surgery, radiotherapy, chemotherapy, and the TNOMT on OS. Moreover, the nomogram resulted in a more accurate prognostic prediction. However, in our study, radiotherapy showed no survival benefit, perhaps because detailed data on treatment factors were unavailable and which may have influenced the results., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.)

Background: Soft-tissue sarcomas during infancy are rare and understudied. With no data on this specific condition, we performed a retrospective study of infant-onset sarcomas based on a multi-institutional cohort in Beijing, China, collected over the past decade. We reviewed infantile soft-tissue sarcomas' clinical characteristics, treatments, and outcomes., Materials and Methods: The patients with soft-tissue sarcoma diagnosed from 0 to 12 months in four primary children's hospitals in Beijing from January 2010 to December 2019 were evaluated., Results: Fifty-one patients were enrolled, including 31 males and 20 females. The median age at the diagnosis was five months (range, 0-12), and seven (13.7%) patients were diagnosed in the first month of their life. Histologically, twenty-five patients were diagnosed with rhabdomyosarcoma (RMS), six were diagnosed with extraosseous Ewing sarcoma (EES), and twenty were diagnosed with nonrhabdomyosarcoma soft-tissue sarcoma (NRSTS). The treatment principles and details of RMS focused on reference to the Intergroup Rhabdomyosarcoma Study Group (IRSG) protocols. For EES and NRSTS, chemotherapy was prescribed according to children's oncology group protocols. The five-year EFS/OS rates of RMS were 26.4% ± 19.5%/56.2 ± 17.8%, the five-year EFS/OS rate of EES was 50% ± 20.4%, and the five-year EFS/OS of NRSTS was 85.2% ± 9.8%/100%., Conclusions: Infant-onset soft-tissue sarcoma is heterogeneous. The primary location of the abdominal or pelvic cavity of RMS and EWS was at a later stage and had a poorer prognosis. Multimodal therapy resulted in successful disease control for the majority of patients. Standardization of treatment protocols will facilitate care for such challenging conditions., Competing Interests: None

Background: Primary osseous sarcomas of the mobile spine are rare bony tumors. Ewing sarcoma, chondrosarcoma, chordoma and osteosarcoma constitute the majority of primary bone sarcomas of the spine; however, other rare sarcoma tumors may also affect the spine. In order to perform an epidemiological study of theses tumors, national registries may help to evaluate data for populations with similar characteristics., Methods: A population-based study was designed based on data from the Iran National Cancer Registry (INCR). All morphology codes (M-Code) of primary osseous sarcomas of the mobile spine (C-code 41.2) were derived and analyzed., Results: Among 186 patients with primary osseous sarcomas of the mobile spine, 67.2% were men and 32.8% were women. The median (IQR) age was 37.0 (20.0-59.0) years and the age-standardized incidence rate (ASIR) was 0.37 per million. The majority of cases of Ewing sarcoma (29.5%) were observed in the age group 20-25 years. Among male patients with chondrosarcoma, the median age was 39.0 (30.0-50.0), while females showed a median age of 56.0 (50.0-59.0). The median age of patients with chordoma was 54.0 (47.0-63.0) years. The crude incidence rate of mobile spine osteosarcoma was 0.04 per million., Conclusion: Ewing sarcoma was the most frequent primary osseous sarcoma of the mobile spine. A male predilection was observed among all major sarcomas of the mobile spine. Ewing sarcoma in Iran affects the mobile spine in slightly older ages compared to other studies. Myxoid chondrosarcoma is the most frequent subtype of the mobile spine chondrosarcoma. Chordoma affects male in older ages compared to females. In contrast with other studies which showed a bimodal distribution of osteosarcoma of the spine including young adult and older age groups, 86% of cases in Iran were in the age group of 10-40 years., (© 2021 The Author(s). This is an open-access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.)

Introduction: Primary bone and soft tissue sarcomas are rare, but diagnostically and therapeutically challenging group of tumors, requiring multidisciplinary management. There are limited documented studies from multidisciplinary teams , in the form of comprehensive analysis of these tumors, from our country. This study is an analysis of cases of osteosarcomas, Ewing sarcomas (ESs), chondrosarcomas (CSs), and soft-tissue sarcomas (STSs), registered at our institution during 2012., Methods: Clinical details, including outcomes of cases of bone and STSs, during the year 2012, were retrieved from the medical records of our institution and were further analyzed., Results: Ninety-five high-grade, extremity-based, treatment-naïve cases of osteosarcomas were treated with a novel, dose-dense, nonhigh-dose methotrexate-based OGS-12 protocol. Good histopathologic response (necrosis ≥90%) was achieved in 59% nonmetastatic and 56% metastatic patients. At a median follow-up of 48 months, the estimated 5-year event-free survival and overall survival (OS) were 67% and 78%, respectively. In the metastatic cohort at a median follow-up of 51 months, the 5-year estimated progression-free survival was 24% and OS was 26%. Among 87 (73.2%) nonmetastatic and 32 (26.8%) metastatic, analyzable cases of ES, at a median follow-up of 40 months, the disease-free survival (DFS) and OS in the nonmetastatic group were 62% and 83%; in the metastatic group, they were 37.5% and 65.6%, respectively. Among 40 cases of CSs (33 nonmetastatic and 7 metastatic), 21 had limb salvage surgery while 5 had amputation. Microscopically, 90.4% were Grade II CSs. Five-year OS and DFS were 84.6% and 71%, respectively. Among 189 high-grade, extremity-based STSs (89% nonmetastatic), synovial sarcoma was the most common subtype (31%). Eighty-five percent had limb preservation surgery; a majority were offered adjuvant radiation with or without chemotherapy. At a median follow-up of 51 (1-63) months, 3-year local control, DFS, and OS were 81%, 48%, and 64%, respectively., Conclusions: The novel OGS 12 and Ewing Family of Tumors 2001 protocols have shown comparable outcomes to international standards in cases of osteosarcoma and ES, respectively, and merit wider applications, especially in low- and middle-income countries (LMICs). Outcomes in STS and CSs were also comparable and underscore the importance of a multidisciplinary approach for the management of sarcomas in LMICS., Competing Interests: There are no conflicts of interest

Objective: Due to the low incidence, treatments for Korean soft tissue Ewing sarcoma patients have been heterogeneous, and reported data are limited. In this study, we retrospectively analyzed soft tissue Ewing cases treated at our institution., Methods: We analyzed the clinicopathologic characteristics and treatment outcome of soft tissue Ewing sarcoma patients and compared with those of skeletal cases., Results: Twenty-seven soft tissue Ewing sarcoma cases were evaluated. Patients with soft tissue Ewing sarcoma were older than patients with skeletal tumors (P = 0.03), and tended to have metastasis at diagnosis (P = 0.12). However, sex ratios, pathologies, tumor volumes, and histologic response to preoperative chemotherapy were not different in the two groups. The 5-year overall survival (49.0%) and event-free survival (45.6%) of soft tissue Ewing sarcoma patients were similar to those of skeletal tumor patients (51.8% and 46.0%, respectively). Presence of metastasis at diagnosis and poor histologic response to preoperative chemotherapy were associated with an adverse outcome for both groups. Similar to skeletal tumors, central tumor location, pathology and tumor volume tended to be related to the survival of soft tissue Ewing sarcoma. However, age and the use of a modality other than surgery to achieve local control did not influence the survival of soft tissue Ewing sarcoma patients., Conclusions: Our data could provide a basis to design a collaborative or multinational study targeting Ewing sarcoma family tumors.

In most cancers, the age of the affected patient has a significant influence on how that cancer is approached. This is less often the case in sarcomas in that both children and adults are treated similarly. However, different types of sarcomas are most typical in these populations, and our understanding of these cancers in one group has provided direction for understanding them in the other group. For example, advances at the molecular level in Ewing sarcoma, a disease that affects predominantly children, helped spearhead the uncovering of several signature translocations in adult sarcomas, such as synovial sarcoma and myxoid liposarcomas. The success of chemotherapy in pediatric sarcomas continues to be a benchmark for adult sarcomas to emulate. Conversely, the demonstration of the viability of limb-sparing surgery combined with adjuvant radiation in adult extremity sarcomas helped advance that treatment approach in pediatric sarcomas. To illustrate some of these concepts and to enhance our understanding of sarcomas across the age spectrum, 4 types of sarcoma are discussed. The first 2 are Ewing sarcoma and rhabdomyosarcoma seen mainly, but not exclusively, in children. The other 2 are synovial sarcoma, which can be looked at as a bridge between pediatric and adult sarcomas as it affects mainly young adults, and liposarcoma that is almost exclusively an adult sarcoma.

Sarcomas arise in any part of the body, in any age group. Some sarcomas have a particular predilection for the pediatric population, and often bear specific chromosomal translocations. These "pediatric" sarcomas still occur in adults, often times with higher risk of dying of disease compared to children with a comparable diagnosis. The management of some of these rare tumors is discussed herein, including osteogenic sarcoma, Ewing sarcoma, and rhabdomyosarcoma., ((c) 2008 Wiley-Liss, Inc.)

Primary sarcomas are rare in the spine. Common primary sarcomas include osteosarcoma, Ewing's sarcoma, chondrosarcomas, and retroperitoneal soft-tissue sarcomas. These tumors tend to occur in adolescents and young adults. Surgery remains the mainstay of treatment, although the anatomy of spine and spinal cord often limits complete surgical resection with a wide margin. Chemotherapy and radiation therapy have variable effects on these tumors. With recent advances in surgical techniques and development of new chemotherapy protocols, survival and local control are both improving.

Nine cases of primary sarcoma of the jaw seen over a period of 14 years are presented. They consisted of four osteosarcomas, three fibrosarcomas, one myxosarcoma and one Ewing's sarcoma. The treatment of choice for osteosarcomas of the mandible is hemimandibulectomy with disarticulation at the temporomandibular joint. Bone graft reconstruction from the iliac crest should be done at a second stage. Palliative maxillectomy for extensive fibrosarcoma of the maxilla served to relieve one patient of local symptoms for more than a year. Myxosarcoma, a variant of fibrosarcoma, and Ewing's sarcoma of the jaw, are very rare conditions, and since these lesions are usually very extensive radiotherapy is the treatment of choice.