جستجوی مقالات مرتبط با کلیدواژه « ewing sarcoma » در نشریات گروه « پزشکی »

The number of available musculoskeletal tumor registries is relatively small. We developed a registry system focused on the clinical aspects of musculoskeletal tumors to improve quality of care indexes through the development of updated national protocols. In this study, we describe our protocol, challenges, and the data collected during the implementation of the registry system in a single-specialty orthopedic center in Iran.  

Three main malignant bone tumors, including osteosarcoma, Ewing sarcoma, and chondrosarcoma, were included in the registry. After establishing a steering committee, we defined the minimum data set based on a literature review and suggestions from an expert panel. Accordingly, the data collection forms and the web-based software were developed. The collected information was categorized into 9 classes, including demographics, socioeconomic data, signs and symptoms, past medical history, family history, laboratory tests, tumor characteristics, primary treatment, and follow-up. Data collection was performed both retrospectively and prospectively.  

Until September 21, 2022, a total of 71 patients were registered (21 patients prospectively and 50 patients retrospectively) and consisted of 36 (50.7%) cases of osteosarcoma, 13 (18.3%) cases of Ewing sarcoma, and 22 (31%) cases of chondrosarcoma. The implementation of the registry demonstrated promising data regarding the tumor characteristics, delay patterns, and socioeconomic status of the patients. 

The main lessons learned were to develop a monitoring system to make sure that the new staff is adequately trained for the registration process as well as avoid the inclusion of time-consuming useless data in the minimum data set.

 Extraosseous Ewing sarcomas (EESs) are rare tumors that originate from soft tissues. Upper extremity EESs account for about 3% of all cases. Here we reported a case of ESS of the upper limb whose management became complicated due to the COVID-19 pandemic.

 A 27-year-old female with EES of the right deltoid region presented after 3 months delay when the tumor had reached a huge size. Neoadjuvant therapy was initiated for her with acceptable results, however, her surgical treatment was postponed 3 times due to the cancellation of elective operations in the hospital as well as her involvement with COVID-19 infection. She developed multiple pulmonary metastases shortly after the surgery and passed away within a fortnight due to respiratory complications.

 Although not “emergent” by definition, surgical treatment of patients with cancer, especially those who suffer from malignancies with high metastatic potential such as Ewing sarcoma (including EES), should not be considered as “elective” since the disease may progress in a short time and become incurable.

The effect of race and ethnicity on some kind of malignant bone tumors including Ewing sarcomahas been proven in different studies. In order to evaluate the latter, national cancer registries may help to increaseunderstanding about potential cancer causes, prevention and control strategies, and apply these findings to controlhealth problems among populations with similar characteristics.

A national population-based cancer registry study based on all patients affected by Ewing Sarcoma wasregistered in the Iran National Cancer Registry (INCR) between 2008 and 2015 was designed. Demographic data ofmicroscopically confirmed cases of bone Ewing sarcoma were registered. Patients with Ewing sarcoma were dividedin groups to describe the primary site of the tumor (including axial or appendicular bones) and analyzed. In order toanalyze the survival rate, randomized selection of the patient through the INCR data-base was performed.

A total of 678 cases of malignant Ewing sarcoma of the bone were identified through the INCR. The meanage of Ewing sarcoma in Iran was 21.53 years. Nearly half of patients were observed at the age group of 15-24. Thetotal crude incidence rate of Ewing sarcoma was 1.29 in 1 million. The mean 5 year survival rate was 47%. The Meansurvival rate for study population was 5.53.

The crude incidence rate of Ewing sarcoma in Iran is relatively lower with respect to other registries. Themajority of patients are in 15-25 years group and shows affection by Ewing sarcoma in an older age. Socioeconomicfactors had direct influence on survival rate.Level of evidence: IV

Ewing Sarcoma is the second most common type of bone cancer in children. The dominant features of this malignant bone tumor are the tendency for rapid growth and metastasis.  In addition, Ewing sarcoma of the mandible is extremely rare and can be mistaken for odontogenic infection. We report a 14-year-old girl who had had swelling, pain, and hypoesthesia in the left cheek for three weeks.  She was diagnosed with pericoronitis initially, and then referred toour hospital due to worsening symptoms.  CT and MRI revealed an expanding and destructive mass mainly in the left mandible.  18F-fluorodeoxyglucose (18F-FDG) positron emission tomography (PET) and fused PET/CT demonstratedincreased uptake in the mandibular lesion.  Whole-body 18F-FDG PET images showed no abnormal activity except for the mandibular lesion.  Histologic examination confirmed Ewing sarcoma.  Although this tumor has an aggressive clinical behavior and rapid growth, early diagnosis can reduce patient’s morbidity, mortality and thus it is important to distinguish it from periodontal inflammation.

Primary malignant sarcomas of the spine are not among common types of primary bone sarcomas. There are only few cases reported on the literature about this tumor. Ewing sarcoma occurring in the spine is divided into two types: 1) sacral spine Ewing sarcoma, which is very aggressive with poor prognosis and 2) non-sacral spine Ewing sarcoma, which is an extremely rare occurrence. The patient may present neurological deficit when the tumor extends into the spinal canal causing spinal cord compression; however, a sudden progressive paraplegia also is very rare. Here, we report a case of 8 years girl with sudden weakness and tingling in both lower limbs, inability to walk, progressive neck and upper dorsal pain, and urinary retention problem. Spinal MRI shows extradural mass from T2 to C7 with severe spinal cord compression. Urgent decompressive laminectomy and GTR of the lesion was done with excellent postoperative outcome

Ewing sarcoma/peripheral primitive neuroectodermal tumor (ES/PNET) typically occurs in long or flat bones, soft tissues, or less often, solid organs. Ewing sarcoma/peripheral primitive neuroectodermal tumor arising from the adrenal gland is extremely rare, especially in children, and only limited cases are reported previously. Herein, we review a case of a 22-month-old girl who presented to our department with abdominal pain, bulging of the left flank, and a nonfunctioning adrenal lesion which was found to be an adrenal ES/PNET. The patient was successfully treated with surgery and adjuvant chemotherapy. Since delayed diagnosis may result in metastatic lesions, this case underscores the importance of considering ES/PNET in the differential diagnosis of large adrenal masses.

Bone hardness and strength depends on mineralization, which involves a complex process in which calcium phosphate, produced by bone-forming cells, was shed around the fibrous matrix. This process is strictly regulated, and a number of signal transduction systems were interested in calcium metabolism, such as the phosphoinositide (PI) pathway and related phospholipase C (PLC) enzymes.
Our aim was to search for common patterns of expression in osteoblasts, as well as in ES and SS.
We analysed the PLC enzymes in human osteoblasts and osteosarcoma cell lines MG-63 and SaOS-2. We compared the obtained results to the expression of PLCs in samples of patients affected with Ewing sarcoma (ES) and synovial sarcoma (SS).
In osteoblasts, MG-63 cells and SaOS-2 significant differences were identified in the expression of PLC δ4 and PLC η subfamily isoforms. Differences were also identified regarding the expression of PLCs in ES and SS. Most ES and SS did not express PLCB1, which was expressed in most osteoblasts, MG-63 and SaOS-2 cells. Conversely, PLCB2, unexpressed in the cell lines, was expressed in some ES and SS. However, PLCH1 was expressed in SaOS-2 and inconstantly expressed in osteoblasts, while it was expressed in ES and unexpressed in SS. The most relevant difference observed in ES compared to SS regarded PLC ε and PLC η isoforms.
MG-63 and SaOS-2 osteosarcoma cell lines might represent an inappropriate experimental model for studies about the analysis of signal transduction in osteoblasts

Understanding the differences in genetics of malignancies is crucial for therapeutic decisions. This systematic review was conducted to evaluate the current evidence on genetics of bone tumors in the context of pediatric cancer.
Material and We performed a systematic review of the literature published on genetics of pediatrics bone tumors, using PubMed, Google scholar, Science Citation Index and Embase. The search profiles used were: pediatric/childhood malignant bone tumors, pediatric/ childhood bone cancer/neoplasm, osteosarcoma/bone sarcoma/Ewing's sarcoma and risk factors/etiology. Inclusion criteria were as follows: focused upon biology and genetics mechanism of primary bone tumors and published in the last 15 years in English.
A total of 278 articles were searched for relevancy, determined by article title, abstract, and full copy. After screening the titles and abstracts, 239 articles were excluded because they were the same articles and case reports. Finally, 39 articles were found that fulfilled all inclusion criteria.
This systematic review shows that many genetic studies have been performed on the genetics basis of pediatrics bone tumors. The knowledge base formed by this review should facilitate more informative future research. It is important that orthopedics and other specialists be aware about genetics basis of pediatrics bone tumors.

Ewing sarcoma of bone represents the second most common primary malignant tumor of the bone in children and adolescents. The most common primary bony sites include the long bones, pelvis, chest wall, and spine. However, it is responsible for only 3% to 5% of cases in bones of the hands and feet.
The case was a 13-year-old girl, who attended our clinic with a complaint of 8 months of left ankle swelling and pain. Eight months earlier, she recalled an ankle torsion for which treatment was performed with long leg splint. Her pain was better, but, her swelling remained. The X ray showed a sclerotic lesion in the calcaneus with soft-tissue mass. Next, she underwent open biopsy, and was diagnosed with Ewing’s sarcoma. She underwent chemoradiotherapy and obtained below knee amputation. She was followed up at our clinic.
There are a few articles about early diagnotic effects of Ewing''s sarcoma prognosis. Therefore, this study aimed at presenting an anatomical-rare case of Ewing’s sarcoma as preliminary diagnosis could help the patient and lead to better outcomes.

Understanding the differences in genetics of malignancies is crucial for therapeutic decisions. This systematic review was conducted to evaluate the current evidence on genetics of bone tumors in the context of pediatric cancer.
Material and We performed a systematic review of the literature published on genetics of pediatrics bone tumors, using PubMed, Google scholar, Science Citation Index and Embase. The search profiles used were: pediatric/childhood malignant bone tumors, pediatric/ childhood bone cancer/neoplasm, osteosarcoma/bone sarcoma/Ewing's sarcoma and risk factors/etiology. Inclusion criteria were as follows: focused upon biology and genetics mechanism of primary bone tumors and published in the last 15 years in English.
A total of 278 articles were searched for relevancy, determined by article title, abstract, and full copy. After screening the titles and abstracts, 239 articles were excluded because they were the same articles and case reports. Finally, 39 articles were found that fulfilled all inclusion criteria.
This systematic review shows that many genetic studies have been performed on the genetics basis of pediatrics bone tumors. The knowledge base formed by this review should facilitate more informative future research. It is important that orthopedics and other specialists be aware about genetics basis of pediatrics bone tumors.

Treatment with intensification of chemotherapy using alkylating agents and Topoisomerase II inhibitors and radiotherapy has improved the outcome of patients with solid tumors such as Ewing’s sarcoma. However, there are several reports of secondary malignancy following treatment of these tumors. In this article, we describe a 12 years old girl with ALL who had Ewing’s sarcoma when she was 8 years old and underwent successful treatment but after two and half years at 12 years old, she came back with pallor and muscular pain.