Bone Tumours

2020 ◽  
pp. 219-230
Author(s):  
Stefan Bielack ◽  
Michael Paulussen ◽  
Lee Helman
Keyword(s):  
Computed Tomography ◽  
Ewing Sarcoma ◽  
Local Treatment ◽  
Young Patients ◽  
Disease Free Survival ◽  
Local Therapy ◽  
Childhood And Adolescence ◽  
Bone Sarcomas ◽  
Magnetic Resonance Imaging Mri ◽  
The Many

This chapter reviews the biology, diagnostics, multidisciplinary treatments, and prognosis of the most common bone sarcomas of childhood and adolescence—osteosarcoma and Ewing sarcoma. Both have their peak incidence among teenagers; both carry a high risk of metastatic spread. Local therapy alone is therefore rarely curative. Combining local therapies with intensive multidrug chemotherapy, usually employed both before and afterwards, can lead to long-term disease-free survival in approximately two thirds of affected young patients. Bone sarcomas should only be treated by experienced centres able to provide access to the full spectrum of required expert care. This includes access to essential diagnostic methods such as conventional radiography and magnetic resonance imaging (MRI) for evaluation of the primary, computed tomography (CT) of the chest to search for lung metastases, and radionuclide bone scan, positron emission tomography/computed tomography (PET/CT) and/or whole-body MRI to search for bone metastases. Local treatment of osteosarcoma is surgery whenever feasible. Limb-saving resections have largely replaced amputations. There is also a trend towards surgery in Ewing sarcoma, where radiotherapy has traditionally held a larger role. Histological response of the primary to preoperative chemotherapy is an important prognostic factor in both malignancies. Attempts to improve the prognosis of poor responders by postoperative treatment modifications have failed in osteosarcoma, but may work to some extent in Ewing sarcoma. Unfortunately, the many recent advances in the knowledge about their tumour biology have not yet resulted in better treatments for either of these two most common bone sarcomas of childhood and adolescence.

Treatment of Nonmetastatic Rhabdomyosarcoma in Childhood and Adolescence: Third Study of the International Society of Paediatric Oncology—SIOP Malignant Mesenchymal Tumor 89

2005 ◽  
Vol 23 (12) ◽  
pp. 2618-2628 ◽  
Author(s):  
Michael C.G. Stevens ◽  
Annie Rey ◽  
Nathalie Bouvet ◽  
Caroline Ellershaw ◽  
Françoise Flamant ◽  
...  
Keyword(s):  
Alkylating Agents ◽  
Randomized Study ◽  
Local Treatment ◽  
Local Therapy ◽  
Paediatric Oncology ◽  
Mesenchymal Tumor ◽  
Childhood And Adolescence ◽  
Free Survival ◽  
Malignant Mesenchymal Tumor ◽  
Better Than

Purpose To improve outcome for children with nonmetastatic rhabdomyosarcoma and to reduce systematic use of local therapy. Patients and Methods Five hundred three previously untreated patients aged from birth to 18 years, recruited between 1989 and 1995, were allocated to one of six treatment schedules by site and stage. Results Five-year overall survival (OS) and event-free survival (EFS) were 71% and 57%, respectively. Primary site, T-stage, and pathologic subtype were independent factors in predicting OS by multivariate analysis. Differences between EFS and OS reflected local treatment strategy and successful re-treatment for some patients after relapse. Patients with genitourinary nonbladder prostate tumors had the most favorable outcome (5-year OS, 94%): the majority were boys with paratesticular tumors treated successfully without alkylating agents. Patients with stage III disease treated with a novel six-drug combination showed improved survival compared with the Malignant Mesenchymal Tumor 84 study (MMT 84; 5-year OS, 60% v 42%, respectively). OS was not significantly better than that achieved in the previous MMT 84 study, but 49% of survivors were cured without significant local therapy. Conclusion Selective avoidance of local therapy is justified in some patients, though further work is required to prospectively identify those for whom this is most applicable. Exclusion of alkylating agents is justified for the most favorable subset of patients. The value of the new six-drug chemotherapy combination is being evaluated further in a randomized study (MMT 95).

scholarly journals Bone sarcoma: success through interdisciplinary collaboration

2021 ◽  
Vol 15 (4) ◽  
pp. 331-336 ◽  
Author(s):  
Stefanie Hecker-Nolting ◽  
Ana Maia Ferreira ◽  
Stefan S. Bielack
Keyword(s):  
Literature Review ◽  
Interdisciplinary Collaboration ◽  
Palliative Therapy ◽  
Local Therapy ◽  
Bone Sarcoma ◽  
Childhood And Adolescence ◽  
Malignant Bone Tumours ◽  
Bone Sarcomas ◽  
Relapsed Disease ◽  
Effective Drugs

Purpose Osteosarcoma and Ewing sarcoma are the most frequent malignant bone tumours of childhood and adolescence. This review summarizes the oncologist’s view of these diseases and their treatment. Methods A non-systematic literature review was performed, the personal impressions and experience of the authors is described. Results Local therapy and chemotherapy, each on their own, will not cure patients with malignant bone sarcomas. Together, they present a highly efficacious combination. While the most effective drugs were defined decades ago, progress since then has been limited. It is hoped that substances shown to be active in relapsed disease will be forwarded into even more efficacious frontline treatments. Good palliative therapy is necessary when cure is no longer an option. Conclusion Close interdisciplinary collaboration is the key to successful treatment of bone sarcomas in paediatric patients.

Computed tomography and magnetic resonance features of renal ewing sarcoma

2008 ◽  
Vol 49 (9) ◽  
pp. 1085-1090 ◽  
Author(s):  
T. Ekram ◽  
K.M. Elsayes ◽  
R.H. Cohan ◽  
I.R. Francis
Keyword(s):  
Computed Tomography ◽  
Magnetic Resonance ◽  
Ewing Sarcoma ◽  
Pediatric Population ◽  
English Literature ◽  
Adult Population ◽  
Mri Features ◽  
Magnetic Resonance Imaging Mri ◽  
Mri Characteristics ◽  
Ct And Mri

Ewing sarcoma (ES) is a rare malignant tumor that primarily involves long and flat bones but can develop in almost any bone or soft tissue. ES accounts for 2.3–3.5% of tumors in patients under the age of 19, and is rarely found in the adult population. Sarcomas, in general, account for less than 1% of tumors in adults. Several reports of renal ES have been described in the pediatric population, but only a few cases have been described in the adult population. To the best of our knowledge, fewer than 10 cases of renal Ewing sarcoma in adults have been described in the English literature. None of these cases described the computed tomography (CT) and magnetic resonance imaging (MRI) features. We report a case of a 46-year-old woman, including CT and MRI characteristics.

scholarly journals CLINICAL AND THERAPEUTICAL ASPECTS OF EWING SARCOMA IN CHILDREN AND ADOLESCENTS – A SINGLE CENTER EXPERIENCE

2016 ◽  
Vol 63 (1) ◽  
pp. 39-44
Author(s):  
Razvan-Cosmin Petca ◽  
◽  
Stefan Gavriliu ◽  
Gheorghe Burnei ◽  
◽  
...  
Keyword(s):  
Children And Adolescents ◽  
Ewing Sarcoma ◽  
Histopathological Examination ◽  
Local Therapy ◽  
Childhood And Adolescence ◽  
Tumor Biopsy ◽  
Female Ratio ◽  
Single Center Experience ◽  
The Mean ◽  
Rare Malignancy

Ewing sarcoma, a rare malignancy of childhood and adolescence, has become a model of progress in diagnosis and treatment through long-standing research efforts in multinational clinical trials. The aim of this study is to present the specific diagnostic and therapeutic approach of Ewing sarcoma in children and adolescents. Materials and method. A retrospective and prospective analysis of all Ewing sarcoma treated in a large referral center – the Department of Pediatric and Orthopedic Surgery, Maria Sklodowska Curie Emergency Hospital for Children, between 2005 and 2012 is presented. A total of 28 patients were identified, 19 boys and 9 girls, with a male to female ratio of 2.11:1. Diagnosis was based on the result of the histopathological examination of tumor biopsy. Results. The mean age of the patients was 12.3 years (range 3-19 years) and the mean tumor volume was 197.96 cm3 (range 8-1,200 cm3). 8/28 patients (28.57%) had metastatic disease at diagnosis, mainly in the lungs (7 cases). Chemotherapy was administred to 26 patients (92.85%). Local therapy consisted of surgery in all patients and of surgery combined with radiation, in 5 patients. After a mean follow-up of 51.5 months, 9 patients have died, 17 (60.71%) patients are alive and free of disease and 2 patients are alive with disease. Conclusions. The management of a child or adolescent with Ewing sarcoma is best carried out in a specialized center under the care of a multidisciplinary team, in order to obtain the best outcome for the patient. Ewing sarcoma has a high mortality rate in Romania, especially because of late diagnosis.

Challenges and Opportunities for Neuroimaging in Young Patients with Traumatic Brain Injury: a Coordinated Effort towards Advancing Discovery from the ENIGMA Pediatric Moderate/Severe TBI Group

2019 ◽  
Author(s):  
Emily L. Dennis ◽  
Karen Caeyenberghs ◽  
Robert F. Asarnow ◽  
Talin Babikian ◽  
Brenda Bartnik-Olson ◽  
...  
Keyword(s):  
Traumatic Brain Injury ◽  
Brain Injury ◽  
Meta Analysis ◽  
Young Patients ◽  
Future Research ◽  
Childhood And Adolescence ◽  
Developmental Issues ◽  
Challenges And Opportunities ◽  
Severe Tbi ◽  
Advance Research

Traumatic brain injury (TBI) is a major cause of death and disability in children in both developed and developing nations. Children and adolescents suffer from TBI at a higher rate than the general population; however, research in this population lags behind research in adults. This may be due, in part, to the smaller number of investigators engaged in research with this population and may also be related to changes in safety laws and clinical practice that have altered length of hospital stays, treatment, and access to this population. Specific developmental issues also warrant attention in studies of children, and the ever-changing context of childhood and adolescence may require larger sample sizes than are commonly available to adequately address remaining questions related to TBI. The ENIGMA (Enhancing NeuroImaging Genetics through Meta-Analysis) Pediatric Moderate-Severe TBI (msTBI) group aims to advance research in this area through global collaborative meta-analysis. In this paper we discuss important challenges in pediatric TBI research and opportunities that we believe the ENIGMA Pediatric msTBI group can provide to address them. We conclude with recommendations for future research in this field of study.

scholarly journals A243 A RETROSPECTIVE REVIEW OF THE RADIOGRAPHIC DIAGNOSIS AND SURGICAL RESECTION RATES OF PANCREATIC SEROUS CYSTS

2021 ◽  
Vol 4 (Supplement_1) ◽  
pp. 300-301
Author(s):  
M Monachese ◽  
S Li ◽  
M Salim ◽  
L Guimaraes ◽  
P D James
Keyword(s):  
Magnetic Resonance Imaging ◽  
Computed Tomography ◽  
Magnetic Resonance ◽  
Surgical Resection ◽  
Predictive Value ◽  
Resected Specimen ◽  
Cystic Lesions ◽  
Resonance Imaging ◽  
Guided Biopsy ◽  
Magnetic Resonance Imaging Mri

Abstract Background Pancreatic cystic lesions are increasingly identified in persons undergoing abdominal imaging. Serous cystic neoplasms (SCNs) have a very low risk of malignant transformation. Resection of SCNs is not recommended in the absence of related symptoms. The accuracy of computed tomography (CT) and magnetic resonance imaging (MRI) to identify SCNs is not known and may impact clinical care. Aims To evaluate the accuracy of computed tomography (CT) and magnetic resonance imaging (MRI) for the diagnosis of SCN. To see how this can impact the decision to resect suspected SCNs. Methods Retrospective cohort study of patients from the University Health Network with suspected SCNs from 2017–2020 who underwent either a CT or MRI of the abdomen. Reports noting pancreatic cystic lesions were identified and reviewed. Only cases with suspected SCNs were included. Clinical (age, sex, symptoms, treatment) and radiographic (type of imaging, reported cyst characteristics) data was collected. Pathology was reviewed for all cases where the cysts was biopsied or resected during follow-up. The gold standard for the diagnosis for SCN was pathology of resected specimen or EUS-guided biopsy cytopathology showing no evidence of a mucinous lesion, CEA level below 10ug per L and amylase level below 50 U/L. Results 163 patients were included in the study. 99 (61%) were female and 98 (60%) underwent CT scan. EUS-guided biopsy was performed in 24 (15%) of patients and 8 (5%) had surgical resection. Multidisciplinary review was performed in 6 of the 8 cases that went to surgery. Of the resected specimens, 5 (63%) were SCN, 1 was a mucinous cystic lesion, 1 was a neuroendocrine tumor and 1 was a carcinoma. Two patients underwent EUS evaluation prior to surgical resection. In one case SCN was resected when EUS reported an undetermined cyst type. Reasons for surgical resection were: the diagnosis of serous cyst was not definitive (n=5), symptoms (n=2), and high-risk mucinous cystic neoplasm identified on EUS (n=1). Of 30 patients with pathology available, 15 (50%) were confirmed to have a SCN. CT and MRI had a sensitivity, specificity, positive predictive value and negative predictive value of 93%, 25%, 52% and 80%, respectively. Conclusions Surgical resection for SCN lesions is driven by diagnostic uncertainty after cross-sectional imaging. Multidisciplinary review and EUS evaluation may improve diagnostic accuracy and should be considered prior to surgical resection of possible SCN lesions. Funding Agencies None

scholarly journals Outcome and Toxicity of an Ifosfamide-Based Soft Tissue Sarcoma Treatment Protocol in Children. The Importance of Local Therapy

Sarcoma ◽  
1998 ◽  
Vol 2 (3-4) ◽  
pp. 171-177
Author(s):  
S. Murray Yule ◽  
Roderick Skinner ◽  
Martin W. English ◽  
Mike Cole ◽  
Andrew D. J. Pearson ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcoma ◽  
Primary Tumour ◽  
Treatment Protocol ◽  
Disease Free Survival ◽  
Local Therapy ◽  
Complete Response ◽  
One Year ◽  
Sarcoma Treatment

Background.Although the survival of children with soft tissue sarcoma (STS) has improved considerably, the outcome of patients with metastatic disease, and those with primary tumours of the extremities or parameningeal sites remains disappointing. We describe the clinical outcome of an ifosfamide-based regimen with local therapy directed only to children who failed to achieve a complete response to initial chemotherapy.Patients and Methods.Twenty-one children with STS (16 rhabdomyosarcoma) who presented with unresectable tumours were treated with five courses of ifosfamide (9 g/m2) and etoposide (600 mg/m2). Patients who did not achieve a complete response then received local therapy. Chemotherapy with ifosfamide combined with etoposide, vincristine (1.5 mg/m2and doxorubicin (60 mg/m2) or vincristine and actinomycin D (1.5 mg/m2) was continued for one year.Results and Discussion.Objective responses to five courses of ifosfamide and etoposide were seen in all patients. Disease free survival (DFS) at a median follow up of 59 months was 57% (95% CI 29–75%). The DFS of children who received local therapy was 89% compared with 33% in those who received chemotherapy alone (p=0.027). Locoregional recurrences did not occur in children who received radiotherapy to the site of the primary tumour. Ifosfamide-based chemotherapy does not reduce the incidence of loco-regional recurrence in children who do not receive local therapy.

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