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1.
J Surg Oncol ; 129(2): 424-435, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37754672

RESUMO

The use of three-dimensional printed implants in the field of orthopedic surgery has become increasingly popular and has potentiated hip reconstruction in the setting of oncologic resections of the pelvis and acetabulum. In this review, we examine and discuss the indications and technical considerations for custom implant reconstruction of pelvic defects.


Assuntos
Procedimentos Ortopédicos , Próteses e Implantes , Humanos , Pelve/cirurgia , Acetábulo/cirurgia , Impressão Tridimensional
2.
J Surg Oncol ; 2024 May 19.
Artigo em Inglês | MEDLINE | ID: mdl-38764259

RESUMO

BACKGROUND: Given the paucity of data, the objective of this study is to evaluate the association between obesity and major wound complications following pelvic bone sarcoma surgery specifically. METHODS: Patients who underwent pelvic resection for bone sarcoma from 2005 to 2021 with a minimum 6-month follow-up were reviewed. Patients with benign tumors, primary soft tissue sarcomas, local recurrence at presentation, pelvic metastatic disease, and underweight patients were excluded. A major wound complication was defined as the need for a secondary debridement procedure. Differences in baseline demographics, surgical factors, postoperative complications, and functional outcomes were compared between obese and nonobese patients. A multivariate logistic regression was performed to identify independent risk factors for major wound complications, and a Kaplan-Meier analysis to estimate overall survival between both groups. RESULTS: Of the 93 included patients, 21 were obese (body mass index ≥ 30 kg/m2). The obesity group had a significantly higher rate of major wound complication (52% vs. 26%, p = 0.034) and a lower Toronto Extremity Salvage Score at 1-year postoperatively (47.5 vs. 71.4, p = 0.025). Obesity was the only independent risk factor in the multivariate analysis. No differences in overall survival were demonstrated between groups. CONCLUSIONS: Obesity is a significant risk factor for major wound complications in pelvic bone sarcoma treatment. This highlights the importance of careful perioperative optimization and wound management.

3.
J Surg Oncol ; 129(7): 1364-1373, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38533914

RESUMO

INTRODUCTION: Recycled bone autografts prepared using extracorporeal irradiation (ECIR) or liquid nitrogen freezing (LNF) methods have been used for the reconstruction of skeletal elements after wide resection of sarcomas involving bone tissues. Few reports include long-term follow-up data for histological analyses of recycled autografts, particularly in the case of ECIR autografts. MATERIALS: A total of 34 malignant bone and soft tissue tumors were resected and reconstructed using 11 ECIR- and 23 LNF-recycled autografts; the mean postoperative follow-ups were 14 and 8 years, respectively. ECIR was used for either osteosarcomas or Ewing sarcomas, whereas in addition to these tumors LNF was used for chondrosarcomas and soft tissue sarcomas involving bone tissues. Recycled bone was implanted as total bone, osteoarticular, or intercalary grafts, with or without prosthesis or vascularized fibular grafts. RESULTS: The 10-year graft survival rate was similar between groups, 81.8% using ECIR and 70.2% using LNF. There were no autograft-related tumor recurrences in either group. Graft survival was unrelated to type of graft or additional procedures. Complication rates tended to be higher using ECIR (64%) compared with LNF (52%) and the infection rate was significantly higher with ECIR (27%) versus LNF (0%). At the final assessment, plain radiographs revealed original recycled bone was present in 7 of 11 ECIR cases and in zero cases treated with LNF autografts, indicating that recycled bone treated with LNF autografts was remodeled into new bone. Histological examination of ECIR-treated bones revealed a delayed and incomplete endochondral ossification process, necrosis and empty lacunae. Conversely, LNF autografts showed remodeled bones with normal trabecular structures. CONCLUSIONS: ECIR and LNF treatment of autografts provided adequate tumor control with acceptable clinical results as a reconstruction method.


Assuntos
Neoplasias Ósseas , Transplante Ósseo , Nitrogênio , Humanos , Neoplasias Ósseas/cirurgia , Neoplasias Ósseas/radioterapia , Neoplasias Ósseas/patologia , Transplante Ósseo/métodos , Masculino , Feminino , Adulto , Adolescente , Pessoa de Meia-Idade , Criança , Adulto Jovem , Condrossarcoma/cirurgia , Condrossarcoma/radioterapia , Condrossarcoma/patologia , Osteossarcoma/cirurgia , Osteossarcoma/patologia , Osteossarcoma/radioterapia , Sobrevivência de Enxerto , Seguimentos , Sarcoma de Ewing/cirurgia , Sarcoma de Ewing/radioterapia , Sarcoma de Ewing/patologia , Autoenxertos , Sarcoma/cirurgia , Sarcoma/radioterapia , Sarcoma/patologia , Congelamento , Neoplasias de Tecidos Moles/radioterapia , Neoplasias de Tecidos Moles/cirurgia , Neoplasias de Tecidos Moles/patologia
4.
J Surg Oncol ; 2024 Jun 30.
Artigo em Inglês | MEDLINE | ID: mdl-38946219

RESUMO

BACKGROUND: Limited understanding exists regarding early sarcoma symptoms presented during general practitioner (GP) consultations. The study explores GP visit patterns and recorded diagnoses in the 12 months preceding sarcoma diagnosis. METHODS: Sarcoma cases diagnosed from 2010 to 2020 were identified through the Netherlands Cancer Registry alongside general practice data. Sarcoma cases were age and gender matched to cancer-free controls (2:1 or 1:1 ratio). RESULTS: A total of 787 individuals with soft-tissue sarcoma (STS) and 188 individuals with bone sarcoma (BS) were identified. There was a significant difference in monthly GP contacts from 4 months to the last month before STS diagnosis, and 2 months before BS diagnosis between cases and controls. Most prevalent diagnoses recorded by the GP for STS cases included musculoskeletal neoplasm (26.6%), uncomplicated hypertension (15.6%), and cystitis/other urinary infections (12.2%). For BS cases, musculoskeletal neoplasm (42.8%), knee symptoms/complaints (9.7%), and shoulder symptoms/complaints (9.7%) were most frequent. CONCLUSIONS AND DISCUSSION: A significant difference in GP contacts between cases and controls preceding sarcoma diagnosis. STS cases were predominantly diagnosed with nonspecific symptoms, whereas BS cases with diagnoses more suggestive of BS. Better understanding of the prediagnostic trajectory could aid GPs in early identification of sarcoma.

5.
Int J Clin Oncol ; 2024 Jun 11.
Artigo em Inglês | MEDLINE | ID: mdl-38858229

RESUMO

BACKGROUND: No previous reports have characterized national bone sarcoma profiles overall. We examined the nationwide statistics for bone sarcoma in Japan using data from the National Cancer Registry (NCR), a population-based cancer registry. METHODS: We identified 3,755 patients with bone sarcomas entered in the NCR during 2016-2019 using International Classification of Diseases-Oncology, Third Edition codes for cancer topography and morphology. We extracted data on patient demographics, tumor details (reason for diagnosis, tumor location, histology, extent of disease), hospital volume/type, treatment, and prognosis for each patient. RESULTS: Bone sarcoma showed a slight male preponderance. The age distribution peaked at ages 10-20 and 60-80; approximately 44% of patients were aged over 60 years. Chordoma, chondrosarcoma, and malignant fibrous histiocytoma of bone peaked in the elderly, and Ewing's sarcoma peaked in children. Osteosarcoma had two peaks in Japan as well as in Western countries. The most frequent tumor locations were the limb (45%) and the pelvis (21%). Extent of disease was categorized as: "localized" (39%), "regional" (27%), and "distant" (11%). We found significant associations between overall survival and age, tumor location, facility type, hospital volume, histologic subtype, reason for diagnosis, and extent of disease. The latter had the poorest survival. CONCLUSIONS: This is the first study to outline the epidemiology, clinical features, treatment, prognosis, and significant factors affecting prognosis of bone sarcoma in Japan using the NCR. Documenting our data regarding elderly patients' outcomes is essential so other countries showing similar population-aging trends can learn from our experiences. LEVEL OF EVIDENCE: Prognostic studies, Level III.

6.
Biochem Genet ; 62(2): 798-813, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-37452172

RESUMO

Immune-modulating therapies exhibit abundant promise compared to traditional treatment for osteosarcoma. We aim to establish an immune-related prognostic signatures in osteosarcoma. We identified the differentially expressed genes in osteosarcoma compared with normal controls using the GEO dataset. The intersection with immune-related genes was considered as differentially expressed immune genes. Potential prognosis-related differentially expressed genes were first analyzed with the multifactor Cox regression and then the step function performed the iteration. The best model was finally chosen as the immunological risk score signature model. And finally, we evaluated the correlation of genes in the prognostic model with immune cells, common immune checkpoints, and immune checkpoint blockade responses. We identified 1527 significantly upregulated and 2407 significantly downregulated genes in osteosarcoma compared to normal samples. In the 258 differentially expressed immune genes, 20 genes with independent prognostic significance were included in the step function. Finally, we constructed a prognostic signature for overall survival based on five immune genes (JAG2, PLXNB1, CMKLR1, NUDT6, and PSMC4) in osteosarcoma. These five genes are closely related to immune infiltration. Osteosarcoma with high JAG2 expression or low CMKLR1 expression may be associated with better immune checkpoint blockade response. JAG2 overexpression or CMKLR1 inhibition induced sensitivity to PD-1 antibody in osteosarcoma cells. We constructed a prognosis prediction signature containing five immune-related genes (JAG2, PLXNB1, CMKLR1, NUDT6, and PSMC4) with a significant prognostic value in osteosarcoma. Significant differences in immune infiltration and immunotherapy responses were identified between groups with different levels of these genes.

7.
J Orthop Sci ; 2024 Jul 03.
Artigo em Inglês | MEDLINE | ID: mdl-38964957

RESUMO

BACKGROUND: Few studies have compared the clinical outcomes of patients with pelvic bone sarcomas treated surgically and those treated with particle beam therapy. This is a multicenter retrospective cohort study which compared the clinical outcomes of patients with pelvic bone sarcoma who underwent surgical treatment and particle beam therapy in Japan. METHODS: A total of 116 patients with pelvic bone sarcoma treated at 19 specialized sarcoma centers in Japan were included in this study. Fifty-seven patients underwent surgery (surgery group), and 59 patients underwent particle beam therapy (particle beam group; carbon-ion radiotherapy: 55 patients, proton: four patients). RESULTS: The median age at primary tumor diagnosis was 52 years in the surgery group and 66 years in the particle beam group (P < 0.001), and the median tumor size was 9 cm in the surgery group and 8 cm in the particle beam group (P = 0.091). Overall survival (OS), local control (LC), and metastasis-free survival (MFS) rates were evaluated using the Kaplan-Meier method and compared among 116 patients with bone sarcoma (surgery group, 57 patients; particle beam group, 59 patients). After propensity score matching, the 3-year OS, LC, and MFS rates were 82.9% (95% confidence interval [CI], 60.5-93.2%), 66.0% (95% CI, 43.3-81.3%), and 78.4% (95% CI, 55.5-90.5%), respectively, in the surgery group and 64.9% (95% CI, 41.7-80.8%), 86.4% (95% CI, 63.3-95.4%), and 62.6% (95% CI, 38.5-79.4%), respectively, in the particle beam group. In chordoma patients, only surgery was significantly correlated with worse LC in the univariate analysis. CONCLUSIONS: The groups had no significant differences in the OS, LC, and MFS rates. Among the patients with chordomas, the 3-year LC rate in the particle beam group was significantly higher than in the surgery group.

8.
Medicina (Kaunas) ; 60(5)2024 May 08.
Artigo em Inglês | MEDLINE | ID: mdl-38792961

RESUMO

Background and Objectives: Primary malignant bone tumors are rare lesions, and their complex treatment can lead to functional impairment. It is important to have a postoperative assessment tool for patients' functional outcomes to be evaluated and to consequently adapt future treatments in the pursuit of a continuous improvement of their quality of life. The Musculoskeletal Tumor Society Score (MSTS) is a validated specific system score that is used frequently in the follow-up of these patients. We found no information about a valid translated Romanian version of this score neither for the upper limb nor for the lower limb. We proposed in this study to translate the original version of the MSTS Score into Romanian and to perform validation analysis of the Romanian-language MSTS Score. Materials and Methods: We selected 48 patients who underwent limb-salvage surgery after resection of bone sarcomas. Patients were interrogated twice according to the translated Romanian version of the MSTS Score during their follow-up. The translation was performed according to the recommended guidelines. A total number of 96 questionnaires were valid for statistical analysis. Results: Internal consistency and reliability were good for both sets of questionnaires' analytic measurements, with Cronbach's alpha values of 0.848 (test) and 0.802 (retest). The test-retest evaluation proved to be statistically strong for reproducibility and validity with Spearman's rho = 0.9 (p < 0.01, 95% CI). Conclusions: This study permitted the translation of this score and the validation of psychometric data. Our results showed that the Romanian version of the MSTS is a reliable means of assessment of the functional outcome of patients who received limb-salvage surgery for the upper and lower extremities.


Assuntos
Neoplasias Ósseas , Humanos , Masculino , Feminino , Romênia , Neoplasias Ósseas/cirurgia , Adulto , Inquéritos e Questionários , Reprodutibilidade dos Testes , Pessoa de Meia-Idade , Qualidade de Vida , Osteossarcoma/cirurgia , Comparação Transcultural , Sarcoma/cirurgia , Psicometria/instrumentação , Psicometria/métodos , Traduções , Adolescente , Idoso
9.
Foot Ankle Surg ; 2024 May 29.
Artigo em Inglês | MEDLINE | ID: mdl-38849215

RESUMO

BACKGROUND: The study examines the characteristics and outcomes of foot-originating malignant bone tumors via Surveillance Epidemiology and End Results (SEER) database analysis. METHODS: A retrospective review of 14,695 malignant bone tumor cases from 2000 to 2019 was conducted. RESULTS: Of the eligible cases, 147 (2.3 %) were foot-origin tumors, typically smaller and more commonly treated with surgery than those in other locations. These tumors were more frequently treated with surgical resection, with a higher proportion undergoing amputation. In contrast, foot-origin tumors were less often managed with chemotherapy and radiation. Foot-origin tumors exhibited higher survival rates compared to non-foot-origin tumors as shown in univariate analysis, although multivariate analysis did not reflect significant differences. CONCLUSION: Foot-originating malignant bone tumors tend to be smaller and are frequently surgically treated, correlating with favorable survival outcomes. These findings point to early detection as a potential factor in the improved survival rates, not necessarily the tumor's origin.

10.
Cancer ; 129(22): 3564-3573, 2023 11 15.
Artigo em Inglês | MEDLINE | ID: mdl-37530385

RESUMO

BACKGROUND: Rare primary malignant bone sarcomas (RPMBS) account for 5%-10% of primary high-grade bone tumors and represent a major treatment challenge. The outcome of patients with RPMBS enrolled in the EUROpean Bone Over 40 Sarcoma Study (EURO-B.O.S.S) is presented. METHODS: Inclusion criteria were as follows: age from 41 to 65 years and a diagnosis of high-grade spindle cell, pleomorphic, or vascular RPMBS. The chemotherapy regimen included doxorubicin 60 mg/m2 , ifosfamide 9 g/m2 , and cisplatin 90 mg/m2 ; postoperative methotrexate 8 g/m2 was added in case of a poor histologic response. Version 2.0 of the Common Terminology Criteria for Adverse Events, Kaplan-Meier curves, log-rank tests, and univariate Cox regression models were used. RESULTS: In total, 113 patients were evaluable for analysis. The median patient age was 52 years (range, 40-66 years), and 67 patients were men. Eighty-eight tumors were categorized as undifferentiated pleomorphic sarcomas (UPS), 20 were categorized as leiomyosarcomas, three were categorized as fibrosarcomas, and two were categorized as angiosarcomas. Eighty-three of 113 tumors were located in the extremities. Ninety-five of 113 patients presented with no evidence of metastases. After a median follow-up of 6.8 years (interquartile range [IQR], 3.5-9.8 years), the 5-year overall survival rate for patients with localized disease was 68.4% (IQR, 56.9%-77.5%), and it was 71.7% (IQR, 58.1%-81.6%) for patients with UPS and 54.9% (IQR, 29.5%-74.5%) for patients with leiomyosarcoma. Grade III-IV hematologic toxicity was reported in 81% patients; 23% had grade II-III neurotoxicity, and 37.5% had grade I-II nephrotoxicity. Five-year overall survival was significantly better for patients with localized disease, for patients who obtained surgical complete remission, and when the primary tumor was located in the extremities. CONCLUSIONS: The survival of patients who had RPMBS in the current series was similar to that of age-matched patients who had high-grade osteosarcoma treated according to the same protocol. An osteosarcoma-like chemotherapy may be proposed in patients who have RPMBS.


Assuntos
Neoplasias Ósseas , Leiomiossarcoma , Osteossarcoma , Sarcoma , Masculino , Humanos , Adulto , Pessoa de Meia-Idade , Idoso , Feminino , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Sarcoma/patologia , Osteossarcoma/tratamento farmacológico , Terapia Combinada , Neoplasias Ósseas/patologia , Doxorrubicina , Ifosfamida , Leiomiossarcoma/tratamento farmacológico
11.
Histopathology ; 82(4): 531-540, 2023 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-36464647

RESUMO

BACKGROUND AND OBJECTIVES: Bone tumours are relatively rare and, as a consequence, treatment in a centre with expertise is required. Current treatment guidelines also recommend review by a specialised pathologist. Here we report on international consensus-based datasets for the pathology reporting of biopsy and resection specimens of bone sarcomas. The datasets were produced under the auspices of the International Collaboration on Cancer Reporting (ICCR), a global alliance of major (inter-)national pathology and cancer organisations. METHODS AND RESULTS: According to the ICCR's process for dataset development, an international expert panel consisting of pathologists, an oncologic orthopaedic surgeon, a medical oncologist, and a radiologist produced a set of core and noncore data items for biopsy and resection specimens based on a critical review and discussion of current evidence. All professionals involved were bone tumour experts affiliated with tertiary referral centres. Commentary was provided for each data item to explain the rationale for selecting it as a core or noncore element, its clinical relevance, and to highlight potential areas of disagreement or lack of evidence, in which case a consensus position was formulated. Following international public consultation, the documents were finalised and ratified, and the datasets, including a synoptic reporting guide, were published on the ICCR website. CONCLUSION: These first international datasets for bone sarcomas are intended to promote high-quality, standardised pathology reporting. Their widespread adoption will improve the consistency of reporting, facilitate multidisciplinary communication, and enhance comparability of data, all of which will help to improve management of bone sarcoma patients.


Assuntos
Patologia Clínica , Sarcoma , Humanos , Oncologia , Biópsia
12.
BMC Cancer ; 23(1): 742, 2023 Aug 11.
Artigo em Inglês | MEDLINE | ID: mdl-37563551

RESUMO

Bone sarcomas are rare tumors representing 0.2% of all cancers. While osteosarcoma and Ewing sarcoma mainly affect children and young adults, chondrosarcoma and chordoma have a preferential incidence in people over the age of 40. Despite this range in populations affected, all bone sarcoma patients require complex transdisciplinary management and share some similarities. The cornerstone of all bone sarcoma treatment is monobloc resection of the tumor with adequate margins in healthy surrounding tissues. Adjuvant chemo- and/or radiotherapy are often included depending on the location of the tumor, quality of resection or presence of metastases. High dose radiotherapy is largely applied to allow better local control in case of incomplete primary tumor resection or for unresectable tumors. With the development of advanced techniques such as proton, carbon ion therapy, radiotherapy is gaining popularity for the treatment of bone sarcomas, enabling the delivery of higher doses of radiation, while sparing surrounding healthy tissues. Nevertheless, bone sarcomas are radioresistant tumors, and some mechanisms involved in this radioresistance have been reported. Hypoxia for instance, can potentially be targeted to improve tumor response to radiotherapy and decrease radiation-induced cellular toxicity. In this review, the benefits and drawbacks of radiotherapy in bone sarcoma will be addressed. Finally, new strategies combining a radiosensitizing agent and radiotherapy and their applicability in bone sarcoma will be presented.


Assuntos
Neoplasias Ósseas , Condrossarcoma , Osteossarcoma , Sarcoma de Ewing , Sarcoma , Criança , Adulto Jovem , Humanos , Sarcoma/radioterapia , Sarcoma/cirurgia , Osteossarcoma/patologia , Neoplasias Ósseas/radioterapia , Neoplasias Ósseas/patologia , Sarcoma de Ewing/patologia , Condrossarcoma/radioterapia , Condrossarcoma/cirurgia
13.
BMC Cancer ; 23(1): 907, 2023 Sep 26.
Artigo em Inglês | MEDLINE | ID: mdl-37752419

RESUMO

BACKGROUND: Heterozygous isocitrate dehydrogenase (IDH) mutations occur in about half of conventional central bone chondrosarcomas (CCBC). Aim of this study was to assess the frequency and prognostic impact of IDH mutations in high grade CCBC patients. METHODS: 64 patients with G2 and G3 CCBC were included. DNA extraction, PCR amplification of IDH1/2 exon 4s, and sequencing analysis with Sanger were performed. RESULTS: IDH mutations were detected in 24/54 patients (44%): IDH1 in 18, IDH2 in 4, and both IDH1/2 in 2 patients. The frequency of mutations was 37% in G2 vs. 69% in G3 (p = 0.039), and 100% in three Ollier disease associated chondrosarcoma. 5-year overall survival (OS) at 124 months (range 1-166) was 51%, with no significant difference based on the IDH mutational status: 61% in IDHmut vs. 44% in IDH wild type (IDHwt). The 5-year relapse free survival (RFS) was 33% (95% CI:10-57) for IDHmut vs. 57% (95%CI: 30-77) for IDHwt. Progression free survival (PFS) was 25% (95%CI:1-65) IDHmut vs. 16% (95%CI: 0.7-52) IDHwt. 55% (5/9) of IDHmut G2 became higher grade at the recurrence, as compared with 25% (3/12) of G2 IDHwt. CONCLUSIONS: This study shows a higher frequency of IDH mutations in G3 CCBC as compared with G2. No significant differences in OS, RFS, and PFS by mutational status were detected. After relapse, a higher rate of G3 for IDH mutated CCBC was observed.


Assuntos
Neoplasias Ósseas , Condrossarcoma , Humanos , Isocitrato Desidrogenase/genética , Mutação , Condrossarcoma/genética , Éxons , Neoplasias Ósseas/genética
14.
J Surg Oncol ; 128(2): 367-374, 2023 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-37079428

RESUMO

INTRODUCTION: Soft tissue (ST) complications after resection of bone and ST sarcomas of the pelvis occur more frequently than in appendicular tumors. We sought to identify risk factors for complications within 30 days of surgery. METHODS: The National Surgical Quality Improvement Program database was used for this study. Patients with sarcomas of bone and ST of the pelvis were retrieved using Current Procedural Terminology and International Classification of Diseases codes. Outcomes assessed were ST complications, overall complication rates, 30-day reoperation, and mortality. RESULTS: A total of 770 patients with pelvic bone and ST sarcoma were included. The ST complication rate was 12.6%, including 4.9% superficial and 4.7% deep surgical site infections. Higher ST complication rates were seen in patients >30 years, with partially dependent health status, hematocrit <30%, bone tumors, tumor >5 cm, amputation procedures, and longer operative times. ST complication rates were 1.5 and 3 times higher in pelvic sarcoma surgeries than in the lower and upper extremities, respectively. Age >30 years (odds ratio [OR] = 5.07), hematocrit <30% (OR = 1.84), operative time 1-3 h (OR = 2.97), and >3 h (OR = 4.89) were risk factors for ST complications. CONCLUSION: One in nine patients with pelvic sarcoma surgery will develop ST complications within 30 days. Risk factors for ST complications were age >30, hematocrit <30%, and longer operative time.


Assuntos
Neoplasias Ósseas , Neoplasias Pélvicas , Sarcoma , Neoplasias de Tecidos Moles , Humanos , Adulto , Melhoria de Qualidade , Sarcoma/cirurgia , Sarcoma/patologia , Fatores de Risco , Neoplasias Ósseas/cirurgia , Neoplasias Ósseas/patologia , Neoplasias Pélvicas/patologia , Pelve/patologia , Estudos Retrospectivos , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Neoplasias de Tecidos Moles/cirurgia , Neoplasias de Tecidos Moles/patologia
15.
Pediatr Blood Cancer ; 70 Suppl 6: e30583, 2023 09.
Artigo em Inglês | MEDLINE | ID: mdl-37501549

RESUMO

The Children's Oncology Group (COG) Bone Tumor Committee is responsible for clinical trials and biological research on localized, metastatic, and recurrent osteosarcoma and Ewing sarcoma (EWS). Results of clinical trials in localized disease completed and published in the past 10 years have led to international standard-of-care chemotherapy for osteosarcoma and EWS. A recent focus on identifying disease subgroups has led to the identification of biological features associated with poor outcomes including the presence of circulating tumor DNA (ctDNA) at diagnosis, and specific genomic alterations-MYC amplification for osteosarcoma and STAG2 and TP53 mutation for EWS. Studies validating these potential biomarkers are under way. Clinical trials evaluating the addition of multitargeted kinase inhibitors, which are active in relapsed bone sarcomas, to standard chemotherapy are under way in osteosarcoma and planned in EWS. In addition, the Committee has data analyses and a clinical trial under way to evaluate approaches to local management of the primary tumor and metastatic sites. Given the rarity of bone sarcomas, we have prioritized international interactions and are in the process of forming an international data-sharing consortium to facilitate refinement of risk stratification and study of rare disease subtypes.


Assuntos
Neoplasias Ósseas , Tumores Neuroectodérmicos Primitivos Periféricos , Osteossarcoma , Sarcoma de Ewing , Criança , Humanos , Recidiva Local de Neoplasia , Neoplasias Ósseas/tratamento farmacológico , Neoplasias Ósseas/genética , Sarcoma de Ewing/tratamento farmacológico , Sarcoma de Ewing/genética , Osteossarcoma/tratamento farmacológico , Osteossarcoma/genética
16.
Pediatr Blood Cancer ; 70(3): e30135, 2023 03.
Artigo em Inglês | MEDLINE | ID: mdl-36524611

RESUMO

BACKGROUND: Diagnostic delays in cancers are frequent in developing countries due to poor health infrastructure. Existing literature from developed countries suggests that diagnostic interval in bone sarcomas is primarily dictated by tumour biology with no impact on survival. This study evaluates the social and biological determinants of the diagnostic interval in bone sarcomas in a resource-challenged setting and assesses its impact on treatment outcomes. METHODS: A retrospective single-institutional study was conducted on patients with high-grade bone sarcomas recorded in the sarcoma clinic database between 2003 and 2018. Baseline clinical characteristics and treatment outcomes were recorded. Logistic regression was performed to assess the impact of baseline clinical and social characteristics (distance from treating centre and rural vs. urban residence) on the diagnostic interval. Further, the impact of diagnostic interval on histologic response to neoadjuvant chemotherapy, amputation requirement in extremity sarcomas and survival was evaluated. RESULTS: A total of 1227 patients were included for analysis. The median diagnostic interval was 4 months (3-7 months). Age above 18 years, Ewing sarcoma (ES) diagnosis, absence of fever at presentation and tumour size above 7.5 cm were predictors of a longer diagnostic interval (>4 months). The length of the diagnostic interval did not impact amputation requirement or survival outcomes. However, the proportion of patients with good necrosis post-neoadjuvant chemotherapy was lower among patients with longer diagnostic intervals (25% vs. 34·16%; p-value = .04). CONCLUSION: Tumour characteristics rather than social factors determined the diagnostic interval. Diagnostic interval did not impact survival outcomes even in a resource-constrained setting.


Assuntos
Neoplasias Ósseas , Osteossarcoma , Sarcoma de Ewing , Sarcoma , Humanos , Adolescente , Estudos Retrospectivos , Neoplasias Ósseas/diagnóstico , Neoplasias Ósseas/terapia , Sarcoma de Ewing/diagnóstico , Sarcoma de Ewing/terapia , Sarcoma/patologia
17.
Health Qual Life Outcomes ; 21(1): 81, 2023 Jul 29.
Artigo em Inglês | MEDLINE | ID: mdl-37516838

RESUMO

BACKGROUND: Validated self-reporting tools are required to evaluate the functional outcome and health-related quality of life (HRQOL) for those who had extremity bone sarcomas in their childhood or adolescence. Our study pursued cross-cultural adaptation and validation of the pediatric Toronto Extremity Salvage Score (pTESS) and Toronto Extremity Salvage Score (TESS) to assess the functional outcome for Egyptian children and adult survivors following surgeries of extremity bone sarcomas. In the modified versions of pTESS and TESS, mental domains were added to allow the evaluation of HRQOL using a specific instrument for childhood bone cancer. METHODS: The internal consistency and test-retest reliability of the studied forms were assessed with Cronbach's alpha and Intra-class coefficients (ICC), respectively. For convergent validity, correlations between scores of the generic Pediatric Quality of Life Inventory (PedsQL 4.0) and pTESS /TESS scores were reported. Factor Analysis was feasible for pTESS-leg; due to the insufficient samples, only the average inter-item correlation coefficients were reported for the remaining versions. RESULTS: Out of 233 participants, 134 responded to pTESS-leg, 53 to TESS-leg, 36 to pTESS-arm, and only 10 to TESS-arm. All versions showed excellent internal consistency (Cronbach's alpha >0.9), good test-retest reliability (ICC >0.8), moderate to strong correlations with PedsQL, and acceptable average inter-item correlation coefficients (≥0.3). Three factors were extracted for the pTESS-leg, in which all mental items were loaded on one separate factor with factor loadings exceeding 0.4. Active chemotherapy, less than one year from primary surgery, or tibial tumors were associated with significantly inferior pTESS/TESS scores in the lower extremity group. CONCLUSION: The Egyptian pTESS and TESS are valid and reliable self-reporting tools for assessing the functional outcome following surgeries for extremity bone sarcomas. The modified pTESS and TESS versions, which include additional mental domains, enabled the assessment of the overall health status of our population. Future studies should include a larger sample size and evaluate the ability of pTESS/TESS to track progress over time.


Assuntos
Neoplasias Ósseas , Comparação Transcultural , Qualidade de Vida , Sarcoma , Adolescente , Adulto , Criança , Humanos , Egito , Extremidade Inferior , Reprodutibilidade dos Testes , Sarcoma/cirurgia , Neoplasias Ósseas/cirurgia
18.
Qual Life Res ; 2023 Dec 11.
Artigo em Inglês | MEDLINE | ID: mdl-38079025

RESUMO

PURPOSE: We conducted a systematic review of studies reporting on measurement of health-related quality of life (HRQoL), with a special focus on the use of the preference-weighted instruments, in patients with extremity bone sarcoma treated with limb-salvage surgery or amputation. METHODS: We searched MedLine, Embase, Cochrane Library and Web of Science for English-language studies reporting on HRQoL of patients with bone sarcoma from inception to 28 August 2023. All records found were independently reviewed by two reviewers. We used the Newcastle-Ottawa Scale (NOS) and the CONSORT 2010 checklist to assess the quality of the cohort and randomised studies, respectively. RESULTS: The search identified 1225 records, of which 16 studies were included for data extraction. Only one study used a preference-weighted instrument for measuring HRQoL in a small sample of patients (n = 28). Ten studies used the generic SF-36 questionnaire, but no preference-weighted HRQoL based on SF-6D was derived from the SF-36 scores. Most studies comparing HRQoL between amputation and limb-salvage surgery reported no significant differences. Twelve cohort studies scored six or more out of nine points based on the NOS. The only randomised study scored 54% on the CONSORT 2010 checklist. CONCLUSIONS: The approaches used to measure HRQoL were inconsistent and outcome scores varied substantially. Only one study used preference-weighted instruments for HRQoL measurement. Future research into the surgical treatment of extremity bone sarcoma should consider the use of preference-weighted instruments to measure HRQoL, which will therefore enable economic evaluation for the growing orthopaedic armamentarium of novel surgical interventions. REGISTRATION: This systematic review was registered with the PROSPERO International prospective register of systematic reviews (CRD42021282380).

19.
Int J Clin Oncol ; 28(1): 12-27, 2023 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-35347494

RESUMO

Bone sarcoma often occurs in childhood, as well as in adolescents and young adults (AYAs). AYAs differ from pediatric patients in that their bone is skeletally mature and the physis has almost disappeared with the completion of growth. Although AYAs spend less time outside, they often participate in sports activities, as well as driving, working, and raising a family, which are natural activities in daily living. Multidisciplinary approaches involving imaging, multi-agent chemotherapy, surgical procedures, and careful postoperative care has facilitated an increase in limb-sparing surgery for bone sarcoma. In addition, recent advances in imaging modalities and surgical techniques enables joint-preservation surgery, preserving the adjacent epiphysis, for selected patients following the careful assessment of the tumor margins and precise tumor excision. An advantage of this type of surgery is that it retains the native function of the adjacent joint, which differs from joint-prosthesis replacement, and provides excellent limb function. Various reconstruction procedures are available for joint-preserving surgery, including allograft, vascularized fibula graft, distraction osteogenesis, and tumor-devitalized autografts. However, procedure-related complications may occur, including non-union, infection, fracture, and implant failure, and surgeons should fully understand the advantages and disadvantages of these procedures. The longevity of the normal limb function for natural activities and the curative treatment without debilitation from late toxicities should be considered as a treatment goal for AYA patients. This review discusses the concept of joint-preservation surgery, types of reconstruction procedures associated with joint-preservation surgery, and current treatment outcomes.


Assuntos
Neoplasias Ósseas , Osteossarcoma , Procedimentos de Cirurgia Plástica , Sarcoma , Humanos , Adolescente , Adulto Jovem , Criança , Neoplasias Ósseas/cirurgia , Neoplasias Ósseas/patologia , Osteossarcoma/cirurgia , Osteossarcoma/patologia , Sarcoma/cirurgia , Resultado do Tratamento , Estudos Retrospectivos
20.
World J Surg Oncol ; 21(1): 187, 2023 Jun 21.
Artigo em Inglês | MEDLINE | ID: mdl-37344874

RESUMO

BACKGROUND: Computer-assisted navigation has made bone sarcoma resections more precise. However, further clinical studies involving accuracy analyses under navigation are still warranted. METHODS: A retrospective study for analysis of computer-assisted navigation accuracy was carried out. Between September 2008 and November 2017, 39 cases of bone sarcomas around the knee joint were resected under computer-assisted navigation. The control group comprised 117 cases of bone sarcomas around the knee treated by limb salvage surgery wherein bony cutting was achieved freehand. The length difference (LD) was defined as the specimen length minus the planned resection length. The LDs were detected in both groups and compared. The margin accuracy (MA) was defined as the achieved margin minus the desired margin at the bone cutting site and was detected in the navigation group. RESULTS: The LDs between the postoperative specimen length and the preoperative planned length were compared. In the navigation group, the LD was 0.5 ± 2.5 mm (range, - 5 to 5 mm), while in the freehand group, the LD was 3.4 ± 9.6 mm (range, - 20 to 29 mm), with a significant difference (P < 0.01). In the absolute value analysis, the LD absolute value was 2.0 ± 1.6 mm in the navigation group and 8.3 ± 6.0 mm in the freehand group, with a significant difference (P < 0.01). In the navigation group, the MA was 0.3 ± 1.5 mm (range, - 3 to 3 mm) and the MA absolute value was 1.1 ± 1.0 mm. CONCLUSIONS: Better accuracy can be achieved when computer-assisted navigation is conducted for bone sarcoma resection around the knee. LEVEL OF EVIDENCE: IV.


Assuntos
Neoplasias Ósseas , Osteossarcoma , Sarcoma , Cirurgia Assistida por Computador , Humanos , Estudos Retrospectivos , Osteossarcoma/cirurgia , Neoplasias Ósseas/cirurgia , Sarcoma/cirurgia , Articulação do Joelho/cirurgia , Computadores
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