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1.
Orphanet J Rare Dis ; 16(1): 294, 2021 07 02.
Artigo em Inglês | MEDLINE | ID: mdl-34215312

RESUMO

BACKGROUND: Tenosynovial Giant-Cell Tumour (TGCT) is a benign clonal neoplastic proliferation arising from the synovium, causing a variety of symptoms and often requiring repetitive surgery. This study aims to define the economic burden-from a societal perspective-associated with TGCT patients and their health-related quality of life (HRQOL) in six European countries. METHODS: This article analyses data from a multinational, multicentre, prospective observational registry, the TGCT Observational Platform Project (TOPP), involving hospitals and tertiary sarcoma centres from six European countries (Austria, France, Germany, Italy, the Netherlands, and Spain). It includes information on TGCT patients' health-related quality of life and healthcare and non-healthcare resources used at baseline (the 12-month period prior to the patients entering the registry) and after 12 months of follow-up. RESULTS: 146 TGCT patients enrolled for the study, of which 137 fulfilled the inclusion criteria. Their mean age was 44.5 years, and 62% were female. The annual average total costs associated with TGCT were €4866 at baseline and €5160 at the 12-month follow-up visit. The annual average healthcare costs associated with TGCT were €4620 at baseline, of which 67% and 18% corresponded to surgery and medical visits, respectively. At the 12-month follow-up, the mean healthcare costs amounted to €5094, with surgery representing 70% of total costs. Loss of productivity represented, on average, 5% of the total cost at baseline and 1.3% at follow-up. The most-affected HRQOL dimensions, measured with the EQ-5D-5L instrument, were pain or discomfort, mobility, and the performance of usual activities, both at baseline and at the follow-up visit. Regarding HRQOL, patients declared a mean index score of 0.75 at baseline and 0.76 at the 12-month follow-up. CONCLUSION: The results suggest that TGCT places a heavy burden on its sufferers, which increases after one year of follow-up, mainly due to the healthcare resources required-in particular, surgical procedures. As a result, this condition has a high economic impact on healthcare budgets, while the HRQOL of TGCT patients substantially deteriorates over time.


Assuntos
Efeitos Psicossociais da Doença , Qualidade de Vida , Adulto , Áustria , Cuidadores , Estudos Transversais , Europa (Continente) , Feminino , França , Alemanha , Custos de Cuidados de Saúde , Humanos , Itália , Masculino , Pessoa de Meia-Idade , Países Baixos , Assistência ao Paciente , Sistema de Registros , Perfil de Impacto da Doença , Fatores Socioeconômicos , Espanha , Inquéritos e Questionários
3.
Clin Radiol ; 76(4): 313.e1-313.e13, 2021 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-33483087

RESUMO

Over the last decades, technological developments in the field of radiology have resulted in a widespread use of imaging for personalising medicine in oncology, including patients with a sarcoma. New scanner hardware, imaging protocols, image reconstruction algorithms, radiotracers, and contrast media, enabled the assessment of the physical and biological properties of tumours associated with response to treatment. In this context, medical imaging has the potential to select sarcoma patients who do not benefit from (neo-)adjuvant treatment and facilitate treatment adaptation. Due to the biological heterogeneity in sarcomas, the challenge at hand is to acquire a practicable set of imaging features for specific sarcoma subtypes, allowing response assessment. This review provides a comprehensive overview of available clinical data on imaging-based response monitoring in sarcoma patients and future research directions. Eventually, it is expected that imaging-based response monitoring will help to achieve successful modification of (neo)adjuvant treatments and improve clinical care for these patients.


Assuntos
Imagem Multimodal , Planejamento de Assistência ao Paciente , Medicina de Precisão , Sarcoma/diagnóstico por imagem , Sarcoma/terapia , Biomarcadores , Proliferação de Células , Fibroblastos/patologia , Glucose/metabolismo , Humanos , Hipóxia/patologia , Terapia Neoadjuvante , Metástase Neoplásica , Sarcoma/irrigação sanguínea , Sarcoma/patologia
4.
ESMO Open ; 6(1): 100047, 2021 02.
Artigo em Inglês | MEDLINE | ID: mdl-33516150

RESUMO

BACKGROUND: Health-related quality of life (HRQoL) data of sarcoma survivors are scarce and the impact of age remains unclear. The aims of this population-based study were to (i) compare HRQoL scores amongst three age-groups [adolescents and young adults (AYA, aged 18-39 years), older adults (OA, aged 40-69 years) and elderly (aged ≥70 years)]; (ii) compare HRQoL of each sarcoma survivor age group with an age- and sex-matched normative population sample; (iii) determine factors associated with low HRQoL per age group. METHODS: Dutch sarcoma survivors, who were 2-10 years after diagnosis, were invited to complete the European Organization for Research and Treatment of Cancer Quality of Life Questionnaire-Core 30-questions questionnaire on HRQoL. RESULTS: In total, 1099 survivors (58% response rate) completed the questionnaire: 186 AYAs, 748 OAs and 165 elderly. The median time since diagnosis for all patients was 5.2 years. Bone sarcomas were seen in 41% of AYAs, 22% of OAs and in 16% of elderly survivors (P < 0.01). AYA and OA survivors reported statistically significant and clinically meaningful worse physical, role, cognitive, emotional and social functioning compared with a matched norm population, which was not the case for elderly survivors. AYAs reported significantly worse scores on emotional and cognitive functioning compared with OA and elderly survivors. Malignant peripheral nerve sheath tumour, osteosarcoma and chordoma were the subtypes of which survivors reported the lowest HRQoL scores in comparison with the norm. For all age groups, chemotherapy, having a bone sarcoma and having comorbidities were most frequently associated with low scores on HRQoL subscales, whereas a shorter time since diagnosis was not. CONCLUSION: In this nationwide sarcoma survivorship study, the disease and its treatment had relatively more impact on the HRQoL of AYA and OA survivors than on elderly survivors. These results emphasise the need for personalised follow-up care that not only includes risk-adjusted care related to disease relapse, but also age-adjusted care.


Assuntos
Neoplasias Ósseas , Osteossarcoma , Adolescente , Adulto , Idoso , Neoplasias Ósseas/epidemiologia , Neoplasias Ósseas/terapia , Humanos , Qualidade de Vida , Inquéritos e Questionários , Sobreviventes , Adulto Jovem
5.
Acta Oncol ; 60(1): 35-41, 2021 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-32988268

RESUMO

INTRODUCTION: A meningeal solitary fibrous tumor (SFT), also called hemangiopericytoma, is a rare mesenchymal malignancy. Due to anatomic constrains, even after macroscopic complete surgery with curative intent, the local relapse risk is still relatively high, thus increasing the risk of dedifferentiation and metastatic spread. This study aims to better define the role of postoperative radiotherapy (RT) in meningeal SFTs. PATIENTS AND METHODS: A retrospective study was performed across seven sarcoma centers. Clinical information was retrieved from all adult patients with meningeal primary localized SFT treated between 1990 and 2018 with surgery alone (S) compared to those that also received postoperative RT (S + RT). Differences in treatment characteristics between subgroups were tested using independent samples t-test for continuous variables and chi-square tests for proportions. Local control (LC) and overall survival (OS) rates were calculated as time from start of treatment until progression or death from any cause. LC and OS in groups receiving S or S + RT were compared using Kaplan-Meier survival curves. RESULTS: Among a total of 48 patients, 7 (15%) underwent S and 41 (85%) underwent S + RT. Median FU was 65 months. LC was significantly associated with treatment. LC after S at 60 months was 60% versus 90% after S + RT (p = 0.052). Furthermore, R1 resection status was significantly associated with worse LC (HR 4.08, p = 0.038). OS was predominantly associated with the mitotic count (HR 3.10, p = 0.011). CONCLUSION: This retrospective study, investigating postoperative RT in primary localized meningeal SFT patients, suggests that combining RT to surgery in the management of this patient population may reduce the risk for local failures.


Assuntos
Hemangiopericitoma , Neoplasias Meníngeas , Tumores Fibrosos Solitários , Adulto , Hemangiopericitoma/radioterapia , Hemangiopericitoma/cirurgia , Humanos , Neoplasias Meníngeas/radioterapia , Neoplasias Meníngeas/cirurgia , Recidiva Local de Neoplasia , Estudos Retrospectivos , Tumores Fibrosos Solitários/radioterapia , Tumores Fibrosos Solitários/cirurgia
6.
Calcif Tissue Int ; 108(3): 346-353, 2021 03.
Artigo em Inglês | MEDLINE | ID: mdl-33226445

RESUMO

Malignant transformation of fibrous dysplasia lesions has been reported in patients with fibrous dysplasia/McCune-Albright syndrome (FD/MAS). Recently, we have observed an increased risk for breast cancer. In this study, the prevalence of skeletal and extraskeletal malignancies in patients with FD/MAS in the Netherlands was assessed by analyzing data from our cohort of FD/MAS patients, the Dutch Pathology Registry (PALGA), and the Netherlands Cancer Registry (NCR). We extracted data on sex, age at diagnosis of FD/MAS, type of FD/MAS, type of malignancy, and age at diagnosis of malignancy and histology of bone and malignant tissue when available, including GNAS-mutation analysis from patients' medical records. Standardized Morbidity Ratios (SMRs) with 95% confidence intervals were calculated. Twelve malignancies were identified in the LUMC FD/MAS cohort and 100 in the PALGA cohort. In this cohort, SMR was increased for osteosarcoma (19.7, 95% CI 3.5-48.9), cervical cancer (4.93, 95%CI 1.7-8.2), thyroid cancer (3.71, 95% CI 1.1-7.8), prostate cancer (3.08, 95% CI 1.8-4.6), and melanoma (2.01, 95%CI 1.2-3.1). SMRs for pancreatic cancer or hepatocellular carcinoma could not be calculated due to low numbers. The small number of malignancies identified in our FD/MAS cohort precluded the calculation of SMRs for our cohort specifically. Our findings show that patients with FD/MAS appear to have an increased risk for osteosarcoma, cervical, thyroid, and prostate cancer and melanoma. However, these data should be interpreted with caution, as true incidence rates of the identified malignancies may be influenced by the inclusion of only patients with histologically confirmed FD/MAS. The etiology of this increased risk for malignancies still needs to be elucidated.


Assuntos
Displasia Fibrosa Poliostótica , Neoplasias , Displasia Fibrosa Poliostótica/epidemiologia , Humanos , Neoplasias/epidemiologia , Países Baixos , Prevalência , Sistema de Registros
7.
J Bone Joint Surg Am ; 102(19): 1703-1713, 2020 10 07.
Artigo em Inglês | MEDLINE | ID: mdl-33027124

RESUMO

BACKGROUND: Osteofibrous dysplasia-like adamantinoma (OFD-AD) and classic adamantinoma (AD) are rare, neoplastic diseases with only limited data supporting current treatment protocols. We believe that our retrospective multicenter cohort study is the largest analysis of patients with adamantinoma to date. The primary purpose of this study was to describe the disease characteristics and evaluate the oncological outcomes. The secondary purpose was to identify risk factors for local recurrence after surgical treatment and propose treatment guidelines. METHODS: Three hundred and eighteen confirmed cases of OFD-AD and AD for which primary treatment was carried out between 1985 and 2015 were submitted by 22 tertiary bone tumor centers. Proposed clinical risk factors for local recurrence such as size, type, and margins were analyzed using univariable and multivariate Cox regression analysis. RESULTS: Of the 318 cases, 128 were OFD-AD and 190 were AD. The mean age at diagnosis was 17 years (median, 14.5 years) for OFD-AD and 32 years (median, 28 years) for AD; 53% of the patients were female. The mean tumor size in the OFD-AD and AD groups combined was 7.8 cm, measured histologically. Sixteen percent of the patients sustained a pathological fracture prior to treatment. Local recurrence was recorded in 22% of the OFD-AD cases and 24% of the AD cases. None of the recurrences in the OFD-AD group progressed to AD. Metastatic disease was found in 18% of the AD cases and fatal disease, in 11% of the AD cases. No metastatic or fatal disease was reported in the OFD-AD group. Multivariate Cox regression analysis demonstrated that uncontaminated resection margins (hazard ratio [HR] = 0.164, 95% confidence interval [CI] = 0.092 to 0.290, p < 0.001), pathological fracture (HR = 1.968, 95% CI = 1.076 to 3.600, p = 0.028), and sex (female versus male: HR = 0.535, 95% CI = 0.300 to 0.952, p = 0.033) impacted the risk of local recurrence. CONCLUSIONS: OFD-AD and AD are parts of a disease spectrum but should be regarded as different entities. Our results support reclassification of OFD-AD into the intermediate locally aggressive category, based on the local recurrence rate of 22% and absence of metastases. In our study, metastatic disease was restricted to the AD group (an 18% rate). We advocate wide resection with uncontaminated margins including bone and involved periosteum for both OFD-AD and AD. LEVEL OF EVIDENCE: Prognostic Level IV. See Instructions for Authors for a complete description of levels of evidence.


Assuntos
Adamantinoma/cirurgia , Doenças do Desenvolvimento Ósseo/cirurgia , Neoplasias Ósseas/cirurgia , Adamantinoma/patologia , Adolescente , Adulto , Doenças do Desenvolvimento Ósseo/patologia , Neoplasias Ósseas/patologia , Feminino , Humanos , Masculino , Recidiva Local de Neoplasia , Prognóstico , Fatores de Risco , Resultado do Tratamento
8.
Surg Oncol ; 35: 261-267, 2020 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-32932224

RESUMO

INTRODUCTION: Recurrence rates remain high after surgical treatment of diffuse-type Tenosynovial Giant Cell Tumour (TGCT). Imatinib Mesylate (IM) blocks Colony Stimulating Factor1 Receptor (CSF1R), the driver mechanism in TGCT. The aim of this study was to determine if IM reduces the tumour metabolic activity evaluated by PET-CT and to compare this response with the response seen on MR imaging. MATERIALS AND METHODS: 25 Consecutive patients treated with IM (off label use) for locally advanced (N = 12) or recurrent (N = 13) diffuse-type TGCT were included, 15 male and median age at diagnosis 39 (IQR 31-47) years. The knee was most frequently affected (n = 16; 64%). The effect of IM was assessed pre- and post-IM treatment by comparing MR scans and PET-CT. MR scans were assessed by Tumour Volume Score (TVS), an estimation of the tumour volume as a percentage of the total synovial cavity. PET-CT scans were evaluated based on maximum standardized uptake value (SUV-max). Partial response was defined as more than 50% tumour reduction with TVS and a decrease of at least 30% on SUV-max. RESULTS: Median duration of IM treatment was 7.0 (IQR 4.2-11.5) months. Twenty patients (80%) discontinued IM treatment for poor response or intended surgery. Twenty patients experienced an adverse event grade 1-2, three patients grade 3 (creatinine increment, neutropenic sepsis, liver dysfunction). MR assessment of all joints showed 32% (6/19) partial response and 63% (12/19) stable disease, with a mean difference of 12% (P = 0.467; CI -22.4-46.0) TVS between pre- and post-IM and a significant mean difference of 23% (P = 0.021; CI 4.2-21.6) in all knee lesions. PET-CT, all joints, showed a significantly decreased mean difference of 5.3 (P = 0.004; CI 1.9-8.7) SUV-max between pre- and post-IM treatment (58% (11/19) partial response, 37% (7/19) stable disease). No correlation between MR imaging and PET-CT could be appreciated in 15 patients with complete radiological data. CONCLUSION: This study confirms the moderate radiological response of IM in diffuse-type TGCT. PET-CT is a valuable additional diagnostic tool to quantify response to tyrosine kinase inhibitor treatment. Its value should be assessed further to validate its efficacy in the objective measurement of biological response in targeted systemic treatment of TGCT.


Assuntos
Antineoplásicos/farmacologia , Tumor de Células Gigantes de Bainha Tendinosa/tratamento farmacológico , Mesilato de Imatinib/farmacologia , Adulto , Idoso , Inglaterra/epidemiologia , Feminino , Tumor de Células Gigantes de Bainha Tendinosa/diagnóstico por imagem , Tumor de Células Gigantes de Bainha Tendinosa/patologia , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/epidemiologia , Recidiva Local de Neoplasia/patologia , Tomografia por Emissão de Pósitrons , Estudos Retrospectivos , Resultado do Tratamento
9.
J Bone Joint Surg Am ; 102(12): 1042-1049, 2020 Jun 17.
Artigo em Inglês | MEDLINE | ID: mdl-32265356

RESUMO

BACKGROUND: Improved survival rates for patients with primary bone tumors of the extremities have increased the demand for reliable and durable reconstruction techniques. Some authors have stated that, after successful ingrowth, allografts are a durable long-term solution. This hypothesis is largely based on small studies with short-to-midterm follow-up. In order to determine the durability of intercalary allograft reconstructions in the lower extremities, we evaluated the long-term clinical outcomes at a minimum of 10 years. METHODS: All patients who received an intercalary allograft reconstruction in a lower extremity between 1980 and 2006 were included in this retrospective multicenter cohort study. One hundred and thirty-one patients with a median age of 19 years were included. Eighty-nine (68%) had a femoral reconstruction, and 42 (32%) had a tibial reconstruction. The most prevalent diagnoses were osteosarcoma (55%), Ewing sarcoma (17%), and chondrosarcoma (12%). The median follow-up was 14 years. A competing risk model was employed to estimate the cumulative incidences of mechanical failure and infection. Patient mortality or progression of the disease was used as a competing event. RESULTS: Nonunion occurred in 21 reconstructions (16%), after a median of 16 months, and was associated with intramedullary nail-only fixation (p < 0.01) and fixation with nonbridging plate(s) (p = 0.03). Allograft fracture occurred in 25 reconstructions (19%) after a median of 42 months (range, 4 days to 21.9 years). Thirteen (52%) of the allograft fractures occurred within 5 years; 8 (32%), between 5 and 10 years; and 4 (16%), at >10 years. With failure for mechanical reasons as the end point, the cumulative incidences of reconstruction failure at 5, 10, and 15 years were 9%, 14%, and 21%, respectively. CONCLUSIONS: Intercalary allograft reconstruction is an acceptable reconstructive option, mainly because of the absence of superior alternatives with a known track record. However, a considerable and continuing risk of mechanical complications should be taken into account. LEVEL OF EVIDENCE: Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.


Assuntos
Neoplasias Ósseas/cirurgia , Transplante Ósseo/efeitos adversos , Fêmur , Complicações Pós-Operatórias/epidemiologia , Procedimentos Cirúrgicos Reconstrutivos/efeitos adversos , Tíbia , Adolescente , Adulto , Idoso , Neoplasias Ósseas/diagnóstico por imagem , Neoplasias Ósseas/patologia , Placas Ósseas , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/diagnóstico por imagem , Estudos Retrospectivos , Sarcoma/diagnóstico por imagem , Sarcoma/patologia , Sarcoma/cirurgia , Fatores de Tempo , Resultado do Tratamento , Adulto Jovem
10.
Sci Rep ; 9(1): 14551, 2019 10 10.
Artigo em Inglês | MEDLINE | ID: mdl-31601938

RESUMO

Tenosynovial giant cell tumors (TGCT), are rare colony stimulating factor-1(CSF-1)-driven proliferative disorders affecting joints. Diffuse-type TGCT often causes significant morbidity due to local recurrences necessitating multiple surgeries. Imatinib mesylate (IM) blocks the CSF-1 receptor. This study investigated the long term effects of IM in TGCT. We conducted an international multi-institutional retrospective study to assess the activity of IM: data was collected anonymously from individual patients with locally advanced, recurrent or metastatic TGCT. Sixty-two patients from 12 institutions across Europe, Australia and the United States were identified. Four patients with metastatic TGCT progressed rapidly on IM and were excluded for further analyses. Seventeen of 58 evaluable patients achieved complete response (CR) or partial response (PR). One- and five-year progression-free survival rates were 71% and 48%, respectively. Thirty-eight (66%) patients discontinued IM after a median of 7 (range 1-80) months. Reported adverse events in 45 (78%) patients were among other edema (48%) and fatigue (50%), mostly grade 1-2 (89%). Five patients experienced grade 3-4 toxicities. This study confirms, with additional follow-up, the efficacy of IM in TGCT. In responding cases we confirmed prolonged IM activity on TGCT symptoms even after discontinuation, but with high rates of treatment interruption and additional treatments.


Assuntos
Antineoplásicos/uso terapêutico , Tumor de Células Gigantes de Bainha Tendinosa/tratamento farmacológico , Mesilato de Imatinib/uso terapêutico , Adulto , Austrália , Progressão da Doença , Intervalo Livre de Doença , Europa (Continente) , Feminino , Humanos , Pessoa de Meia-Idade , Metástase Neoplásica , Receptores de Fator Estimulador de Colônias/antagonistas & inibidores , Estudos Retrospectivos , Resultado do Tratamento , Estados Unidos
11.
Pediatr Blood Cancer ; 66(11): e27943, 2019 11.
Artigo em Inglês | MEDLINE | ID: mdl-31389188

RESUMO

BACKGROUND: We investigated the effects of surgical margins, histological response, and radiotherapy on local recurrence (LR), distant metastasis (DM), and survival in Ewing sarcoma. PROCEDURE: Disease evolution was retrospectively studied in 982 patients with Ewing sarcoma undergoing surgery after chemotherapy using a multistate model with initial state surgery, intermediate states LR, pulmonary metastasis (DMpulm), other DM ± LR (DMother), and final state death. Effect of risk factors was estimated using Cox proportional hazard models. RESULTS: The median follow-up was 7.6 years (95% CI, 7.2-8.0). Risk factors for LR are pelvic location, HR 2.04 (1.10-3.80), marginal/intralesional resection, HR 2.28 (1.25-4.16), and radiotherapy, HR 0.52 (0.28-0.95); for DMpulm the risk factors are <90% necrosis, HR 2.13 (1.13-4.00), and previous pulmonary metastasis, HR 4.90 (2.28-8.52); for DMother are 90% to 99% necrosis, HR 1.56 (1.09-2.23), <90% necrosis, HR 2.66 (1.87-3.79), previous bone/other metastasis, HR 3.08 (2.03-4.70); and risk factors for death without LR/DM are pulmonary metastasis, HR 8.08 (4.01-16.29), bone/other metastasis, HR 10.23 (4.90-21.36), and <90% necrosis, HR 6.35 (3.18-12.69). Early LR (0-24 months) negatively influences survival, HR 3.79 (1.34-10.76). Once DMpulm/DMother arise only previous bone/other metastasis remain prognostic for death, HR 1.74 (1.10-2.75). CONCLUSION: Disease extent and histological response are risk factors for progression to DM or death. Tumor site and surgical margins are risk factors for LR. If disease progression occurs, previous risk factors lose their relevance. In case of isolated LR, time to recurrence is important for decision-making. Radiotherapy seems protective for LR especially in pelvic/axial. Low percentages of LR in extremity tumors and associated toxicity question the need for radiotherapy in extremity Ewing sarcoma.


Assuntos
Neoplasias Ósseas/patologia , Modelos Biológicos , Sarcoma de Ewing/patologia , Adolescente , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Neoplasias Ósseas/secundário , Neoplasias Ósseas/terapia , Criança , Pré-Escolar , Terapia Combinada , Progressão da Doença , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Estimativa de Kaplan-Meier , Masculino , Margens de Excisão , Metástase Neoplásica , Recidiva Local de Neoplasia/epidemiologia , Neoplasia Residual , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Medição de Risco , Sarcoma de Ewing/secundário , Sarcoma de Ewing/terapia
12.
J Bone Joint Surg Am ; 101(14): 1309-1318, 2019 07 17.
Artigo em Inglês | MEDLINE | ID: mdl-31318811

RESUMO

BACKGROUND: Localized-type tenosynovial giant cell tumor (TGCT) is a rare, neoplastic disease with only limited data supporting treatment protocols. We describe treatment protocols and evaluate their oncological outcome, complications, and functional results in a large multicenter cohort of patients. A secondary study aim was to identify factors associated with local recurrence after surgical treatment. METHODS: Patients with histologically proven localized TGCT of a large joint were included if they had been treated between 1990 and 2017 in 1 of 31 tertiary sarcoma centers. Of 941 patients with localized TGCT, 62% were female. The median age at initial treatment was 39 years, and the median duration of follow-up was 34 months. Sixty-seven percent of the tumors affected the knee, and the primary treatment at the tertiary center was 1-stage open resection in 73% of the patients. Proposed factors for predicting a first local recurrence after treatment in the tertiary center were tested in a univariate analysis, and those that demonstrated significance were subsequently included in a multivariate analysis. RESULTS: The localized TGCT recurred in 12% of all cases, with local-recurrence-free rates at 3, 5, and 10 years of 88%, 83%, and 79%, respectively. The strongest factor for predicting recurrent disease was a prior recurrence (p < 0.001). Surgical treatment decreased pain and swelling in 71% and 85% of the patients, respectively, and such treatment was associated with complications in 4% of the patients. Univariate and multivariate analyses of the patients who had not undergone therapy previously yielded positive associations between local recurrence and a tumor size of ≥5 cm versus <5 cm (hazard ratio [HR] = 2.50; 95% confidence interval [CI] = 1.32 to 4.74; p = 0.005). Arthroscopy (versus open surgery) was significantly associated with tumor recurrence in the univariate analysis (p = 0.04) but not in the multivariate analysis (p = 0.056). CONCLUSIONS: Factors associated with recurrence after resection of localized-type TGCT were larger tumor size and initial treatment with arthroscopy. Relatively low complication rates and good functional outcomes warrant an open approach with complete resection when possible to reduce recurrence rates in high-risk patients. LEVEL OF EVIDENCE: Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence.


Assuntos
Tumor de Células Gigantes de Bainha Tendinosa/cirurgia , Artropatias/cirurgia , Sarcoma/cirurgia , Adulto , Artroscopia , Bases de Dados Factuais , Feminino , Humanos , Masculino , Recidiva Local de Neoplasia , Complicações Pós-Operatórias
13.
Sci Rep ; 9(1): 11000, 2019 07 29.
Artigo em Inglês | MEDLINE | ID: mdl-31358784

RESUMO

Accurate survival estimations in Ewing sarcoma are necessary to develop risk- and response adaptive treatment strategies allowing for early decision-making. We aim to develop an easy-to-use survival estimation tool from diagnosis and surgery. A retrospective study of 1314 Ewing sarcoma patients was performed. Associations between prognostic variables at diagnosis/surgery and overall survival (OS), were investigated using Kaplan-Meier and multivariate Cox models. Predictive accuracy was evaluated by cross-validation and Harrell C-statistics. Median follow-up was 7.9 years (95%CI 7.6-8.3). Independent prognostic factors at diagnosis were age, volume, primary tumor localization and disease extent. 5 risk categories (A-E) were identified with 5-year OS of 88% (86-94), 69% (64-74), 57% (50-64), 51% (42-60) and 28% (22-34) respectively. Harrell C-statistic was 0.70. Independent prognostic factors from surgery were age, volume, disease extent and histological response. In categories A-B, 5y OS increased to 92% (87-97) and 79% (71-87) respectively for 100% necrosis and decreased to 76% (67-85) and 62% (55-69) respectively for <100% necrosis. In categories C-E, 5y OS increased to 65% (55-75), 65% (52-78) and 52% (38-66) respectively for ≥90% necrosis and decreased to 38% (22-54), 11% (0-26) and 7% (0-19) respectively for <90% necrosis. We present an easy-to-use survival estimation tool from diagnosis in Ewing sarcoma based on age, volume, primary tumor localization and disease extent. Histological response is a strong additional prognostic factor for OS.


Assuntos
Neoplasias Ósseas/diagnóstico , Neoplasias Ósseas/cirurgia , Sarcoma de Ewing/diagnóstico , Sarcoma de Ewing/cirurgia , Adolescente , Adulto , Neoplasias Ósseas/epidemiologia , Criança , Feminino , Humanos , Masculino , Prognóstico , Modelos de Riscos Proporcionais , Estudos Retrospectivos , Sarcoma de Ewing/epidemiologia , Análise de Sobrevida , Adulto Jovem
14.
J Bone Joint Surg Am ; 101(9): 797-803, 2019 May 01.
Artigo em Inglês | MEDLINE | ID: mdl-31045667

RESUMO

BACKGROUND: Periprosthetic infections after pelvic reconstruction are common, with reported rates ranging from 11% to 53%. Management of these infections is troublesome, as they commonly necessitate multiple surgical interventions and implant removal. The epidemiology and outcomes of these infections are largely unknown. The aim of this study was to analyze the causative microorganisms and the clinical outcome of treatment in a series of patients with pelvic endoprostheses affected by infection following tumor resection. METHODS: In this retrospective, multicenter cohort study, we identified all patients who developed an infection after endoprosthetic reconstruction in periacetabular tumor resection, between 2003 and 2017. The microorganisms that were isolated during the first debridement were recorded, as were the number of reoperations for ongoing infection, the antimicrobial treatment strategy, and the outcome of treatment. RESULTS: In a series of 70 patients who underwent pelvic endoprosthetic reconstruction, 18 (26%) developed an infection. The type of pelvic resection according to the Enneking-Dunham classification was type P2-3 in 14 (78%) of these patients and type P2 in 4 (22%). Median follow-up was 66 months. Fourteen (78%) of the 18 patients with infection had a polymicrobial infection. Enterobacteriaceae were identified on culture for 12 (67%). Of a total 42 times that a microorganism was isolated, the identified pathogen was gram-negative in 26 instances (62%). Microorganisms associated with intestinal flora were identified 32 times (76%). At the time of latest follow-up, 9 (50%) of the patients had the original implant in situ. Of these, 2 had a fistula and another 2 were receiving suppressive antibiotic therapy. In the remaining 9 (50%) of the patients, the original implant had been removed. At the time of final follow-up, 3 of these had a second implant in situ. The remaining 6 patients had undergone no secondary reconstruction. CONCLUSIONS: Infections that affect pelvic endoprostheses are predominantly polymicrobial and caused by gram-negative microorganisms, and may be associated with intestinal flora. This differs fundamentally from mono-bacterial gram-positive causes of conventional periprosthetic joint infections and may indicate a different pathogenesis. Our results suggest that prophylaxis and empiric treatment may need to be re-evaluated. LEVEL OF EVIDENCE: Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.


Assuntos
Artroplastia de Quadril/efeitos adversos , Neoplasias Ósseas/cirurgia , Bactérias Gram-Negativas/isolamento & purificação , Infecções por Bactérias Gram-Negativas/diagnóstico , Ossos Pélvicos , Infecções Relacionadas à Prótese/microbiologia , Adulto , Idoso , Desbridamento , Feminino , Infecções por Bactérias Gram-Negativas/etiologia , Prótese de Quadril/efeitos adversos , Humanos , Masculino , Pessoa de Meia-Idade , Reoperação , Estudos Retrospectivos , Adulto Jovem
15.
Skeletal Radiol ; 48(11): 1735-1746, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31016339

RESUMO

OBJECTIVE: To determine the level of discrepancy between magnetic resonance imaging (MRI) and 18F-FDG PET-CT in detecting osseous metastases in patients with Ewing sarcoma. METHODS: Twenty patients with histopathologically confirmed Ewing sarcoma between 2000 and 2017 who underwent 18F-FDG PET-CT and MRI within a 4-week range were included. Each imaging modality was evaluated by a separate observer. Reference diagnosis of each lesion was based on histopathology or consensus of an expert panel using all available data, including at least 6 months' follow-up. Sensitivity, specificity, and predictive values were determined. Osseous lesions were analyzed on a patient and a lesion basis. Factors possibly related to false-negative findings were evaluated using Pearson's Chi-squared or Fisher's exact test. RESULTS: A total of 112 osseous lesions were diagnosed in 13 patients, 107 malignant and 5 benign. Seven patients showed no metastases on either 18F-FDG PET-CT or MRI. Forty-one skeletal metastases (39%) detected with MRI did not show increased 18F-FDG uptake on 18F-FDG PET-CT (false-negative). Lesion-based sensitivities and specificities were 62% (95%CI 52-71%) and 100% (48-100%) for 18F-FDG PET-CT; and 99% (97-100%) and 100% (48-100%) for MRI respectively. Bone lesions were more likely to be false-negative on 18F-FDG PET-CT if hematopoietic bone marrow extension was widespread and active (p = 0.001), during or after (neo)-adjuvant treatment (p = 0.001) or when the lesion was smaller than 10 mm (p < 0.001). CONCLUSION: Although no definite conclusions can be drawn from this small retrospective study, it shows that caution is needed when using 18F-FDG PET-CT for diagnosing skeletal metastases in Ewing sarcoma. Poor contrast between metastases and active hematopoietic bone marrow, chemotherapeutic treatment, and/or small size significantly decrease the diagnostic yield of 18F-FDG PET-CT, but not of MRI.


Assuntos
Neoplasias Ósseas/diagnóstico por imagem , Fluordesoxiglucose F18 , Imageamento por Ressonância Magnética/métodos , Segunda Neoplasia Primária/diagnóstico por imagem , Tomografia por Emissão de Pósitrons combinada à Tomografia Computadorizada/métodos , Compostos Radiofarmacêuticos , Sarcoma de Ewing/diagnóstico por imagem , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Reprodutibilidade dos Testes , Estudos Retrospectivos , Sarcoma de Ewing/patologia , Sensibilidade e Especificidade , Adulto Jovem
16.
Bone Joint J ; 101-B(3): 272-280, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30813787

RESUMO

AIMS: The aim of this study was to evaluate health-related quality of life (HRQoL) and joint function in tenosynovial giant cell tumour (TGCT) patients before and after surgical treatment. PATIENTS AND METHODS: This prospective cohort study run in two Dutch referral centres assessed patient-reported outcome measures (PROMs; 36-Item Short-Form Health Survey (SF-36), visual analogue scale (VAS) for pain, and Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC)) in 359 consecutive patients with localized- and diffuse-type TGCT of large joints. Patients with recurrent disease (n = 121) and a wait-and-see policy (n = 32) were excluded. Collected data were analyzed at specified time intervals preoperatively (baseline) and/or postoperatively up to five years. RESULTS: A total of 206 TGCT patients, 108 localized- and 98 diffuse-type, were analyzed. Median age at diagnosis of localized- and diffuse-type was 41 years (interquartile range (IQR) 29 to 49) and 37 years (IQR 27 to 47), respectively. SF-36 analyses showed statistically significant and clinically relevant deteriorated preoperative and immediate postoperative scores compared with general Dutch population means, depending on subscale and TGCT subtype. After three to six months of follow-up, these scores improved to general population means and continued to be fairly stable over the following years. VAS scores, for both subtypes, showed no statistically significant or clinically relevant differences pre- or postoperatively. In diffuse-type patients, the improvement in median WOMAC score was statistically significant and clinically relevant preoperatively versus six to 24 months postoperatively, and remained up to five years' follow-up. CONCLUSION: Patients with TGCT report a better HRQoL and joint function after surgery. Pain scores, which vary hugely between patients and in patients over time, did not improve. A disease-specific PROM would help to decipher the impact of TGCT on patients' daily life and functioning in more detail. Cite this article: Bone Joint J 2019;101-B:272-280.


Assuntos
Tumor de Células Gigantes de Bainha Tendinosa/cirurgia , Recidiva Local de Neoplasia/cirurgia , Medidas de Resultados Relatados pelo Paciente , Qualidade de Vida , Atividades Cotidianas , Adulto , Feminino , Tumor de Células Gigantes de Bainha Tendinosa/reabilitação , Humanos , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/reabilitação , Estudos Prospectivos , Amplitude de Movimento Articular , Recuperação de Função Fisiológica
17.
Surg Oncol ; 27(4): 695-701, 2018 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-30449495

RESUMO

PURPOSE: There is increasing interest in personalized prediction of disease progression for soft tissue sarcoma patients. Currently, available prediction models are limited to predictions from time of surgery or diagnosis. This study updates predictions of overall survival at different times during follow-up by using the concept of dynamic prediction. PATIENTS AND METHODS: Information from 2232 patients with high-grade extremity soft tissue sarcoma, who underwent surgery at 14 specialized sarcoma centers, was used to develop a dynamic prediction model. The model provides updated 5-year survival probabilities from different prediction time points during follow-up. Baseline covariates as well as time-dependent covariates, such as status of local recurrence and distant metastases, were included in the model. In addition, the effect of covariates over time was investigated and modelled accordingly in the prediction model. RESULTS: Surgical margin and tumor histology show a significant time-varying effect on overall survival. The effect of margin is strongest shortly after surgery and diminishes slightly over time. Development of local recurrence and distant metastases during follow-up have a strong effect on overall survival and updated predictions must account for their occurrence. CONCLUSION: The presence of time-varying effects, as well as the effect of local recurrence and distant metastases on survival, suggest the importance of updating predictions during follow-up. This newly developed dynamic prediction model which updates survival probabilities over time can be used to make better individualized treatment decisions based on a dynamic assessment of a patient's prognosis.


Assuntos
Extremidades/patologia , Recidiva Local de Neoplasia/mortalidade , Sarcoma/mortalidade , Progressão da Doença , Extremidades/cirurgia , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Gradação de Tumores , Metástase Neoplásica , Recidiva Local de Neoplasia/patologia , Recidiva Local de Neoplasia/cirurgia , Valor Preditivo dos Testes , Estudos Retrospectivos , Fatores de Risco , Sarcoma/patologia , Sarcoma/cirurgia , Taxa de Sobrevida
18.
Bone Joint J ; 100-B(10): 1392-1398, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-30295518

RESUMO

AIMS: The aim of this study was to assess the current trends in the estimation of survival and the preferred forms of treatment of pathological fractures among national and international general and oncological orthopaedic surgeons, and to explore whether improvements in the management of these patients could be identified in this way. MATERIALS AND METHODS: All members of the Dutch Orthopaedic Society (DOS) and the European Musculoskeletal Oncology Society (EMSOS) were invited to complete a web-based questionnaire containing 12 cases. RESULTS: A total of 96 of 948 members of the DOS (10.1%; groups 1 and 2) and 33 of 182 members of the EMSOS (18%; group 3) replied. The estimation of survival was accurate by more than 50% of all three groups, if the expected survival was short (< 3 months) or long (> 12 months). General orthopaedic surgeons preferred using an intramedullary nail for fractures of the humerus and femur, irrespective of the expected survival or the origin of primary tumour or the location of the fracture. Oncological orthopaedic surgeons recommended prosthetic reconstruction in patients with a long expected survival. CONCLUSION: Identifying patients who require centralized care, as opposed to those who can be adequately treated in a regional centre, can improve the management of patients with pathological fractures. This differentiation should be based on the expected survival, the type and extent of the tumour, and the location of the fracture. Cite this article: Bone Joint J 2018;100-B:1392-8.


Assuntos
Fixação de Fratura/tendências , Fraturas Espontâneas/cirurgia , Neoplasias/complicações , Padrões de Prática Médica/tendências , Adolescente , Adulto , Europa (Continente) , Feminino , Fixação de Fratura/métodos , Fraturas Espontâneas/etiologia , Fraturas Espontâneas/mortalidade , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias/mortalidade , Inquéritos e Questionários , Adulto Jovem
19.
Surg Oncol ; 27(3): 402-408, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-30217294

RESUMO

BACKGROUND: There are few studies detailing the incidence, patient outcomes and prognostic factors for chondrosarcomas (CS). Those that do exist have small sample sizes and/or use older datasets. The purpose of this study was to determine the incidence, overall survival (OS) and prognostic factors for OS of CS patients, as well as investigate the efficacy of curettage. METHODS: We analyzed data of 2186 patients diagnosed with chondrosarcomas between '89-'13 from the Netherlands Cancer Registry. The effect of risk factors on OS was assessed with a multivariate Cox regression. Median Follow-up was determined with reversed Kaplan-Meier. OS was estimated using Kaplan-Meier method. RESULTS: The relative incidence of CS was 2.88 per million citizens between '89-'96, 4.15 between '96-'04 and 8.78 between '05-'13. Most of the increase in incidence came from atypical cartilaginous tumours/grade I chondrosarcoma (ACT/CS I). The 3-, 5- and 10-years survival were, respectively, 96%, 93% and 88% for ACT/CS I, 82%, 74% and 62% for grade II CS and 38%, 31% and 26% for grade III CS. Prognostics factors significantly associated with OS were age, histological grade, year of diagnosis, tumour location and size. CONCLUSION: The incidence of CS, and especially ACT/CS I, has increased over time, which could be driven by both an ageing population and increased diagnostic imaging. With the increased number of diagnosed ACT/CS I, the number of preventative curettages of this tumour has also increased. Despite the supposed preventative character of this treatment, the incidence of high-grade CS did not decrease.


Assuntos
Neoplasias Ósseas/epidemiologia , Neoplasias Ósseas/mortalidade , Condrossarcoma/epidemiologia , Condrossarcoma/mortalidade , Adolescente , Adulto , Neoplasias Ósseas/patologia , Criança , Pré-Escolar , Condrossarcoma/patologia , Feminino , Seguimentos , Humanos , Incidência , Lactente , Recém-Nascido , Masculino , Países Baixos/epidemiologia , Prognóstico , Estudos Retrospectivos , Fatores de Risco , Taxa de Sobrevida , Adulto Jovem
20.
Surg Oncol ; 27(3): 544-550, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-30217317

RESUMO

AIM: Current development of novel systemic agents requires identification and monitoring of extensive Tenosynovial Giant Cell Tumours (TGCT). This study defines TGCT extension on MR imaging to classify severity. METHODS: In part one, six MR parameters were defined by field-experts to assess disease extension on MR images: type of TGCT, articular involvement, cartilage-covered bone invasion, and involvement of muscular/tendinous tissue, ligaments or neurovascular structures. Inter- and intra-rater agreement were calculated using 118 TGCT MR scans. In part two, the previously defined MR parameters were evaluated in 174 consecutive, not previously used, MR-scans. TGCT severity classification was established based on highest to lowest Hazard Ratios (HR) on first recurrence. RESULTS: In part one, all MR parameters showed good inter- and intra-rater agreement (Kappa≥0.66). In part two, cartilage-covered bone invasion and neurovascular involvement were rarely appreciated (<13%) and therefore excluded for additional analyses. Univariate analyses for recurrent disease yielded positive associations for type of TGCT HR12.84(95%CI4.60-35.81), articular involvement HR6.00(95%CI2.14-16.80), muscular/tendinous tissue involvement HR3.50(95%CI1.75-7.01) and ligament-involvement HR4.59(95%CI2.23-9.46). With these, a TGCT severity classification was constructed with four distinct severity-stages. Recurrence free survival at 4 years (log rank p < 0.0001) was 94% in mild localized (n56, 1 recurrence), 88% in severe localized (n31, 3 recurrences), 59% in moderate diffuse (n32, 12 recurrences) and 36% in severe diffuse (n55, 33 recurrences). CONCLUSION: The proposed TGCT severity classification informs physicians and patients on disease extent and risk for recurrence after surgical treatment. Definition of the most severe subgroup attributes to a universal identification of eligible patients for systemic therapy or trials for novel agents.


Assuntos
Tumor de Células Gigantes de Bainha Tendinosa/classificação , Tumor de Células Gigantes de Bainha Tendinosa/patologia , Imageamento por Ressonância Magnética/métodos , Índice de Gravidade de Doença , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico
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