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1.
Zhong Nan Da Xue Xue Bao Yi Xue Ban ; 44(6): 720-724, 2019 Jun 28.
Artigo em Chinês | MEDLINE | ID: mdl-31304936

RESUMO

Chondrosarcoma original from the zygomatic arch is a very rare disease with high malignancy. Surgery is the main means of treatment at present for duo to its poor sensitivity to radiochemotherapy. We reported a young patient who was recovery well in a 4-years follow-up without radiochemotherapy after a total resection of the tumor.


Assuntos
Neoplasias Ósseas , Condrossarcoma , Quimiorradioterapia , Humanos , Autoimagem , Zigoma
2.
Pan Afr Med J ; 32: 143, 2019.
Artigo em Francês | MEDLINE | ID: mdl-31303915

RESUMO

Chondrosarcoma is a rare malignant bone tumor. It can arise de novo or secondary to a malignant transformation of a benign underlying cartilage tumor. Secondary chondrosarcoma arising from solitary benign osteochondroma is extremely rare and data show that the reported incidence of osteochondroma of the pelvis is very low. We here report the case of a 20-year old patient with chondrosarcoma secondary to malignant transformation of an osteochondroma of the right wing of ilium, adjacent to the sacroiliac joint.


Assuntos
Neoplasias Ósseas/diagnóstico , Condrossarcoma/diagnóstico , Osteocondroma/diagnóstico , Neoplasias Ósseas/patologia , Condrossarcoma/patologia , Humanos , Ílio/patologia , Masculino , Osteocondroma/patologia , Adulto Jovem
3.
Cytogenet Genome Res ; 158(1): 17-24, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31261155

RESUMO

Osteoarthritis (OA) is a degenerative disease characterized by progressive articular cartilage destruction and joint marginal osteophyte formation with different degrees of synovitis. Docosahexaenoic acid (DHA) is an unsaturated fatty acid with anti-inflammatory, antioxidant, and antiapoptotic functions. In this study, the human chondrosarcoma cell line SW1353 was cultured in vitro, and an OA cell model was constructed with inflammatory factor IL-1ß stimulation. After cells were treated with DHA, cell apoptosis was measured. Western blot assay was used to detect protein expression of apoptosis-related factors (Bax, Bcl-2, and cleaved caspase-3) and mitogen-activated protein kinase (MAPK) signaling pathway family members, including extracellular signal-regulated kinase (ERK), c-JUN N-terminal kinase (JNK), and p38 MAPK. Our results show that IL-1ß promotes the apoptosis of SW1353 cells, increases the expression of Bax and cleaved caspase-3, and activates the MAPK signaling pathway. In contrast, DHA inhibits the expression of IL-1ß, inhibits IL-1ß-induced cell apoptosis, and has a certain inhibitory effect on the activation of the MAPK signaling pathway. When the MAPK signaling pathway is inhibited by its inhibitors, the effects of DHA on SW1353 cells are weakened. Thus, DHA enhances the apoptosis of SW1353 cells through the MAPK signaling pathway.


Assuntos
Apoptose/efeitos dos fármacos , Neoplasias Ósseas/patologia , Condrossarcoma/patologia , Ácidos Docosa-Hexaenoicos/farmacologia , Interleucina-1beta/antagonistas & inibidores , Sistema de Sinalização das MAP Quinases/efeitos dos fármacos , Osteoartrite/tratamento farmacológico , Proteínas Reguladoras de Apoptose/biossíntese , Proteínas Reguladoras de Apoptose/genética , Butadienos/farmacologia , Linhagem Celular Tumoral , Avaliação Pré-Clínica de Medicamentos , Ativação Enzimática/efeitos dos fármacos , Indução Enzimática/efeitos dos fármacos , Regulação Neoplásica da Expressão Gênica/efeitos dos fármacos , Humanos , Interleucina-1beta/biossíntese , Interleucina-1beta/genética , Interleucina-1beta/farmacologia , Sistema de Sinalização das MAP Quinases/fisiologia , Proteínas Quinases Ativadas por Mitógeno/biossíntese , Proteínas Quinases Ativadas por Mitógeno/genética , Proteínas de Neoplasias/biossíntese , Proteínas de Neoplasias/genética , Nitrilos/farmacologia , Inibidores de Proteínas Quinases/farmacologia
4.
Bone Joint J ; 101-B(6): 739-744, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31154835

RESUMO

AIMS: The aim of this study was to identify factors that determine outcomes of treatment for patients with chondroblastic osteosarcomas (COS) of the limbs and pelvis. PATIENTS AND METHODS: The authors carried out a retrospective review of prospectively collected data from 256 patients diagnosed between 1979 and 2015. Of the 256 patients diagnosed with COS of the pelvis and the limbs, 147 patients (57%) were male and 109 patients (43%) were female. The mean age at presentation was 20 years (0 to 90). RESULTS: In all, 82% of the patients had a poor response to chemotherapy, which was associated with the presence of a predominantly chondroblastic component (more than 50% of tumour volume). The incidence of local recurrence was 15%. Synchronous or metachronous metastasis was diagnosed in 60% of patients. Overall survival was 51% and 42% after five and ten years, respectively. Limb localization and wide surgical margins were associated with a lower risk of local recurrence after multivariable analysis, while the response to chemotherapy was not. Local recurrence, advanced patient age, pelvic tumours, and large volume negatively influenced survival. Resection of pulmonary metastases was associated with a survival benefit in the limited number of patients in whom this was undertaken. CONCLUSION: COS demonstrates a poor response to chemotherapy and a high incidence of metastases. Wide resection is associated with improved local control and overall survival, while excision of pulmonary metastases is associated with improved survival in selected patients. Cite this article: Bone Joint J 2019;101-B:739-744.


Assuntos
Neoplasias Ósseas/cirurgia , Condrossarcoma/cirurgia , Extremidades/cirurgia , Osteossarcoma/cirurgia , Neoplasias Pélvicas/cirurgia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Neoplasias Ósseas/patologia , Criança , Pré-Escolar , Condrossarcoma/patologia , Terapia Combinada , Extremidades/patologia , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/patologia , Osteossarcoma/patologia , Neoplasias Pélvicas/patologia , Estudos Retrospectivos , Resultado do Tratamento
5.
J Biol Regul Homeost Agents ; 33(2 Suppl. 1): 39-43, 2019 Mar-Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31169001

RESUMO

With the advent of the molecularly targeted therapies, identifying molecular therapeutic targets and molecolar marker is increasingly important, especially in neoplastic diseases. Several studies show VEGF is involved in neo-angiogenesis in many solid cancers, as breast, lung, renal, gastric carcinomas, through promoting endothelial cell growth and migration. Conversely the relationship between VEFG and tumours of the musculoskeletal system is yet unclear, in particular the role of VEGF has not yet been completely understood in these tumours. Chondrosarcoma, Ewing's Sarcoma and Osteosarcoma are the tumours of the musculoskeletal system in which the activity of VEGF has been closely studied. The present study aims to give an overview focused on the relationship between VEGF and these three cancers.


Assuntos
Neoplasias Ósseas/diagnóstico , Condrossarcoma/diagnóstico , Osteossarcoma/diagnóstico , Sarcoma de Ewing/diagnóstico , Fator A de Crescimento do Endotélio Vascular/sangue , Biomarcadores Tumorais/sangue , Humanos , Prognóstico
7.
J Surg Oncol ; 120(2): 200-205, 2019 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-31111502

RESUMO

BACKGROUND/OBJECTIVES: Proton therapy (PRT) has emerged as a treatment option for chordomas/chondrosarcomas to escalate radiation dose more safely. We report results of a phase I/II trial of PRT in patients with chordoma/chondrosarcoma. METHODS: Twenty adult patients with pathologically confirmed, nonmetastatic chordoma or chondrosarcoma were enrolled in a single-institution prospective trial of PRT from 2010 to 2014. Seventeen patients received adjuvant PRT and three received definitive PRT. Median dose was 73.8 Gy(RBE; range 68.4-79.2 Gy) using PRT-only (n = 6) or combination PRT/intensity-modulated radiotherapy (IMRT) (n = 14). Quality-of-life (QOL) and fatigue were assessed weekly and every 3 months posttreatment with the Functional Assessment of Cancer Therapy - Brain (FACTBr) and Brief Fatigue Inventory. Primary endpoint was feasibility (90% completing treatment with < 10 day treatment delay and ≤ 20% unexpected acute grade ≥ 3 toxicity). RESULTS: Tumors included chordomas of the skull base (n = 10), sacrum (n = 5), and cervical spine (n = 3), and skull base chondrosarcomas (n = 2). Median age was 57. The 80% had positive margins/gross disease. Median follow-up was 37 months. Feasibility endpoints were met. The 3-year local control and progression-free survival was 86% and 81%. There were no deaths. Two patients had acute grade 3 toxicity (both fatigue). One had late grade 3 toxicity (epistaxis and osteoradionecrosis). There were no significant differences in patient reported fatigue or QOL from baseline to the end-of-treatment. CONCLUSIONS: We report favorable local control, survival, and toxicity following PRT.


Assuntos
Vértebras Cervicais , Condrossarcoma/radioterapia , Cordoma/radioterapia , Terapia com Prótons , Neoplasias da Base do Crânio/radioterapia , Neoplasias da Coluna Vertebral/radioterapia , Adulto , Idoso , Idoso de 80 Anos ou mais , Intervalo Livre de Doença , Estudos de Viabilidade , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Dosagem Radioterapêutica
8.
Medicine (Baltimore) ; 98(17): e15388, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-31027132

RESUMO

RATIONALE: Chondrosarcoma is a malignant mesenchymal tumor originating from cartilage. The pelvis, ribs, femur, and humerus are the most frequently affected sites, and scapula involvement is relatively rare. The aim of the present study was to report a case of chondrosarcoma in the scapula. PATIENT CONCERNS: A 42-year-old woman presented with a 3-month history of a painful mass in the right scapula. DIAGNOSES AND INTERVENTION: The patient underwent tumor resection. The post-operative pathological diagnosis was scapula chondrosarcoma. OUTCOMES: Following resection, the patient continued to receive routine follow-up care. There was no recurrence or tumor metastasis at a follow-up of 5 years. CONCLUSIONS: Surgery remains the primary therapy for chondrosarcoma. One of the greatest challenges in the management of chondrosarcoma is to accurately assess tumor grade before surgical intervention. Chemotherapy and radiotherapy have been applied without success. Chemo- and radioresistance have been examined beyond classic phenotypic properties to identify more efficient therapeutic strategies. Therefore, development of future novel therapies is contingent upon elucidating the molecular mechanisms of chondrosarcoma.


Assuntos
Neoplasias Ósseas/diagnóstico , Condrossarcoma/diagnóstico , Escápula , Adulto , Neoplasias Ósseas/patologia , Neoplasias Ósseas/cirurgia , Condrossarcoma/patologia , Condrossarcoma/cirurgia , Diagnóstico Diferencial , Feminino , Humanos , Escápula/patologia , Escápula/cirurgia
9.
Medicine (Baltimore) ; 98(15): e15207, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30985717

RESUMO

RATIONALE: Extraskeletal myxoid chondrosarcoma is a slow-growing soft tissue tumor of adults with a propensity for local recurrence and eventual metastasis. Only 17 pediatric and adolescent cases have been reported. PATIENT CONCERNS: Here we present an 11-year-old boy with a 3-year history of a slowly growing painless left leg mass. Magnetic resonance imaging of the lesion revealed a subfascial well-circumscribed lesion with intramuscular extension in the medial gastrocnemius muscle of the left leg. DIAGNOSES: He underwent wide local excision of the mass and the histomorphological and immunohistochemical findings were consistent with extraskeletal myxoid chondrosarcoma. INTERVENTIONS: Possible radiotherapy was the further management plan. OUTCOMES: He was in good condition with no evidence of recurrence at 6 months postsurgery. LESSONS: Although pediatric cases of extraskeletal myxoid chondrosarcoma were reported to be aggressive, the tumor in this case demonstrated indolent behavior. Furthermore, the tumor in this case showed primitive round cell foci which adds to a previous study that especially reported this morphology in pediatric cases.


Assuntos
Condrossarcoma/diagnóstico , Condrossarcoma/cirurgia , Neoplasias de Tecido Conjuntivo e de Tecidos Moles/diagnóstico , Neoplasias de Tecido Conjuntivo e de Tecidos Moles/cirurgia , Criança , Condrossarcoma/patologia , Diagnóstico Diferencial , Humanos , Perna (Membro) , Masculino , Neoplasias de Tecido Conjuntivo e de Tecidos Moles/patologia
10.
Anticancer Res ; 39(4): 1761-1765, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30952715

RESUMO

BACKGROUND/AIM: Primary bone neoplasms include osteosarcomas (OS), chondrosarcomas (CS), and giant cell tumors (GCT). Nicotinamide phosphoribosyl transferase (NAMPT) catalyzes the rate-limiting step of nicotinamide adenine dinucleotide synthesis and is increased in multiple tumor types. In malignancies, NAMPT expression often correlates positively with tumor grade, chemotherapy resistance, and metastatic potential. MATERIALS AND METHODS: Tissue microarray was used to examine NAMPT expression in benign bone and cartilage, GCTs, OS, and different CS grades. RESULTS: For the first time, we showed that NAMPT expression was increased in GCTs and OS compared to benign bone, and in CS compared to benign cartilage. Its expression also increased with higher CS grade. CONCLUSION: Our data indicate that NAMPT plays a role in bone sarcomas and GCTs, and its higher expression may contribute to increased tumor aggressiveness.


Assuntos
Biomarcadores Tumorais/análise , Neoplasias Ósseas/enzimologia , Osso e Ossos/enzimologia , Cartilagem/enzimologia , Condrossarcoma/enzimologia , Citocinas/análise , Nicotinamida Fosforribosiltransferase/análise , Osteossarcoma/enzimologia , Neoplasias Ósseas/patologia , Osso e Ossos/patologia , Cartilagem/patologia , Condrossarcoma/patologia , Tumor de Células Gigantes do Osso/enzimologia , Tumor de Células Gigantes do Osso/patologia , Humanos , Imuno-Histoquímica , Gradação de Tumores , Osteossarcoma/patologia , Análise Serial de Tecidos , Regulação para Cima
11.
Eklem Hastalik Cerrahisi ; 30(1): 24-31, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30885105

RESUMO

OBJECTIVES: This study aims to evaluate the clinical characteristics and treatment outcomes of patients with primary malignant tumors located in the proximal fibula. PATIENTS AND METHODS: This retrospective study included 23 patients (15 males, 8 females; mean age 22.1 years; range, 9 to 63 years) with primary malignant tumors located in the proximal fibula between May 2007 and May 2017. The anamnesis or medical history, physical examination, plain chest radiography, lung computed tomography, direct radiograph, and magnetic resonance imaging of the affected extremity and routine laboratory tests of all patients were evaluated. RESULTS: Of the patients, 11 were diagnosed with osteosarcoma (47.8%), nine with Ewing's sarcoma (39.1%), two with chondrosarcoma (8.7%), and one was diagnosed with synovial sarcoma (4.3%). Pain and palpable mass were the most common symptoms. Six patients had lung metastases at the time of diagnosis. Of the patients, eight were performed Malawer type 1 resection (34.8%), nine type 2 resection (39.1%), four above knee amputation (17.4%), and two proximal tibia tumor resection prosthesis (8.6%). Mean follow-up duration was 36 months (range, 12 to 119 months). Local recurrence developed in three patients. Mean Musculoskeletal Tumor Society (MSTS) score of all patients was 62. CONCLUSION: Surgical treatment of primary malignant tumors of the proximal fibula is problematic. In appropriate indications, Malawer type 1 resection should be the treatment of choice due to lower local recurrence rates and higher MSTS scores.


Assuntos
Neoplasias Ósseas/cirurgia , Condrossarcoma/cirurgia , Neoplasias Pulmonares/secundário , Recidiva Local de Neoplasia , Osteossarcoma/cirurgia , Sarcoma de Ewing/cirurgia , Adolescente , Adulto , Amputação , Neoplasias Ósseas/patologia , Criança , Condrossarcoma/secundário , Feminino , Fíbula , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Osteossarcoma/secundário , Implantação de Prótese , Estudos Retrospectivos , Sarcoma de Ewing/secundário , Resultado do Tratamento , Adulto Jovem
12.
Cochrane Database Syst Rev ; 3: CD010778, 2019 Mar 07.
Artigo em Inglês | MEDLINE | ID: mdl-30845364

RESUMO

BACKGROUND: Grade I or low-grade chondrosarcoma (LGCS) is a primary bone tumour with low malignant potential. Historically, it was treated by wide resection, since accurate pre-operative exclusion of more aggressive cancers can be challenging and under-treatment of a more aggressive cancer could negatively influence oncological outcomes. Intralesional surgery for LGCS has been advocated more often in the literature over the past few years. The potential advantages of less aggressive treatment are better functional outcome and lower complication rates although these need to be weighed against the potential for compromising survival outcomes. OBJECTIVES: To assess the benefits and harms of intralesional treatment by curettage compared to wide resection for central low-grade chondrosarcoma (LGCS) of the long bones. SEARCH METHODS: We searched the Cochrane Central Register of Controlled Trials (CENTRAL; 2018, Issue 4), MEDLINE and Embase up to April 2018. We extended the search to include trials registries, reference lists of relevant articles and review articles. We also searched 'related articles' of included studies suggested by PubMed. SELECTION CRITERIA: In the absence of prospective randomised controlled trials (RCTs), we included retrospective comparative studies and case series that evaluated outcome of treatment of central LGCS of the long bones. The primary outcome was recurrence-free survival after a minimal follow-up of 24 months. Secondary outcomes were upgrading of tumour; functional outcome, as assessed by the Musculoskeletal Tumor Society (MSTS) score; and occurrence of complications. DATA COLLECTION AND ANALYSIS: We used standard methodological procedures recognised by Cochrane. We conducted a systematic literature search using several databases and contacted corresponding authors, appraised the evidence using the ROBINS-I risk of bias tool and GRADE, and performed a meta-analysis. If data extraction was not possible, we included studies in a narrative summary. MAIN RESULTS: We included 18 studies, although we were only able to extract participant data from 14 studies that included a total of 511 participants; 419 participants were managed by intralesional treatment and 92 underwent a wide resection. We were not able to extract participant data from four studies, including 270 participants, and so we included them as a narrative summary only. The evidence was at high risk of performance, detection and reporting bias.Meta-analysis of data from 238 participants across seven studies demonstrated little or no difference in recurrence-free survival after intralesional treatment versus wide resection for central LGCS in the long bones (risk ratio (RR) 0.98; 95% confidence interval (CI) 0.92 to 1.04; very low-certainty evidence). MSTS scores were probably better after intralesional surgery (mean score 93%) versus resection (mean score 78%) with a mean difference of 12.69 (95% CI 2.82 to 22.55; P value < 0.001; 3 studies; 72 participants; low-certainty evidence). Major complications across six studies (203 participants) were lower in cases treated by intralesional treatment (5/125 cases) compared to those treated by wide resection (18/78 cases), with RR 0.23 (95% CI 0.10 to 0.55; low-certainty evidence). In four people (0.5% of total participants) a high-grade (grade 2 or dedifferentiated) tumour was found after a local recurrence. Two participants were treated with second surgery with no evidence of disease at their final follow-up and two participants (0.26% of total participants) died due to disease. Kaplan-Meier analysis of data from 115 individual participants across four studies demonstrated 96% recurrence-free survival after a maximum follow-up of 300 months after resection versus 94% recurrence-free survival after a maximum follow-up of 251 months after intralesional treatment (P value = 0.58; very low-certainty evidence). Local recurrence or metastases were not reported after 41 months in either treatment group. AUTHORS' CONCLUSIONS: Only evidence of low- and very low-certainty was available for this review according to the GRADE system. Included studies were all retrospective in nature and at high risk of selection and attrition bias. Therefore, we could not determine whether wide resection is superior to intralesional treatment in terms of event-free survival and recurrence rates. However, functional outcome and complication rates are probably better after intralesional surgery compared to wide resection, although this is low-certainty evidence, considering the large effect size. Nevertheless, recurrence-free survival was excellent in both groups and a prospective RCT comparing intralesional treatment versus wide resection may be challenging for both practical and ethical reasons. Future research could instead focus on less invasive treatment strategies for these tumours by identifying predictors that help to stratify participants for surgical intervention or close observation.


Assuntos
Neoplasias Ósseas/cirurgia , Condrossarcoma/cirurgia , Curetagem/métodos , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Neoplasias Ósseas/mortalidade , Neoplasias Ósseas/patologia , Condrossarcoma/mortalidade , Condrossarcoma/patologia , Curetagem/efeitos adversos , Intervalo Livre de Doença , Feminino , Humanos , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Gradação de Tumores , Recidiva Local de Neoplasia , Estudos Retrospectivos , Adulto Jovem
13.
Zhonghua Bing Li Xue Za Zhi ; 48(3): 199-203, 2019 Mar 08.
Artigo em Chinês | MEDLINE | ID: mdl-30831645

RESUMO

Objective: To investigate the histological type and clinicopathological characteristics of the craniocerebral slope tumors with chondromucinous features. Methods: Retrospective analysis was conducted to analyze chondromucinous tumors in the slope area diagnosed at Henan Provincial People's Hospital from October 2011 to June 2018. Relevant clinical and pathological data were reviewed, and immunohistochemistry was used to investigate the immunophenotype of the tumors. Results: Eight cases were identified, including 4 males and 4 females with patient age ranging from 20 to 48 years. Histologically, there were 1 case of chordoid meningioma, 1 chondromyxoid fibroma, 1 mucinous chondrosarcoma, 1 Maffucci syndrome, and 4 chondroid chordomas. Conclusion: Chondromucinous tumors of the slope area include chordoma, chordoid meningioma, chondromyxoid fibroma, and myxoid chondrosarcoma and their correct diagnosis is mainly based on the morphological characteristics, immunophenotype and comprehensive analysis of clinical data.


Assuntos
Neoplasias Ósseas , Condrossarcoma/patologia , Cordoma/patologia , Fibroma/patologia , Neoplasias Meníngeas/patologia , Meningioma/patologia , Neoplasias Cranianas/patologia , Adulto , Diagnóstico Diferencial , Encondromatose/patologia , Feminino , Humanos , Imuno-Histoquímica , Imunofenotipagem , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Adulto Jovem
14.
Bone Joint J ; 101-B(3): 266-271, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30813783

RESUMO

AIMS: The purpose of this study was to investigate the potential for achieving local and systemic control after local recurrence of a chondrosarcoma of bone. PATIENTS AND METHODS: A total of 126 patients with local recurrence (LR) of chondrosarcoma (CS) of the pelvis or a limb bone were identified from a prospectively maintained database, between 1990 and 2015 at the Royal Orthopaedic Hospital, Birmingham, United Kingdom. There were 44 female patients (35%) and 82 male patients (65%) with a mean age at the time of LR of 56 years (13 to 96). The 126 patients represented 24.3% of the total number of patients with a primary CS (519) who had been treated during this period. Clinical data collected at the time of primary tumour and LR included the site (appendicular, extremity, or pelvis); primary and LR tumour size (in centimetres); type of operation at the time of primary or LR (limb-salvage or amputation); surgical margin achieved at resection of the primary tumour and the LR; grade of the primary tumour and the LR; gender; age; and oncological outcomes, including local recurrence-free survival and disease-specific survival. A minimum two years' follow-up and complete histopathology records were available for all patients included in the study. RESULTS: For patients without metastases prior to or at the time of local recurrence, the disease-specific survival after local recurrence was 62.5% and 45.5% at one and five years, respectively. After univariable analysis, significant factors predicting disease-specific survival were grade (p < 0.001) and surgical margin (p = 0.044). After multivariable analysis, grade, increasing age at the time of diagnosis of local recurrence, and a greater time interval from primary surgery to local recurrence were significant factors for disease-specific survival. A secondary local recurrence was seen in 26% of patients. Wide margins were a good predictor of local recurrence-free survival for subsequent recurrences after univariable analysis when compared with intralesional margins (p = 0.002) but marginal margins did not reach statistical significance when compared with intralesional margins (p = 0.084). CONCLUSION: In cases of local recurrence of a chondrosarcoma of bone, we have shown that if the tumour is non-metastatic at re-staging, an increase in disease-specific survival and in local recurrence-free survival is achievable, but only by resection of the local recurrence with a wide margin. Cite this article: Bone Joint J 2019;101-B:266-271.


Assuntos
Neoplasias Ósseas/cirurgia , Condrossarcoma/cirurgia , Recidiva Local de Neoplasia/cirurgia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Neoplasias Ósseas/mortalidade , Neoplasias Ósseas/patologia , Condrossarcoma/mortalidade , Condrossarcoma/patologia , Extremidades/patologia , Extremidades/cirurgia , Feminino , Humanos , Masculino , Margens de Excisão , Pessoa de Meia-Idade , Gradação de Tumores , Metástase Neoplásica , Recidiva Local de Neoplasia/mortalidade , Recidiva Local de Neoplasia/patologia , Estadiamento de Neoplasias , Ossos Pélvicos/patologia , Ossos Pélvicos/cirurgia , Prognóstico , Estudos Retrospectivos , Análise de Sobrevida , Adulto Jovem
16.
J Neurooncol ; 142(3): 557-563, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30827010

RESUMO

BACKGROUND: We sought to evaluate the effectiveness of definitive or adjuvant external-beam proton therapy on local control and survival in patients with skull-base chondrosarcoma. METHODS: We reviewed the medical records of 43 patients with a median age of 49 years (range, 23-80 years) treated with double-scattered 3D conformal proton therapy for skull-base chondrosarcomas between January 2007 and February 2016. Proton therapy-related toxicities were scored using CTCAE v4.0. RESULTS: The median radiotherapy dose was 73.8 Gy(RBE) (range, 64.5-74.4 Gy[RBE]). Thirty-six (84%) and 7 (16%) patients underwent surgical resection or biopsy alone. Tumor grade distribution included: grade 1, 19 (44%) patients; grade 2, 22 (51%); and grade 3, 2 (5%). Forty patients had gross disease at the time of radiotherapy and 7 patients were treated for locally recurrent disease following surgery. The median follow-up was 3.7 years (range, 0.7-10.1 years). There were no acute grade 3 toxicities related to RT. At 4 years following RT, actuarial rates of overall survival, cause-specific survival, local control, and RT-related grade 3 toxicity-free survival were 95%, 100%, 89%, and 95%. CONCLUSION: High-dose, double-scattered 3D conformal proton therapy alone or following surgical resection for skull-base chondrosarcoma is an effective treatment with a high rate of local control with no acute grade 3 radiation-related toxicity. Further follow-up of this cohort is necessary to better characterize long-term disease control and late toxicities.


Assuntos
Neoplasias Ósseas/radioterapia , Condrossarcoma/radioterapia , Terapia com Prótons/mortalidade , Neoplasias da Base do Crânio/radioterapia , Adulto , Idoso , Idoso de 80 Anos ou mais , Neoplasias Ósseas/patologia , Condrossarcoma/patologia , Feminino , Seguimentos , Humanos , Masculino , Pessoa de Meia-Idade , Dosagem Radioterapêutica , Neoplasias da Base do Crânio/patologia , Taxa de Sobrevida , Resultado do Tratamento , Adulto Jovem
18.
BMC Musculoskelet Disord ; 20(1): 134, 2019 Mar 28.
Artigo em Inglês | MEDLINE | ID: mdl-30922289

RESUMO

BACKGROUND: Sufficient data on outcome of patients with clinically and radiologically aggressive enchondromas and atypical cartilaginous tumors (ACT) is lacking. We therefore analyzed both conservatively and surgically treated patients with lesions, which were not distinguishable between benign enchondroma and low-grade malignant ACT based upon clinical and radiologic appearance. METHODS: The series included 228 consecutive cases with a follow-up > 24 months to assess radiological, histological, and clinical outcome including recurrences and complications. Pain, satisfaction, functional limitations, and the musculoskeletal tumor society (MSTS) score were evaluated to judge both function and emotional acceptance at final follow-up. RESULTS: Follow-up took place at a mean of 82 (median 75) months. The 228 patients all had comparable clinical and radiological findings. Of these, 153 patients were treated conservatively, while the other 75 patients underwent intralesional curettage. Besides clinical and radiological aggressiveness, most lesions were histologically judged as benign enchondromas. 9 cases were determined to be ACT, while the remaining 7 cases had indeterminate histology. After surgery, three patients developed a recurrence, and a further seven had complications of which six were related to osteosynthesis. Both groups had excellent and almost equal MSTS scores of 96 and 97%, respectively, but significantly less functional limitations were found in the non-surgery group. Further sub-analyses were performed to reduce selection bias. Sub-analysis of histologically diagnosed enchondromas in the surgery group found more pain, less function, and worse MSTS score compared to the non-surgery group. Sub-analysis of smaller lesions (< 4.4 cm) did not show significant differences. In contrast, larger lesions displayed significantly worse results after surgery compared to conservative treatment (enchondromas > 4.4 cm: MSTS score: 94.0% versus 97.3%, p = 0.007; pain 2.3 versus 0.8, p = 0.001). The majority of lesions treated surgically was filled with polymethylmethacrylate bone-cement, while the remainder was filled with cancellous-bone, without significant difference in clinical outcome. CONCLUSION: Feasibility of intralesional curettage strategies for symptomatic benign to low-grade malignant chondrogenic tumors was supported. Surgery, however, did not prove superior compared to conservative clinical and radiological observation. Due to the low risk of transformation into higher-grade tumors and better functional results, more lesions might just be observed if continuous follow-up is assured.


Assuntos
Neoplasias Ósseas/terapia , Condroma/terapia , Condrossarcoma/terapia , Tratamento Conservador/métodos , Curetagem/métodos , Ossos do Braço/diagnóstico por imagem , Ossos do Braço/patologia , Ossos do Braço/cirurgia , Cimentos para Ossos/uso terapêutico , Neoplasias Ósseas/diagnóstico por imagem , Neoplasias Ósseas/epidemiologia , Neoplasias Ósseas/patologia , Condroma/diagnóstico por imagem , Condroma/patologia , Condrossarcoma/epidemiologia , Condrossarcoma/patologia , Tomada de Decisão Clínica , Tratamento Conservador/efeitos adversos , Curetagem/efeitos adversos , Feminino , Seguimentos , Humanos , Ossos da Perna/diagnóstico por imagem , Ossos da Perna/patologia , Ossos da Perna/cirurgia , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/epidemiologia , Recidiva Local de Neoplasia/prevenção & controle , Medição da Dor , Dor Pós-Operatória/diagnóstico , Dor Pós-Operatória/epidemiologia , Dor Pós-Operatória/etiologia , Satisfação do Paciente , Seleção de Pacientes , Polimetil Metacrilato/uso terapêutico , Estudos Retrospectivos , Resultado do Tratamento
19.
Cell Commun Signal ; 17(1): 17, 2019 02 26.
Artigo em Inglês | MEDLINE | ID: mdl-30808351

RESUMO

BACKGROUND: Chondrosarcoma is a malignant cartilaginous neoplasm of the bone which resistant to radiation therapy and chemotherapy. Cyclin-dependent kinase 4 (CKD4) is highly expressed in human cancer, and palbociclib, the inhibitor of CDK4 has been used clinically under FDA approval for application in cancer therapeutic remedies. However, the level of CDK4 and the treatment possibility in chondrosarcoma require further exploration. Thus, we aim to investigate the level of CDK4 and accompanying therapeutic effects of palbociclib in chondrosarcoma. METHODS: We used immunohistochemistric analysis to evaluate human CDK4 productions in chondrosarcoma tissues. The inhibitory expression of CDK4 by siRNA or palbociclib on cell proliferation, invasion, migration, apoptosis and cycle arrest of chondrosarcoma were determined by MTT, wound healing, transwell and flow cytometry. CDK4/Rb signaling pathway were determined by western blot and Immunofluorescence assay. The inhibition effect of palbociclib on tumor growth within the bone were determined by bioluminescence imaging in vivo. RESULTS: CDK4 was found to express significantly in human chondrosarcoma samples. The enhanced levels of CDK4 were interlinked with malignant metastasis and undesirable prognosis of chondrosarcoma patients. CDK4 was also highly expressed in human chondrosarcoma cell lines and its inhibition by specific siRNA and palbociclib lead to a decrease in cell proliferation, accompanied by the phosphorylation of Rb. Furthermore, palbociclib also induced cell cycle arrest in G1 phase and decreased cell migration and invasion via CDK4/Rb signaling pathway. Administration of palbociclib in vivo could reduce tumor burden in chondrosarcoma. CONCLUSIONS: In summary, these data highlight CDK4 inhibitors, such as palbociclib, as potential promising therapeutics in the treatment of human chondrosarcoma.


Assuntos
Condrossarcoma/tratamento farmacológico , Condrossarcoma/enzimologia , Quinase 4 Dependente de Ciclina/antagonistas & inibidores , Terapia de Alvo Molecular , Piperazinas/uso terapêutico , Piridinas/uso terapêutico , Animais , Apoptose/efeitos dos fármacos , Pontos de Checagem do Ciclo Celular/efeitos dos fármacos , Linhagem Celular Tumoral , Movimento Celular/efeitos dos fármacos , Proliferação de Células/efeitos dos fármacos , Condrossarcoma/genética , Condrossarcoma/patologia , Quinase 4 Dependente de Ciclina/metabolismo , Regulação para Baixo/efeitos dos fármacos , Regulação para Baixo/genética , Feminino , Regulação Neoplásica da Expressão Gênica/efeitos dos fármacos , Inativação Gênica/efeitos dos fármacos , Humanos , Masculino , Camundongos Endogâmicos BALB C , Camundongos Nus , Pessoa de Meia-Idade , Metástase Neoplásica , Fosforilação/efeitos dos fármacos , Piperazinas/farmacologia , Prognóstico , Piridinas/farmacologia , Proteína do Retinoblastoma/metabolismo , Carga Tumoral
20.
Cell Physiol Biochem ; 52(1): 1-15, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30790501

RESUMO

BACKGROUND/AIMS: Chondrosarcoma is the second most common primary malignancy of bone, characterized by a high metastatic potential. Increasing clinical data highlight the important role played by lymphangiogenesis in cancer metastasis. Amphiregulin (AR) has been implicated in tumor metastasis and lymphangiogenesis, but its association with vascular endothelial growth factor-C (VEGF-C) expression and lymphangiogenesis in chondrosarcoma is unclear. METHODS: We used qPCR, ELISA and Western blotting to detect AR-induced VEGF-C expression in chondrosarcoma cells. Lymphangiogenesis was investigated by lymphatic endothelial cells (LECs) migration and tube formation. An in vivo experiment examined AR expression in tumor-associated lymphangiogenesis. RESULTS: In this study, we found that both AR and VEGF-C expression correlated with tumor stage and were significantly higher than levels found in normal cartilage. Exogenous AR promoted VEGF-C expression in chondrosarcoma cells in a time- and dose-dependent manner and subsequently increased migration and tube formation of LECs. AR also increased VEGF-C expression and lymphangiogenesis through the Src/MEK/ERK/STAT3 signaling pathway. However, it is unclear as to how an EGFR ligand (AR) induces activation of the Src kinase. Knockdown of AR decreased VEGF-C expression in chondrosarcoma cells. Similarly, lymphangiogenesis was abolished in AR knockdown cells in an in vivo model of chondrosarcoma. CONCLUSION: These results indicate that AR occurs through the Src/MEK/ERK/STAT-3 pathway, activating VEGF-C expression and contributing to lymphangiogenesis in human chondrosarcoma. Thus, AR could be a therapeutic target in metastasis and lymphangiogenesis of chondrosarcoma.


Assuntos
Anfirregulina/metabolismo , Neoplasias Ósseas/metabolismo , Condrossarcoma/metabolismo , Regulação Neoplásica da Expressão Gênica , Linfangiogênese , Proteínas de Neoplasias/metabolismo , Fator de Transcrição STAT3/metabolismo , Fator C de Crescimento do Endotélio Vascular/biossíntese , Neoplasias Ósseas/patologia , Linhagem Celular Tumoral , Condrossarcoma/patologia , Humanos
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