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1.
Int J Surg Pathol ; : 10668969241246473, 2024 May 05.
Artigo em Inglês | MEDLINE | ID: mdl-38706146

RESUMO

Periosteal Ewing sarcoma (ES) is an exceedingly rare topographic subtype of the ES. To our knowledge, only 60 patients have been reported in the medical English language literature. It predominantly affects men in the second decade of life and arises in the long tubular bone diaphysis. PES rarely develops distant metastases. We report two patients of this rare ES location that were found on the distal tibial shaft and proximal femoral diaphysis of a 21-year-old man and an 8-year-old boy, respectively. Both patients were treated with neoadjuvant chemotherapy, wide resection, and adjuvant chemotherapy. One of our patients had lung metastases at the time of diagnosis and died 5 years later. The other patient presented intramedullary humeral bone metastasis 19 years after diagnosis. There has been no evidence of disease in the 26 years of follow-up. Close follow-up of periosteal ES is recommended because distant metastases may exceptionally occur, even several years after diagnosis.

2.
Biomed Pharmacother ; 173: 116397, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38479181

RESUMO

Angiosarcoma is a rare soft tissue sarcoma originating from endothelial cells. Given that current treatments for advanced disease have shown limited efficacy, alternative therapies need to be identified. In rare diseases, patient-derived cell models are crucial for screening anti-tumour activity. In this study, cell line models were characterised in 2D and 3D cultures. The cell lines' growth, migration and invasion capabilities were explored, confirming them as useful tools for preclinical angiosarcoma studies. By screening a drug library, we identified potentially effective compounds: 8-amino adenosine impacted cell growth and inhibited migration and invasion at considerably low concentrations as a single agent. No synergistic effect was detected when combining with paclitaxel, gemcitabine or doxorubicin. These results suggest that this compound could be a potentially useful drug in the treatment of AGS.


Assuntos
Hemangiossarcoma , Sarcoma , Humanos , Hemangiossarcoma/tratamento farmacológico , Células Endoteliais/patologia , Doxorrubicina/farmacologia , Doxorrubicina/uso terapêutico , Sarcoma/tratamento farmacológico , Paclitaxel/farmacologia , Paclitaxel/uso terapêutico
3.
Insights Imaging ; 15(1): 1, 2024 Jan 07.
Artigo em Inglês | MEDLINE | ID: mdl-38185710

RESUMO

BACKGROUND: Desmoid tumours (DTs) or deep fibromatosis are benign soft-tissue tumours, sometimes locally aggressive, requiring intervention on some cases. Surgery has been the gold standard, but new less invasive techniques such as percutaneous cryoablation have proved their effectiveness, reducing health resources and complications. The study aimed to compare the total cost of percutaneous cryoablation and conventional surgery for patients with extra-abdominal and/or abdominal wall DTs, candidates for local ablative treatment in Spain. METHODS: A cost-analysis model was developed. An expert panel provided data about resource consumption for the percutaneous cryoablation technique and validated the epidemiology used for target population estimation. Unitary resources cost (€ 2022) derived from local cost databases. A retrospective analysis of 54 surgical cases in 3 Spanish hospitals was performed to estimate the cost of conventional surgery based on the cost of the Diagnosis-Related group (DRG) codes identified on this patient sample, weighted by each DRG proportion. The total cost for each alternative included intervention cost and complications cost, considering debridement required in 4.5% of cases with percutaneous cryoablation and minor surgery for surgical site infection in 18.0% for conventional surgery. RESULTS: The total cost for percutaneous cryoablation (€ 5774.78/patient-year) was lower than the total cost for conventional surgery (€ 6780.98/patient-year), yielding cost savings up to € 80,002 in 1 year for the entire cohort of 80 patients with DTs eligible for intervention estimated in Spain. One-way sensitivity analyses confirmed the results' robustness. CONCLUSION: Percutaneous cryoablation versus conventional surgery would yield cost savings for the management of DT patients in Spain. CRITICAL RELEVANCE STATEMENT: This manuscript provides insight into the economic impact derived from the savings related to the use of percutaneous cryoablation for desmoid-type tumours from the perspective of the Spanish National Healthcare System, providing useful information for the health decision-making process. KEY POINTS: • Desmoid tumours are locally aggressive and may require local therapy. • Percutaneous cryoablation procedure is less invasive than the conventional surgery. • Cost comparison shows savings associated to percutaneous cryoablation use.

4.
J Cutan Pathol ; 51(3): 214-220, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38084789

RESUMO

Cutaneous fibrous histiocytoma (FH) is considered a benign dermal tumor. The cellular variant is rare and poorly documented. Besides presenting a high risk of local recurrence, it has a low but serious metastatic potential. We present a case of metastatic cellular FH and also review the literature on this tumor, given its unusual metastatic development. A 47-year-old male patient presented with a lesion in the anterior surface of the right thigh, which has been present since adolescence but had grown during last year. Anatomopathological evaluation revealed a cellular FH, and the lesion was completely removed. Six months later, tumor recurrence with multiple compartment muscle involvement and pulmonary metastasis were detected. Both lesions were completely resected and after 3 years of follow-up, the patient is asymptomatic and free of the disease. We conclude that FH should be carefully sampled to detect variants with high local recurrence rates or with some metastatic risk such as the cellular one. We recommend wide surgical resection and a close follow-up including chest x-rays or thorax computed tomography (CT) in all cellular FH cases with local recurrence.


Assuntos
Histiocitoma Fibroso Benigno , Neoplasias Pulmonares , Neoplasias Cutâneas , Masculino , Adolescente , Humanos , Pessoa de Meia-Idade , Histiocitoma Fibroso Benigno/patologia , Neoplasias Cutâneas/patologia , Neoplasias Pulmonares/secundário
5.
J Am Acad Orthop Surg ; 32(3): e134-e145, 2024 Feb 01.
Artigo em Inglês | MEDLINE | ID: mdl-37824083

RESUMO

BACKGROUND: Intramedullary nail fixation is commonly used for prophylactic stabilization of impending and fixation of complete pathological fractures of the long bones. However, metallic artifacts complicate imaging evaluation for bone healing or tumor progression and postoperative radiation planning. Carbon-fiber implants have gained popularity as an alternative, given their radiolucency and superior axial bending. This study evaluates incidences of mechanical and nonmechanical complications. METHODS: Adult patients (age 18 years and older) treated with carbon-fiber nails for impending/complete pathological long bone fractures secondary to metastases from 2013 to 2020 were analyzed for incidences and risk factors of mechanical and nonmechanical complications. Mechanical complications included aseptic screw loosening and structural failures of host bone and carbon-fiber implants. Deep infection and tumor progression were considered nonmechanical. Other complications/adverse events were also reported. RESULTS: A total of 239 patients were included; 47% were male, and 53% were female, with a median age of 68 (IQR, 59 to 75) years. Most common secondary metastases were related to breast cancer (19%), lung cancer (19%), multiple myeloma (18%), and sarcoma (13%). In total, 17 of 30 patients with metastatic sarcoma received palliative intramedullary nail fixation for impending/complete pathological fractures, and 13 of 30 received prophylactic nail stabilization of bone radiated preoperatively to manage juxta-osseous soft-tissue sarcomas, where partial resection of the periosteum or bone was necessary for negative margin resection. 33 (14%) patients had complications. Mechanical failures included 4 (1.7%) structural host bone failures, 7 (2.9%) implant structural failures, and 1 (0.4%) aseptic loosening of distal locking screws. Nonmechanical failures included 8 (3.3%) peri-implant infections and 15 (6.3%) tumor progressions with implant contamination. The 90-day and 1-year mortalities were 28% (61/239) and 53% (53/102), respectively. The literature reported comparable failure and mortality rates with conventional titanium treatment. CONCLUSIONS: Carbon-fiber implants might be an alternative for treating impending and sustained pathological fractures secondary to metastatic bone disease. The seemingly comparable complication profile warrants further cohort studies comparing carbon-fiber and titanium nail complications.


Assuntos
Fixação Intramedular de Fraturas , Fraturas Espontâneas , Sarcoma , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Pinos Ortopédicos , Fibra de Carbono , Fixação Intramedular de Fraturas/métodos , Fraturas Espontâneas/etiologia , Titânio , Resultado do Tratamento
6.
Int J Surg Pathol ; : 10668969231208030, 2023 Nov 06.
Artigo em Inglês | MEDLINE | ID: mdl-37933149

RESUMO

INTRODUCTION: A soft tissue aneurysmal bone cyst is an extremely rare tumor. The objective of the article is to present the clinical, radiological, and histopathological features of a very unusual neoplasm of soft tissues. CASE REPORT: A 13-year-old male patient presented a painful, mobile, and rapidly growing mass on the posteromedial aspect of his left knee. Imaging studies revealed a mass that arose from the medial surface of the distal sartorius muscle, with extension to the subcutaneous fat tissue. It was a well-circumscribed solid tumor with a peripheral rim calcification on plain film, computerized tomography, and ultrasound (zonal phenomenon). On magnetic resonance imaging, a heterogenous mass on T1-weighted images (WI) and T2-WI was seen, with a peripheral hypointense rim in both sequences. An outstanding edema on T2-WI extending to the soft tissue and muscles of the medial compartment of the knee was detected. The mass was resected, and the "tumoral mimickers" histopathological and molecular (next-generation sequencing) diagnoses confirmed a soft tissue aneurysmal bone cyst. A follow-up showed that the patient was free of disease 12 months after surgery. CONCLUSION: Soft tissue aneurysmal bone cyst is a rare tumor. Appropriate clinical and radiological correlation should be performed to differentiate it from other tumor mimickers.

7.
Diagn Cytopathol ; 51(8): E248-E254, 2023 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-37243568

RESUMO

INTRODUCTION: Intranodal palisaded myofibroblastoma (IPM) is an exceedingly rare benign mesenchymal tumor of the lymph nodes. Magnetic resonance imaging (MRI) findings are unspecific, which may present diagnostic challenges to fine-needle aspiration cytology (FNAC). The histological and immunohistochemical features of IPM are unique. CASE REPORT: A previously healthy 40-year-old male patient presented a slow-growing solitary left inguinal mass. FNAC revealed clustered cells within a metachromatic stroma, single spindle cells without atypia, hemosiderin pigment, and siderophages. An MRI showed a central hyperintense septum in fat-suppressed, T2-weighted sequences. The excised lymph node contained central haphazard fascicles of spindle cells with focal nuclear palisading, hemosiderin pigment, extravasated erythrocytes, and hemorrhagic areas. Vimentin and smooth muscle actin were diffusely positive. Amianthoid collagen fibers were not clearly observed. CONCLUSION: IPM is an extremely rare mesenchymal benign intranodal tumor that should be included in the differential diagnosis of spindle cell lesions in the inguinal region.


Assuntos
Hemossiderina , Neoplasias de Tecido Muscular , Masculino , Humanos , Adulto , Linfonodos/patologia , Biópsia por Agulha Fina , Neoplasias de Tecido Muscular/patologia , Citodiagnóstico
8.
Int J Mol Sci ; 24(9)2023 May 05.
Artigo em Inglês | MEDLINE | ID: mdl-37176035

RESUMO

Malignant bone tumors are aggressive tumors, with a high tendency to metastasize, that are observed most frequently in adolescents during rapid growth spurts. Pediatric patients with malignant bone sarcomas, Ewing sarcoma and osteosarcoma, who present with progressive disease have dire survival rates despite aggressive therapy. These therapies can have long-term effects on bone growth, such as decreased bone mineral density and reduced longitudinal growth. New therapeutic approaches are therefore urgently needed for targeting pediatric malignant bone tumors. Harnessing the power of the immune system against cancer has improved the survival rates dramatically in certain cancer types. Natural killer (NK) cells are a heterogeneous group of innate effector cells that possess numerous antitumor effects, such as cytolysis and cytokine production. Pediatric sarcoma cells have been shown to be especially susceptible to NK-cell-mediated killing. NK-cell adoptive therapy confers numerous advantages over T-cell adoptive therapy, including a good safety profile and a lack of major histocompatibility complex restriction. NK-cell immunotherapy has the potential to be a new therapy for pediatric malignant bone tumors. In this manuscript, we review the general characteristics of osteosarcoma and Ewing sarcoma, discuss the long-term effects of sarcoma treatment on bones, and the barriers to effective immunotherapy in bone sarcomas. We then present the laboratory and clinical studies on NK-cell immunotherapy for pediatric malignant bone tumors. We discuss the various donor sources and NK-cell types, the engineering of NK cells and combinatorial treatment approaches that are being studied to overcome the current challenges in adoptive NK-cell therapy, while suggesting approaches for future studies on NK-cell immunotherapy in pediatric bone tumors.


Assuntos
Neoplasias Ósseas , Neoplasias , Osteossarcoma , Sarcoma de Ewing , Sarcoma , Adolescente , Humanos , Criança , Sarcoma de Ewing/terapia , Osteossarcoma/terapia , Neoplasias/terapia , Imunoterapia Adotiva , Neoplasias Ósseas/terapia , Células Matadoras Naturais , Imunoterapia , Terapia Baseada em Transplante de Células e Tecidos
9.
Clin Transl Oncol ; 25(11): 3307-3311, 2023 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-37097530

RESUMO

BACKGROUND: Infantile fibrosarcoma is the most frequent soft tissue sarcoma in newborns or children under one year of age. This tumour often implies high local aggressiveness and surgical morbidity. The large majority of these patients carry the ETV6-NTRK3 oncogenic fusion. Hence, the TRK inhibitor larotrectinib emerged as an efficacious and safe alternative to chemotherapy for NTRK fusion-positive and metastatic or unresectable tumours. However, real-world evidence is still required for updating soft-tissue sarcoma practice guidelines. OBJECTIVE: To report our experience with the use of larotrectinib in pediatric patients. METHODS: Our case series shows the clinical evolution of 8 patients with infantile fibrosarcoma under different treatments. All patients enrolled in this study received informed consent for any treatment. RESULTS: Three patients received larotrectinib in first line. No surgery was needed with larotrectinib, which led to the rapid and safe remission of tumours, even in unusual anatomical locations. No significant adverse effects were observed with larotrectinib. CONCLUSION: Our case series supports that larotrectinib may be a therapeutic option for newborn and infant patients with infantile fibrosarcoma, especially in uncommon locations.


Assuntos
Fibrossarcoma , Sarcoma , Neoplasias de Tecidos Moles , Lactente , Humanos , Criança , Recém-Nascido , Fibrossarcoma/tratamento farmacológico , Fibrossarcoma/genética , Fibrossarcoma/patologia , Sarcoma/tratamento farmacológico , Neoplasias de Tecidos Moles/tratamento farmacológico , Neoplasias de Tecidos Moles/genética , Neoplasias de Tecidos Moles/patologia , Inibidores de Proteínas Quinases/uso terapêutico
10.
Cancer ; 129(1): 60-70, 2023 01 01.
Artigo em Inglês | MEDLINE | ID: mdl-36305090

RESUMO

BACKGROUND: Survival in patients who have Ewing sarcoma is correlated with postchemotherapy response (tumor necrosis). This treatment response has been categorized as the response rate, similar to what has been used in osteosarcoma. There is controversy regarding whether this is appropriate or whether it should be a dichotomy of complete versus incomplete response, given how important a complete response is for in overall survival of patients with Ewing sarcoma. The purpose of this study was to evaluate the impact that the amount of chemotherapy-induced necrosis has on (1) overall survival, (2) local recurrence-free survival, (3) metastasis-free survival, and (4) event-free survival in patients with Ewing sarcoma. METHODS: In total, 427 patients who had Ewing sarcoma or tumors in the Ewing sarcoma family and received treatment with preoperative chemotherapy and surgery at 10 international institutions were included. Multivariate Cox proportional-hazards analyses were used to assess the associations between tumor necrosis and all four outcomes while controlling for clinical factors identified in bivariate analysis, including age, tumor volume, location, surgical margins, metastatic disease at presentation, and preoperative radiotherapy. RESULTS: Patients who had a complete (100%) tumor response to chemotherapy had increased overall survival (hazard ratio [HR], 0.26; 95% CI, 0.14-0.48; p < .01), recurrence-free survival (HR, 0.40; 95% CI, 0.20-0.82; p = .01), metastasis-free survival (HR, 0.27; 95% CI, 0.15-0.46; p ≤ .01), and event-free survival (HR, 0.26; 95% CI, 0.16-0.41; p ≤ .01) compared with patients who had a partial (0%-99%) response. CONCLUSIONS: Complete tumor necrosis should be the index parameter to grade response to treatment as satisfactory in patients with Ewing sarcoma. Any viable tumor in these patients after neoadjuvant treatment should be of oncologic concern. These findings can affect the design of new clinical trials and the risk-stratified application of conventional or novel treatments.


Assuntos
Neoplasias Ósseas , Sarcoma de Ewing , Humanos , Sarcoma de Ewing/tratamento farmacológico , Sarcoma de Ewing/cirurgia , Sarcoma de Ewing/patologia , Terapia Neoadjuvante/efeitos adversos , Neoplasias Ósseas/patologia , Recidiva Local de Neoplasia/tratamento farmacológico , Recidiva Local de Neoplasia/etiologia , Necrose/etiologia , Estudos Retrospectivos
11.
Rev Esp Cir Ortop Traumatol ; 66(5): 389-396, 2022.
Artigo em Inglês, Espanhol | MEDLINE | ID: mdl-36165809

RESUMO

OBJECTIVE: To analyze the incidence and survival of patients with oligometastases (solitary and normal) when they are treated in centers that are experts in multidisciplinary approach to patients with sarcoma. MATERIAL AND METHOD: Retrospective analysis of 414 patients with bone metastases secondary to carcinomas at Hospital Universitario La Paz and Hospital MD Anderson Cancer Center (Madrid) between May 2006 and May 2019. Metastases located in the pelvis and axial skeleton were excluded, analyzing a total of 28 patients who met the criterion for solitary metastases or oligometastases with normal criteria. The study survival estimate was carried out following the Kaplan-Meier statistical method. RESULTS: The survival of the patients following the oligometastases criteria (solitary and normal) was 53%. Breast cancer was the most prevalent and had a survival rate of more than 70%. The average age of the patients was 58 years old. DISCUSSION: Systemic treatments in cancer treatment have managed to improve disease-free survival curves and lead us to redirect on the paradigm for the treatment of oligometastases, stating that treatment should be carried out in the centers that are experts in the treatment of sarcomas. CONCLUSIONS: The choice of surgical treatment for patients with oligometastases in the strict sense (solitary) and normal should be evaluated by multidisciplinary teams according to the prognoses of the patient, anatomical location and histiotype of the neoplasm.

12.
J Clin Med ; 11(17)2022 Aug 24.
Artigo em Inglês | MEDLINE | ID: mdl-36078889

RESUMO

Carbon fiber offers numerous material benefits including reduced wear, high strength-to-weight ratio, a similar elastic modulus to that of bone, and high biocompatibility. Carbon fiber implants are increasingly used in multiple arenas within orthopaedic surgery, including spine, trauma, arthroplasty, and oncology. In the orthopaedic oncologic population, the radiolucency of carbon fiber facilitates post-operative imaging for tumor surveillance or recurrence, the monitoring of bony healing and union, and radiation mapping and delivery.

13.
J Plast Reconstr Aesthet Surg ; 75(9): 3140-3148, 2022 09.
Artigo em Inglês | MEDLINE | ID: mdl-35760707

RESUMO

BACKGROUND: Hip joint reconstruction following intra-articular resection of the femoral head in children is a highly demanding challenge. We aimed to describe the outcomes of hip reconstruction in paediatric patients with a free fibular epiphyso-diaphyseal flap based on both anterior tibial and peroneal vessels within a radius allograft. PATIENTS AND METHODS: Four patients underwent hip reconstruction following this technique between 2013 and 2020 at La Paz University Hospital (Madrid, Spain). The postoperative follow-up period ranged between 12 months and seven years. Two of the patients were diagnosed with Ewing's sarcoma and two with osteosarcoma. The median age at the time of surgery was eight years (six to nine). RESULTS: Three patients remained to be disease-free at the time of this study, and one died shortly after surgery, so he was excluded from the reconstruction analysis of results. No postoperative complications requiring reintervention were recorded. Imaging studies (X-ray and MRI) showed three-dimensional growth of the flap and integration of the allograft. Mean leg length discrepancy was 1.3 cm (0 to 2.3). At the last follow-up visit, all patients were able to ambulate. CONCLUSION: For children following oncological resection of the femoral head, reconstruction with a vascularized epiphyso-diaphyseal fibula flap combined with radius allograft is a safe option. This procedure provides encouraging functional results and avoids the complications of previously published techniques or implants.


Assuntos
Neoplasias Ósseas , Retalhos de Tecido Biológico , Osteossarcoma , Procedimentos de Cirurgia Plástica , Neoplasias Ósseas/cirurgia , Transplante Ósseo/métodos , Criança , Fíbula/transplante , Retalhos de Tecido Biológico/cirurgia , Humanos , Masculino , Osteossarcoma/cirurgia , Osteotomia , Procedimentos de Cirurgia Plástica/métodos , Estudos Retrospectivos , Resultado do Tratamento
14.
Rev Esp Patol ; 55(2): 77-84, 2022.
Artigo em Espanhol | MEDLINE | ID: mdl-35483772

RESUMO

BLACKGROUND: The COVID-19 pandemic has over-burdened the Spanish health service and, as a result, affected the treatment and management of oncological patients. The aim of this study is to make a descriptive analysis of the management of oncological patients and the functioning of the tumour committees in the University Hospital La Paz (Madrid) during the first wave of the pandemic. MATERIALS AND METHODS: A descriptive analysis was made, based on the results of a questionnaire given to all 18 adult tumour committees and 3 paediatric tumour committees in the University Hospital La Paz. Further information was obtained from all the hospital services involved in the diagnosis and treatment of oncological patients. RESULTS: During the first wave of the pandemic, there was a significant decrease in diagnostic tests. For many weeks, the majority of oncological surgical procedures were delayed or referred to other hospitals. Highly beneficial systemic and radiotherapeutic treatments were maintained and preoperative treatment was increased. The diagnosis and treatment of paediatric tumours was unaltered. Tumour committees were affected but each one adjusted in a different way. All the departments involved in the diagnosis and treatment of oncological patients made contingency plans to minimalize the effect on patients. CONCLUSION: This study shows how the management of oncological patients and the functioning of tumour committees was affected during the COVID-19 pandemic.


Assuntos
COVID-19 , Neoplasias , Adulto , Criança , Humanos , Neoplasias/epidemiologia , Neoplasias/terapia , Pandemias , Inquéritos e Questionários , Centros de Atenção Terciária
15.
Eur J Orthop Surg Traumatol ; 32(4): 631-639, 2022 May.
Artigo em Inglês | MEDLINE | ID: mdl-34057623

RESUMO

BACKGROUND: Deep soft tissue sarcomas are frequently in contact with bone. The therapeutic decision of a composite resection strategy may be challenging, which is usually based on clinical and radiological criteria. The aims of the study were to evaluate the overall frequency of bone and periosteal infiltration in these patients in whom composite resection was indicated, and evaluate the role of magnetic resonance imaging and bone scintigraphy in this scenario. METHODS: Forty-nine patients with a composite surgical resection (soft tissue sarcoma and bone), treated at a single institution between 2006 and 2018, were retrospectively included. Presurgical planning of the resection limits was based on clinical and imaging findings (magnetic resonance imaging and bone scintigraphy). Magnetic resonance imaging was performed in all patients (100%) and bone scintigraphy in 41 (83.7% of the cases). According to magnetic resonance imaging results, patients were divided into two groups: Group A, in which the tumor is adjacent to the bone without evidence of infiltration (n = 24, 48,9%), and Group B, patients with evidence of bone involvement by magnetic resonance imaging (n = 25, 51,1%). BS showed a pathological deposit in 28 patients (68.3%). Histological analysis of the resection specimen was preceded to identify bone and periosteal infiltration. For the analysis of the diagnostic validity of imaging tests, histological diagnosis was considered as the gold standard in the evaluation of STS bone infiltration. RESULTS: Histological bone infiltration was identified in 49% of patients and isolated periosteal infiltration in 14.3%. In terms of diagnostic accuracy, magnetic resonance imaging and bone scintigraphy sensitivity values were 92% and 90%, and their specificity values were 91.7% and 52.4%, respectively. CONCLUSIONS: The incidence of bone and periosteal infiltration of soft tissue sarcomas in contact with bone is high. Presurgical bone assessment by MRI has proven to be a sensitive and specific tool in the diagnosis of bone infiltration. Due to its high negative predictive value, BS is a useful test to rule out it. In those cases, in which there is suspicion of bone infiltration not confirmed by MRI, new diagnostic protocols should be established in order to avoid inappropriate resections.


Assuntos
Sarcoma , Neoplasias de Tecidos Moles , Humanos , Imageamento por Ressonância Magnética , Radiografia , Estudos Retrospectivos , Sarcoma/diagnóstico por imagem , Sarcoma/patologia , Sarcoma/cirurgia , Neoplasias de Tecidos Moles/diagnóstico por imagem , Neoplasias de Tecidos Moles/patologia , Neoplasias de Tecidos Moles/cirurgia
16.
Cancer Cell Int ; 21(1): 646, 2021 Dec 04.
Artigo em Inglês | MEDLINE | ID: mdl-34863177

RESUMO

BACKGROUND: Eribulin has shown antitumour activity in some soft tissue sarcomas (STSs), but it has only been approved for advanced liposarcoma (LPS). METHODS: In this study, we evaluated the effect of eribulin on proliferation, migration and invasion capabilities in LPS, leiomyosarcoma (LMS) and fibrosarcoma (FS) models, using both monolayer (2D) and three-dimensional (3D) spheroid cell cultures. Additionally, we explored combinations of eribulin with other drugs commonly used in the treatment of STS with the aim of increasing its antitumour activity. RESULTS: Eribulin showed activity inhibiting proliferation, 2D and 3D migration and invasion in most of the cell line models. Furthermore, we provide data that suggest, for the first time, a synergistic effect with ifosfamide in all models, and with pazopanib in LMS as well as in myxoid and pleomorphic LPS. CONCLUSIONS: Our results support the effect of eribulin on LPS, LMS and FS cell line models. The combination of eribulin with ifosfamide or pazopanib has shown in vitro synergy, which warrants further clinical research.

17.
Surg Oncol ; 38: 101619, 2021 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-34157657

RESUMO

BACKGROUND AND OBJECTIVES: Ewing sarcoma is the second most common bone sarcoma of childhood. Ewing sarcomas of the scapula are rare, with little known about their characteristics and outcomes. In this study, we describe the demographic characteristics, tumor characteristics, and oncologic outcomes of patients with Ewing sarcoma of the scapula. METHODS: This is a retrospective case series of thirty-four patients treated at three urban hospitals between 1993 and 2014 for Ewing sarcomas affecting the scapula. Their demographic data, tumor characteristics, and oncologic outcomes are reported and contrasted with data on Ewing sarcoma described in the literature. RESULTS: Patients in our case series were 59% male. The average age at diagnosis was 16 years. 44% of patients had metastatic disease at presentation. 26% of patients had a tumor size >8 cm in largest dimension at diagnosis. 9 patients in our series had the t (11; 22) translocation present. Patients had a survival rate of 68% at five years. No patients had local recurrence of disease. Compared with findings reported in the literature concerning Ewing sarcoma affecting other locations, patients with Ewing sarcoma of the scapula were slightly older at time of diagnosis, had a lower percentage of tumors with size > 8 cm in largest dimension at presentation, and more commonly had metastatic disease at presentation. Patients in our cohort had a 5-year survival rate of 68%, which is higher than the rate of approximately 55% as reported in the general literature. CONCLUSIONS: In this study, we describe a retrospective case series of thirty-four patients with Ewing sarcomas of the scapula. This is the largest case series to date of Ewing sarcoma affecting this location to our knowledge. These results will contribute to the understanding of the clinical profile and oncologic behavior of Ewing sarcomas affecting the scapula.


Assuntos
Neoplasias Ósseas/mortalidade , Recidiva Local de Neoplasia/mortalidade , Sarcoma de Ewing/mortalidade , Escápula/patologia , Adolescente , Neoplasias Ósseas/secundário , Neoplasias Ósseas/terapia , Terapia Combinada , Feminino , Seguimentos , Humanos , Masculino , Recidiva Local de Neoplasia/patologia , Recidiva Local de Neoplasia/terapia , Prognóstico , Estudos Retrospectivos , Sarcoma de Ewing/patologia , Sarcoma de Ewing/terapia , Taxa de Sobrevida
18.
Oncologist ; 25(10): e1562-e1573, 2020 10.
Artigo em Inglês | MEDLINE | ID: mdl-32888360

RESUMO

BACKGROUND: The COVID-19 outbreak has resulted in collision between patients infected with SARS-CoV-2 and those with cancer on different fronts. Patients with cancer have been impacted by deferral, modification, and even cessation of therapy. Adaptive measures to minimize hospital exposure, following the precautionary principle, have been proposed for cancer care during COVID-19 era. We present here a consensus on prioritizing recommendations across the continuum of sarcoma patient care. MATERIAL AND METHODS: A total of 125 recommendations were proposed in soft-tissue, bone, and visceral sarcoma care. Recommendations were assigned as higher or lower priority if they cannot or can be postponed at least 2-3 months, respectively. The consensus level for each recommendation was classified as "strongly recommended" (SR) if more than 90% of experts agreed, "recommended" (R) if 75%-90% of experts agreed and "no consensus" (NC) if fewer than 75% agreed. Sarcoma experts from 11 countries within the Sarcoma European-Latin American Network (SELNET) consortium participated, including countries in the Americas and Europe. The European Society for Medical Oncology-Magnitude of clinical benefit scale was applied to systemic-treatment recommendations to support prioritization. RESULTS: There were 80 SRs, 35 Rs, and 10 NCs among the 125 recommendations issued and completed by 31 multidisciplinary sarcoma experts. The consensus was higher among the 75 higher-priority recommendations (85%, 12%, and 3% for SR, R, and NC, respectively) than in the 50 lower-priority recommendations (32%, 52%, and 16% for SR, R, and NC, respectively). CONCLUSION: The consensus on 115 of 125 recommendations indicates a high-level of convergence among experts. The SELNET consensus provides a tool for sarcoma multidisciplinary treatment committees during the COVID-19 outbreak. IMPLICATIONS FOR PRACTICE: The Sarcoma European-Latin American Network (SELNET) consensus on sarcoma prioritization care during the COVID-19 era issued 125 pragmatical recommendations distributed as higher or lower priority to protect critical decisions on sarcoma care during the COVID-19 pandemic. A multidisciplinary team from 11 countries reached consensus on 115 recommendations. The consensus was lower among lower-priority recommendations, which shows reticence to postpone actions even in indolent tumors. The European Society for Medical Oncology-Magnitude of Clinical Benefit scale was applied as support for prioritizing systemic treatment. Consensus on 115 of 125 recommendations indicates a high level of convergence among experts. The SELNET consensus provides a practice tool for guidance in the decisions of sarcoma multidisciplinary treatment committees during the COVID-19 outbreak.


Assuntos
COVID-19/epidemiologia , Oncologia/organização & administração , Oncologia/normas , Sarcoma/terapia , COVID-19/prevenção & controle , Consenso , Europa (Continente)/epidemiologia , Humanos , América Latina/epidemiologia , Assistência ao Paciente/normas , Guias de Prática Clínica como Assunto , SARS-CoV-2 , Sarcoma/diagnóstico
19.
J Surg Case Rep ; 2020(4): rjaa095, 2020 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-32346469

RESUMO

Besides national and international recommendations, orthopaedic departments face significant changes in daily activity and serious issues to maintain their standards in musculoskeletal care during the pandemic Covid-19 crisis that we are facing. This report retrospectively addresses measures that were progressively put in place to modify in a week time the activity of a busy orthopaedic department in a large tertiary university hospital in face of the pandemic. Surgical priorities and surgical outcomes are key aspects to consider. The experience may offer some insight to areas where the spread of the disease may be slower or delayed. Abrupt stop of scheduled surgery and clinics is useful to adapt an orthopaedic department to the overall hospital resource reorganization. Orthopaedic surgeons need to be aware of the risks to patients and personnel in view of underdiagnosed cases, which make pre-operative Covid-19 evaluation mandatory for all surgical cases.

20.
Clin Orthop Relat Res ; 477(12): 2718-2725, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31764341

RESUMO

BACKGROUND: Tumor surgeons use a variety of endoprosthetic designs for reconstruction after bone tumor resection. However, functional results and implant survival have not been evaluated for each design. Because the outcomes and failure modes (for example, implant breakage, loosening) may differ between prosthetic design types, it is important to examine the problems associated with different designs. Because of experiences in our practice, we became concerned about a surprisingly high frequency of device breakage with one particular design, and we wished to report on that experience. QUESTION/PURPOSES: (1) In a small series of patients, what proportion of a particular design (Zimmer® Segmental [Zimmer Inc, Warsaw, IN, USA]) of rotating-hinge endoprosthesis experienced implant breakage at short-term follow-up? (2) What patient symptoms were associated with this finding? (3) What is the function as assessed by Musculoskeletal Tumor Society (MSTS) score with the use of this implant before and after revision? METHODS: We treated 87 patients in our tertiary center from 1987 to 2014 who had sarcomas around the knee with wide resection and reconstruction with tumoral endoprosthesis; five patients were lost to follow-up. In all, 33 of the remanining 82 prostheses, treated from 1987 to 2006, were reconstructed with fixed-hinge designs. From 2006 to 2014, 49 patients were reconstructed with a knee endoprosthesis, and 48 of them had a rotating-hinge prosthesis. In our center, we mostly used four designs: 16 of 49 patients were reconstructed with GMRS (Stryker Howmedica, Kalamazoo, MI, USA), seven received the LPS™ (DePuy Synthes, Warsaw, IN, USA), 20 of 49 had the METS (Stanmore, Hertfordshire, UK), and six of 49 received the Zimmer Segmental. The focus of this report is on the six patients with the Segmental. We retrospectively gathered clinical and radiologic data from these six patients' records and we assessed radiographic images. We evaluated function with the MSTS score of the 49 patients. The median follow-up duration of the Segmental prosthesis reconstruction was 65 months (range 24 to 85). RESULTS: Three of the six patients had posterior instability and recurrent joint effusion on physical examination. Three patients who did not have hyperextension presented with restricted knee ROM. Six revision procedures were performed in three patients. The median MSTS score at 6 months for the Segmental® prosthesis was 15 of 30 (range 6 to 24). The score in the three patients who had posterior instability was 9 of 30 (range 6 to 15) and it improved to median 25 of 30 (range 19 to 30) 6 months after revision. The patients with the Segmental® prosthesis who did not undergo revision had a median MSTS score of 20 (range 16 to 24). CONCLUSIONS: The Zimmer Segmental rotating-hinge tumoral prosthesis underwent revision for implant breakage at short term in three of six patients after tumor resection and reconstruction of the knee. Bumper breakage was associated with posterior instability that was related to wear of the bushing blocking system. We are unaware of reports of these issues by other observers or in other prosthetic designs, but we feel larger registries should be created to see if this failure mechanism has been observed by others. If so, this design needs to be improved or the blocking system should be avoided.Level of Evidence Level IV, therapeutic study.


Assuntos
Neoplasias Ósseas/cirurgia , Articulação do Joelho/cirurgia , Prótese do Joelho/efeitos adversos , Osteotomia/efeitos adversos , Procedimentos de Cirurgia Plástica/métodos , Complicações Pós-Operatórias/cirurgia , Sarcoma/cirurgia , Adolescente , Adulto , Neoplasias Ósseas/diagnóstico , Feminino , Seguimentos , Humanos , Articulação do Joelho/diagnóstico por imagem , Masculino , Complicações Pós-Operatórias/diagnóstico , Desenho de Prótese , Falha de Prótese , Radiografia , Reoperação , Estudos Retrospectivos , Sarcoma/diagnóstico , Fatores de Tempo , Adulto Jovem
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