1,721,019 research outputs found
Diffuse-type tenosynovial giant cell tumour: Current treatment concepts and future perspectives
No full textAt present, the optimal treatment strategy in patients with diffuse-type tenosynovial giant cell tumour (D-TGCT) is unclear. The purpose of this review was to describe current treatment options, and to highlight recent developments in the knowledge of the molecular pathogenesis of D-TGCT as well as related therapeutic implications. Epidemiology, clinical features, and the pathogenesis of D-TGCT and the most widely used treatment modalities are described. D-TGCT is a benign clonal neoplastic proliferation arising from the synovium. Patients are often symptomatic and require multiple surgical procedures during their lifetime. Currently, surgery is the main treatment for patients with D-TGCT, with relapse rates ranging from 14% to 55%. Radiosynovectomy and external beam radiotherapy have been used in combination with surgical excision or as single modalities. The finding that D-TGCT cells overexpress colony-stimulating factor 1 (CSF1), resulting in recruitment of CSF1 receptor (CSF1R)-bearing macrophages that are polyclonal and make up the bulk of the tumour, has led to clinical trials with CSF1R inhibitors. These inhibitors include small molecules such as imatinib, nilotinib, PLX3397, and the monoclonal antibody RG7155. In conclusion, D-TGCT impairs patients' quality of life significantly. The evidence that the pathogenetic loop of D-TGCT can be inhibited could potentially change the therapeutic armamentarium for this condition. Clinical trials of agents that target D-TGCT are currently ongoing. In the meantime, international registries should be activated in order to provide useful information on this relatively rare tumour
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Diagnosis and prognosis for the Ewing family of tumors
No full textBackground: Ewing's sarcoma was first described by James Ewing in 1921. It is the second most common bone sarcoma seen in children, adolescents and young adults after osteosarcoma and belongs to the group of 'small round blue cell tumors', showing an aggressive natural history. Once almost invariably fatal and treated only with palliative radiation, thanks to a multidisciplinary approach, the probability of survival at 5years is now - 65 - 75% for patients with localized disease. This percentage is no more than 20 - 25% for patients with metastatic disease at presentation. Objective: To review epidemiology, diagnosis and prognosis of Ewing's sarcoma family tumors (EFT) of bone. This entity includes a wide spectrum of tumors, from the less differentiated or classic Ewing's sarcoma to the more differentiated peripheral neuroectodermal tumor. A translocation involving the EWS gene on the chromosome 22 band q12 is characteristic of EFTs. Conclusion: A complete clinical and radiological assessment is essential for initial patient evaluation. Owing to the rarity of this entity, whenever an EFT is suspected patients should be referred to a bone tumor treatment center before a diagnostic biopsy is performed. The molecular characterization of chromosomal translocations has an important role in the diagnosis of EFT. © 2009 Informa UK Ltd. All rights reserved
Advances in treatment for tenosynovial giant cell tumors
No full textIntroduction: Tenosynovial giant cell tumor (TGCT) is a benign clonal neoplastic proliferation arising from the synovium often causing pain, swelling, joint stiffness, and reduced quality of life. The optimal treatment strategy in patients with diffuse-type TGCT (dt-TGCT) is unclear. The purpose of this review is to describe recent advances in the knowledge of TGCT pathogenesis and potential therapeutic implications. Areas covered: Current treatment options for TGCT are discussed, including surgery and radiotherapy. Recent evidence that TGCT cells overexpress colony-stimulating factor 1 (CSF1), resulting in recruitment of CSF1 receptor (CSF1R) positive tumor-associated macrophage and contributing to tumor growth, has created new opportunities for systemic treatments of dt-TGCT. Results of clinical trials with CSF1R inhibitors are now available. These inhibitors include small molecules such as imatinib, nilotinib, pexidartinib, and monoclonal antibodies like emactuzumab, MCS110, and cabiralzumab. Expert opinion: TGCT impairs patients’ quality of life significantly. The confirmation that the pathogenetic loop of TGCT can be inhibited through targeted agents could potentially change the therapeutic armamentarium for this condition
Prognostic significance of immunohistochemical expression of ezrin in non-metastatic high-grade osteosarcoma
No full textBackground. Ezrin is a membrane-cytoskeleton linker protein involved in regulating growth and metastatic behaviour of cancer cells. The study evaluated ezrin expression and its prognostic value in patients with non-metastatic osteosarcoma. Procedures. Ezrin expression and pattern of staining (cytoplasmic or membraneous and cytoplasmic) were assessed using immunohistochemistry on slides from tumour biopsy. We studied 95 patients (median 16 years, range: 4-39 years) with primary non-metastatic osteosarcoma of the extremity treated by neoadjuvant chemotherapy based on methotrexate, cisplatin, doxorubicin and ifosfamide. Results. Seventy-six patients (80%) showed ezrin immunoreactivity: in cytoplasm (37, 47%) and in cytoplasm and membrane (42, 53%) of tumour cells. Immunohistochemical staining score was: 1+ (16, 24%), 2+(10, 13%), 3+ (17, 21.5%) and 4+ (36, 45.5%). Ezrin and score expression were not related to gender, site, alkaline phosphatase (AP), LDH serum levels, chemotherapy-induced tumour necrosis or patient outcome. A significant association was seen between expression pattern and prognosis. The 3-year probability of disease-free survival was 80% for patients with only cytoplasmic immunostaining and 54% for patients with cytoplasmic and membranous immunostaining (P 80% at 5 years can be expected. These results suggest further investigations to define the relation between expression pattern, ezrin functional status and outcome in patients with non-metastatic osteosarcoma. © 2007 Wiley-Liss, Inc
Non-surgical treatment of chondrosarcoma: Current concepts and future perspectives
No full textChondrosarcoma represents a heterogeneous group of malignant cartilage tumors. Surgery is the most important component of treatment. Although convincing evidence of its benefit is lacking, chemotherapy might be useful in the treatment of mesenchymal and dedifferentiated chondrosarcoma. Radiotherapy plays an important role in the treatment of chondrosarcomas located in the spheno-occipital region and cervical spine. New modern radiation techniques could further improve the outcome for patients with lesions in sites that are surgically difficult to access. Finally, better understanding of molecular diagnostics and pathogenesis of chondrosarcoma, together with the development of new therapeutic agents, has resulted in numerous studies investigating potentially active treatment approaches to this disease. However, at present, the majority of these new compounds are being tested in preclinical settings, and their future clinical application still needs to be defined. © 2010 Elsevier Inc. All rights reserved
Going Beyond Counting First Authors in Author Co-citation Analysis
Full text linkThe present study examines one of the fundamental aspects of author co-citation analysis (ACA) - the way co-citation
counts are defined. Co-citation counting provides the data on which all subsequent statistical analyses and mappings
are based, and we compare ACA results based on two different types of co-citation counting - the traditional type that
only counts the first one among a cited work's authors on the one hand and a non-traditional type that takes into
account the first 5 authors of a cited work on the other hand. Results indicate that the picture produced through this non-traditional author co-citation counting contains more coherent author groups and is therefore considerably clearer. However, this picture represents fewer specialties in the research field being studied than that produced through the traditional first-author co-citation counting when the same number of top-ranked authors is selected and analyzed. Reasons for these effects are discussed
Osteosarcoma of the pelvis: a monoinstitutional experience in patients younger than 41 years
Full text linkSupported by: Istituto Ortopedico Riz-zoli research funds. All authors have contributed signifi-cantly to the manuscript and declare that the work is original and has not been submitted or published else-where. None of the authors have any financial disclosure or conflict of inter-est
Variations on the Author
Full text link“Variations on the Author” discusses two of Eduardo Coutinho’s recent films (Um Dia na Vida, from 2010, and Últimas Conversas, posthumously released in 2015) and their contribution to the general question of documentary authorship. The director’s filmography is characterized by a consistent yet self-effacing form of authorial self-inscription: Coutinho often features as an interviewer that rather than express opinions propels discourses; an interviewer that is good at listening. This mode of self-inscription characterizes him as an author who is not expressive but who is nonetheless markedly present on the screen. In Um Dia na Vida, however, Coutinho is completely absent form the image, while Últimas Conversas, on the contrary, includes a confessional prologue that moves the director from the margins to the center of his films. This article examines the ways in which these works stand out in the filmography of a director who offers new insights into the notion of cinematic authorship
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