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"This book is the first manuscript dedicated entirely to Desmoid Tumors. Written by prominent clinicians, researchers and advocacy group experts, patients and professionals alike will find this to be a comprehensive review. Clinical presentation, imaging guidelines and treatment paradigms are highlighted. Both the sporadic and heredity forms (Familial Adenomatous Polyposis) will be discussed. A thorough discussion on the unique issues in children with DT is included. A portion of the book will address the role of the APC gene, the β-catenin protein and the role of mutations in the genesis of DT. Emerging cutting edge research techniques will be revealed. Also included is a thoughtful discussion on the controversial labelling of desmoid tumors as benign and the consequences of such a designation. The role of advocacy groups in supporting research and in promoting awareness of rare diseases such as DT will be outlined. This book will serve as basis to prepare clinicians, researchers and patients to embark on the quest for a cure for desmoid tumors."

"PURPOSE: Familial adenomatous polyposis (FAP)-associated desmoid tumor (DT) is sometimes life threatening. However, the optimal treatment for DTs has not been established. The aim of this study was to analyze the outcomes of surgical and pharmacological treatments for DT in Japanese FAP patients.METHODS: We retrospectively reviewed the data of 303 patients who underwent colectomy for FAP between 2000 and 2012. We analyzed 41 patients with DTs in which the location was apparent. The selection of treatment for intra-abdominal DTs was also evaluated according to Churchs classification.RESULTS: Surgery was frequently used to treat extraabdominal DTs. Multimodal treatments, including surgery, and the administration of non-steroidal antiinflammatory drugs, hormonal therapy, and chemotherapy were widely used for intra-abdominal DTs. The most effective pharmacological treatment was cytotoxic chemotherapy, which was associated with a response rate of 45.5% and a disease control rate of 72.7%. After a median follow-up period of 53.0 months, the 5-year DT-specific survival rate in patients with stage IV disease was 71.4%; in contrast, the rate in patients with other stages was 100%. Four-stage IV patients died of DT due to uncontrollable rapid progression. No cytotoxic chemotherapy was administered; however, incomplete resection was performed in three cases.CONCLUSION: Our findings will provide clues that may help physicians in selecting the optimal strategy for this rare disease."

"This is the first book to be devoted exclusively to rare tumors in children and adolescents, and its aim is to provide up-to-date information on their diagnosis and clinical management. The opening section addresses general issues including epidemiology, risk factors/etiology, biology and genetics, early detection, and screening. It also discusses solutions to assist in the management of rare tumors, such as international networking and internet platforms. In the second section, specific malignancies are described, with practical guidance on diagnostic workup, multimodal therapy, follow-up, and adverse effects. Discussion of differential diagnosis encompasses both frequent and rare tumor types, which should enable the clinician to take rare entities into account during the diagnostic assessment. Each chapter goes on to provide detailed therapeutic guidelines for specific rare tumors. The authors are a multidisciplinary group of specialists who have dedicated themselves to this group of tumors."

"This volume mainly contains information on the diagnosis, therapy, and prognosis of brain tumors. Insights on the understanding of molecular pathways involved in tumor biology are explained, which should lead to the development of effective drugs. Information on pathways (e.g., hedgehog) facilitates targeted therapies in cancer. Tumor models are also presented, which utilize expression data, pathway sensitivity, and genetic abnormalities, representing targets in cancer. For example, rat model of malignant brain tumors using implantation of doxorubicin with drug eluting beads for delivery is explained. The future of pathway-driven therapies for tumors is summarized. The importance of personalizing cancer care is emphasized. The need for supportive measures for survivors of brain cancer is pointed out, so is the quality of life monitoring. The need of rehabilitation therapy for patients with primary and metastatic brain tumors is also emphasized. Role of MicroRNA in distinguishing primary tumors from metastatic primary tumors is discussed. Advantages and limitations of chemotherapy (e.g., temozolomide and doxorubicin) are discussed. The complexity of tumor to tumor transfer is explained; examples discussed are: brain metastases from breast cancer and brain metastases fro non-small cell lung carcinoma. Identification and characterization of biomarkers, including those for metastatic brain tumors, are presented. Genomic analysis for identifying clinically relevant subtypes of glioblastoma is included. A large number of imaging modalities are detailed to study progression and invasion of gliomas."

"Various aspects, including diagnosis, therapy, and prognosis, of two brain tumors (meningioma and schwannoma) , of brain tumors are discussed in this volume. Insights on the understanding of molecular pathways involved in brain tumor biology are explained. For example, the role of E-cadherin gene instability, carbonic anhydrase 11, urokinase plasminogen activator, and Wnt signaling is discussed in detail. Such information will lead to the development of effective aniicancer drugs. The role of molecular genetics and epigenetic mechanisms in schwannomas is explained. Also, is explained the role of cyclin D1 in vestibular schwannoma. The determination of subtypes of meningiomas using perfusion magnetic resonance imaging is explained. Diagnosis of incidentally discovered meningioma and cystic papillary meningioma is also included. Diagnosis of facial nerve schwannoma, vestibular schwannoma, and intermediate nerve schwannoma is explained. Treatments for atypical meningioma, oncocytic meneingioma, intracranial meningioma, and cavernous are presented. Therapeutic methods such as neurosurgery, Gamma knife radiosurgery, and adjuvant radiation for this cancer are included. Large number of other treatments, including radiosurgery, retrosigmoidal craniotomy, and immunotherapy, for vestibular schwannoma patients are detailed."

"The work describes a new method of sphincter salvage in surgery of rectal cancer. Low tumors of the rectum are traditionally treated with amputation of the rectum. However, this operation is not well-received by patients, since it results in a permanent colostomy. By contrast, intersphincteric resection allows sphincter salvage even in low tumors and is now widely accepted among experts in the field of colorectal surgery. The book will describe the basics (pathology,physiology, radiology) as well as the surgical technique and its different modifications."

"The most recent developments in diagnostic and therapeutic aspects of gliomas (glioblastoma) in the brain are presented. The importance of personalized medicine and clinical validation for targeted therapy are discussed. The identification of various types of biomarkers (determined by molecular genetics) is included, along with their advantages and limitations as markers in tumor detection and diagnosis. The identification and validation of brain cancer (glioblastoma) genes are discussed. The role of cancer stem cells in the initiation and persistence of malignant gliomas is explained, response of glioblastoma cancer stem cells to various growth factors, such as epidermal growth factor receptor kinase inhibitor, is explained. The use of surgical resection, chemotherapy (e.g., temozolomide), immunotherapy, and radiation therapy for glioblastoma patients is included. Biological impediments for chemotherapy and radiotherapy for malignant glioblastoma are pointed out. Standard (established) as well as newer imaging modalities (proton magnetic resonance spectroscopy) are discussed. Also included are proton magnetic resonance spectroscopy in intracranial gliomas, and the use of proton magnetic spectroscopic imaging in determining the infiltration zone in gliomas. The role of molecular signaling in the CNS cancer development is explained, including cell death signaling in glioblastoma multiforme."