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Why a book dedicated to rare cancer management? Given the large variety of rare cancers,they take up a significant part of our daily practice. Therefore, we owe it to our patients with rare tumours that we use the knowledge gained in the treatment of the more common cancers to provide them with a better future. This knowledge comes from recent developments in molecular diagnostics, systemic treatments with new drugs and targeted therapies together with advanced technologies in the areas of radiology and radiation therapy. Our authors have analysed the literature and recommend treatments for rare cancers based on results from past studies. We hope that this book will be an aid to those treating rare cancer cases about which, up to now, little has been published, and will reassure our patients that they have not been forgotten. Yazid Belkacémi, MD, PhD is Professor Radiation Oncology and Deputy Head of the Department of Radiation Oncology at the Centre Hospitalo-Universitaire Henri Mondor,Créteil,University of Paris XII, France. His clinical research interests include the management of breast and gynaecologic cancers, hematologic malignancies, rare tumour management and the development of new technologies in radiation oncology. His biology research topics include radio-sensitivity markers in tumours and healthy tissues and combinations of targeted therapies and new drugs with ionising radiation. "As « rarity » is too often synonymous with isolation, it is hoped that this book will contribute to increasing and spreading the knowledge about rare tumours in order to better manage our patients with innovative approaches." René-Olivier Mirimanoff, MD is Professor Radiation Oncology and Head of the Department of Radiation Oncology at the Centre Hospitalier Universitaire Vaudois (CHUV) and the University of Lausanne, Switzerland. He is also the founder of the Rare Cancer Network. (www.rarecancer.net). His main research interests are brain tumours and rare cancers. "Rare types of cancer should no longer be considered as orphan diseases.It is time now to gather solid data to guide our medical, radiation and surgical oncology colleagues towards the best possible decisions." Mahmut Ozsahin, MD, PhD is Associate Professor in the Department of Radiation Oncology of the Centre Hospitalier Universitaire Vaudois/University Hospital of Lausanne, Switzerland. His main interests are head and neck cancer, colorectal cancer and haematological malignancies with a special interest in rare tumours in these sites. His biology research topics include radiosensitivity in healthy tissues and predictive assays in radiotherapy. "As no, or very little, prospective clinical research is carried out on rare tumours, it is important to gather and communicate our findings to improve the outcome of our patients." Table of contents Preface: Paul Levine Foreword: Robert Miller SECTION 1. CNS TUMORS 1. Germinal tumours of the central nervous system in adult patients, S. Villà, M. Caro, V. Tuset 2. Atypical and malignant meningiomas, D. Pasquier, N. Rezvoy 3. Primary central nervous system lymphoma , P. Poortmans 4. Traitment of Medulloblastoma in adults, N. Penel, C. Ferte, E. Le Rhun, Y. Belkacémi 5. Cerebellar glioblastoma multiforme, D. Weber 6. Chordoma and chondrosarcoma of the skull base, A. Pica, R.-O. Mirimanoff 7. Primary spinal epidural lymphoma, V. Monnard, R.-O. Mirimanoff SECTION 2. HEAD AND NECK TUMORS 1. Esthesioneuroblastoma , O. Matzinger 2. Extranodal NK/T - cell lymphoma, nasal type, Y. Li 3. Uncommon tumours of the nasopharynx, E. Ozyar, M. Gültekin 4. Uncommon tumours of the oral cavity and oropharynx, A. Sefik Hosal 5. Rare malignant tumours of the larynx and hypopharynx, C. Ikonomodis, L. Bron 6. Primary Orbital Lymphoma, G. Ugurluer, A. Zouhair, M. Ozsahin 7. Primary MALT lymphoma of the salivary glands, Y Anacak, Serra Kamer SECTION 3. GASTRO-INTESTINAL TUMORS 1. Mucosal melanoma of the upper aero-digestive tract, M. Krengli, L Masini, F. Pia 2.Gastrointestinal stromal Tumours, M. Montemurro, J.-Y. Blay, S. Levraz 3.Small cell carcinomas of the gastrointestinal tract, M. Shilkrut, A. Peer, A Kuten 4. Anorectal melanoma, S. Vieillot, D. Azria 5. Primary adenocarcinoma of the anal canal, Y. Belkacémi, P. Rouanet, M. Jafari 6. Anal margin tumours, M Schlienger, E Touboul 7.Uncommon Hepatobiliary Tumours, M. Hübner, N. Halkic, N. Demartines 8.Unusual pancreatic tumours, F Nguyen, A.-N. Dupont-Gossart SECTION 4. GENITOURINARY 1.Rare tumours of the renal pelvis and ureter, M. Ozsahin, A. Zouhair 2.Rare tumours of the urethra, F Oner Dincbas 3. Uncommon bladder cancers, C. Durdux, O. Mir 4. Neuroendocrine small cell carcinoma of the prostate, M. Stein, A. Kuten 5. Uncommon tumours of the testis, I. Alexandre, E. Horn, F. Selle 6. Primary Testicular lymphoma, A Zouhair, RO. Mirimanoff, M. Ozsahin 7. Human chorionic gonadotropin-producing seminoma, RO. Mirimanoff, A. Zouhair SECTION 5. GYNECOLOGICAL 1. Rare ovarian cancers, F. Gligorov, I. Ray-Coquard, P. Pautier, E. Pujade Lauraine, F. Selle, C. Lhommé, J.-P. Lotz, P. Morice, P. Duvillard 2. Extraovarian primary peritoneal carcinoma, C. Nay Fellay, M. Fiche, J.-F. Delaloye, J. Bauer 3. Uterine papillary serous carcinoma, V. Samouelian, M.-C. Baranzelli, A. Lesoin 4. Uterine sarcomas and rare endometrial carcinomas, H. Boussen, K. Rahal, N. Bouaouina 5. Rare Tumours of the uterine cervix, H. Lamallem, A. Toledano, P. Tsoutsou 6. Tumours of the vagina and tumours of the vulva, C. Haie-Meder SECTION 6. BREAST 1. Invasive breast cancer and ductal carcinoma in situ in young women, G Grüber, S. Aebi 2.Tubular carcinoma of the breast, T. Sullivan, A. Taghian 3. Adenoid Cystic Carcinoma of the Breast, K. Khanfir 4. Primary squamous cell carcinoma of the breast, L. Scandolaro 5. Primary breast lymphoma,W. Jeanneret Sozzi, R.-O. Mirimanoff 6. Phyllodes tumours of the breast, G. Bousquet, J. Bonneterre, Y. Belkacemi 7. Primary Breast Sarcoma, L. Zelek, M. Spielmann, A. Le Cesne 8. Angiosarcoma of the breast, Y. Kirova, A. Fourquet 9. Male breast cancer, B. Cutuli SECTION 7. LUNG AND INTRATHORACIC MALIGNANCIES 1. Primary uncommon tumours of the lung : lymphomas, sarcomas and melanomas , K. Peignaux, F. Maingon 2 .Thymoma and thymic carcinoma, N. Girard, F. Mornex, P. van Houtte, J.-F. Cordier 3. Large Cell Neuroendocrine Carcinoma of the Lung, J.-Y. Perentes, H.-B. Ris 4. Neuroendocrine tumours of the loung, A. Bonnichon, H. Le Floch, F. Rivière, J. Margery, F. Vaylet 5. Pleural mesothelioma, E. Dansin, S Dewas, H Porte 6. bronchioalveolar carcinoma and adenocarcinoma, J. Cadranel, M Antoine, M Wislez 7. Primary adenoid cystic carcinoma of the trachea and bronchus, M Roelandts, P. van Houtte 8.Tracheal cancers, E. Touboul, F. Hughet SECTION 8. MALIGNANCIES OF THE SKIN 1. Unusual cutaneous malignancies, B. Guillot 2. Kaposi's sarcoma, Y. Kirova 3. Dermatofibrosarcoma protuberans, R. Panizzon 4. Merkel cell carcinoma, K. Beer 5. Langerhans cell histicytotis, M. Heinrich Seegenschmiedt SECTION 9. OTHER MALIGNANCIES 1. Primary bone lymphoma (Osteolymphoma), D. Christie 2. Erdheim-Chester disease, RC Miller 3. Solitarylasmacytoma, R. Tsang 4. Neuroendocrine tumours, K. Oberg
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