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Contributors x Preface xx Abbreviations xxi Section 1 General Principles of Care of Uncommon Cancers 1 A Structured Approach to Uncommon Cancers 1 Derek Raghavan 2 What Patients Need to Know: Information Needs and Information Sources of Patients Diagnosed with Rare Cancers 6 Dana L. Ladd 3 Involving the Public in Rare Cancer Care and Research: Shaping the Future for Rare and Uncommon Cancers 12 Jack S. Nunn, Clare L. Scott, John W. Stubbs, Sarah F. Cherry, and Marie M. Bismark Section 2 Genitourinary Cancers 4 Uncommon Tumors of the Kidney 19 Guillermo De Velasco, Sabina Signoretti, Brian I. Rini, and Toni K. Choueiri 5 Uncommon Cancers of the Bladder 41 Humaid Al???Shamsi, Donna E. Hansel, Joaquim Bellmunt, and Arlene O. Siefker???Radtke 6 Urethral Cancer 54 Tanya B. Dorff, Leslie K. Ballas, Anne K. Schuckman, Manju Aron and David I. Quinn 7 Uncommon Cancers of the Prostate 68 Scott T. Tagawa, Jaspreet S. Batra, Brian D. Robinson and Ana Aparicio 8 Rare Tumors of the Testis and Paratesticular Tissues 97 Robert Huddart, Anna Patrikidou, and Stephen Hazell 9 Penile Carcinoma 122 J. Ryan Mark, Stephen B. Riggs, David L. Graham, Derek R. McHaffie, Chad A. Livasy, and Earle F. Burgess Section 3 Head and Neck 10 Uncommon Tumors of the Oral Cavity and Adjacent Structures 134 Madalina Tuluc, Voichita Bar???Ad, David Cognetti, Jennifer Johnson, and Rita Axelrod 11 Rare Tumors of the Larynx 152 Kenneth J. Niermann, Jill Gilbert, Kyle Mannion, Joseph M. Aulino, Kim Ely, and Barbara A. Murphy 12 Nasopharyngeal Carcinoma in Nonendemic Populations 165 Lachlan McDowell, Annette Lim, and June Corry 13 Esthesioneuroblastoma 196 Christine H. Chung, Gary L. Gallia, Bruce M. Wenig, John Arrington, and Jimmy J. Caudell Section 4 Thoracic Tumors 14 Thymoma and Thymic Carcinoma 214 Mohammad Abu Zaid, Kenneth Kesler, Jessica Smith, Sunil Badve, and Patrick J. Loehrer Sr 15 Primary Sarcomas of the Lung 248 Vinod Ravi, Mara Antonoff, Hannah Wingate, Wei???Lien Wang, and Kelly K. Hunt 16 Mesothelioma 266 Anna K. Nowak, John Edwards, and Jenette Creaney 17 Primary Melanoma of the Lung 285 Louise A. Jackett, Richard A. Scolyer, James F. Bishop, and John F. Thompson 18 Large Cell Neuroendocrine Carcinoma 293 Nanna H. Sulai, Edward S. Kim, and Vijayalakshmi Ananthanarayanan 19 Lung Adenocarcinoma: Current Classifications and Uncommon Mutations 305 Kathryn F. Mileham, Jennifer L. Ersek, Edward S. Kim, and Ignacio I. Wistuba 20 Primary Adenoid Cystic Carcinoma of the Lung 315 Charles B. Simone II, John G. Devlin, Sunil Singhal, and Corey J. Langer Section 5 Breast Cancers 21 Metaplastic Breast Cancer 327 R.K. Basho, H. Gobbi, B.T. Hennessy, M.Z. Gilcrease, A.B. Chakravarthy, G. Babiera, W. Yang, V. Valero, G.N. Hortobagyi, S.L. Moulder, and I.A. Mayer 22 Tubular Carcinoma of the Breast 338 Melinda E. Sanders, Ingrid A. Mayer, and A. Bapsi Chakravarthy 23 Breast Sarcoma 345 Mai???Kim Gervais, Rebecca A. Gladdy, Abha Gupta, Rita Kandel, and Charles Catton 24 Adenoid Cystic Carcinoma of the Breast 357 Antoinette R. Tan and Chad A. Livasy 25 Breast Cancer in Men 364 Suleiman Alfred Massarweh and George W. Sledge Jr Section 6 Gastrointestinal Tumors 26 Uncommon Tumors of the Esophagus 377 Cristina H. Hajdu, Robert Rosser, and Lawrence Leichman 27 Uncommon Cancers of the Stomach 395 Elizabeta Popa, Felice Schnoll???Sussman, Arun Jesudian, Govind Nandakumar, and Manish A. Shah 28 Uncommon Cancers of the Small Bowel 416 Kanwal Raghav, Mathew H. G. Katz, and Michael J. Overman 29 Uncommon Cancers of the Pancreas 429 Maria Diab and Philip A. Philip 30 Uncommon Hepatobiliary Tumors 444 Anthony El???Khoueiry 31 Unusual Tumors of the Colon, Rectum, and Anus 458 Diane Reidy???Lagunes, Christopher H. Lieu, Wells A. Messersmith, and Karyn Goodman 32 Gastrointestinal Stromal Tumors 470 Margaret von Mehren, Douglas B. Flieder, Brian P. Rubin, and Chandrajit P. Raut Section 7 Gynecological Cancers 33 Surgery for Uncommon Gynecological Cancers 493 R. Wendel Naumann 34 Histologies That Cross Organ Types 508 Amanda Jackson and Preetha Ramalingam 35 Germ Cell Tumors of the Ovary 518 Daniela E. Matei, A. Lindsay Frazier, Jean A. Hurteau, and Farzana D. Pashankar 36 Sex Cord Stromal Tumors of the Ovary 533 Jubilee Brown, Isabelle Ray???Coquard, and KrisAnn Schultz 37 Clear Cell Carcinoma of the Ovary 550 Keiichi Fujiwara, Katsutoshi Oda, Masashi Takano, and Noriomi Matsumura 38 Mucinous Carcinoma of the Ovary 557 Caroline C. Billingsley and David M. Gershenson 39 Low???Grade Serous Carcinoma of the Ovary 564 Amanda Nickles Fader and Anais Malpica 40 Borderline Tumors of the Ovary 572 Erin K. Crane and Premal H. Thaker 41 Fallopian Tube and Peritoneal Cancers 582 Destin Black, Kara Long Roche, and Ginger Gardner 42 Nonendometrioid Endometrial Carcinomas and Uterine Sarcomas 591 Matthew A. Powell 43 Tumors of the Cervix 618 Krishnansu S. Tewari and Bradley J. Monk 44 Tumors of the Vulva and Vagina 643 Vance Broach and Mario M. Leitao Jr 45 Gestational Trophoblastic Disease 653 John Lurain, Michael Seckl, and Julian Schink 46 Targeted Therapy in Gynecological Malignancy 663 Behrouz Zand, Shannon N. Westin, and Robert L. Coleman Section 8 Endocrine Tumors 47 Adrenal Neoplasms 681 Ashley A. Stewart and Ellen Shannon Story 48 Uncommon Cancers of the Thyroid 713 Bhavana Konda and Manisha H. Shah 49 Parathyroid Carcinoma 730 Colleen Veloski and John A. Ridge Section 9 Hematological Malignancies 50 Rare Acute Leukemias 740 B. Douglas Smith and Jonathan Webster 51 Unusual Myelodysplastic and Myelodysplastic/Myeloproliferative Neoplasms 752 Eric Padron and Rami Komrokji 52 Rare B Cell Lymphoproliferative Disorders 760 Andrew G. Evans and Paul M. Barr 53 Uncommon Presentations of Plasma Cell Dyscrasias 776 Jason Brayer, Mohamad A. Hussein, and Rachid Baz 54 Rare Bone Marrow Failure Conditions 793 Amy S. Duffield and Amy E. DeZern 55 Myeloproliferative Neoplasms: Chronic Myelogenous Leukemia, Polycythemia Vera, Essential Thrombocythemia, and Primary Myelofibrosis 816 Raoul Tibes and Ruben A. Mesa 56 Rare T Cell Lymphomas 830 Aleksandr Lazaryan and John Sweetenham Section 10 Neurological Tumors 57 Primary Melanotic Tumors of the Central Nervous System 840 L. Burt Nabors, Rong Li, Cheryl A. Palmer, and Philip R. Chapman 58 Comprehensive Management of Chordoma 847 Varun R. Kshettry, Jennifer S. Yu, Vyshak A. Venur, Manmeet S. Ahluwalia, and Pablo F. Recinos 59 Atypical and Malignant Meningiomas 856 Brandyn A. Castro, Melissa M. J. Chua, and Priscilla K. Brastianos 60 Primary Central Nervous System Lymphoma 871 Macarena I. de la Fuente and Lisa M. DeAngelis 61 Choroid Plexus Tumors 883 Jonathan Aicardi and Nicholas A. Butowski 62 Diffuse Low???Grade Gliomas 888 Nancy Ann Oberheim Bush, Daniel Yang, Jennifer Clarke, and Daphne Haas???Kogan 63 High???Grade Gliomas 899 Ashley L. Sumrall 64 Ependymomas 907 Roberta Rudà, Mark R. Gilbert, and Riccardo Soffietti 65 Medulloblastoma in Adults 917 Anna M. Kenney, Robert C. Castellino, Paul L. Moots, Christopher D. Corso, and Minesh P. Mehta 66 Pineal Parenchymal Tumors 939 Yosuke Kitagawa, Patrick Y. Wen, and Shota Tanaka 67 Craniopharyngiomas 949 Danilo Silva, Mayur Sharma, Gazanfar Rahmathulla, and Gene H. Barnett 68 Ophthalmic Cancers 964 Maria M. Choudhary and Arun D. Singh Section 11 Cutaneous Malignancies 69 Unusual Cutaneous Malignancies 976 Charles T. Darragh, Lee E. Wheless, William G. Stebbins, Jeffrey P. Zwerner, Anthony J. Crimaldi, Thomas Olencki, and Laura Y. McGirt 70 Dermatofibrosarcoma Protuberans 994 Carrie Luu, Jane L. Messina, Andrew S. Brohl, and Vernon K. Sondak 71 Unusual Melanomas 1002 Morganna Freeman, Richard D. Carvajal, Mark Faries, and Omid Hamid Section 12 Musculoskeletal Tumors 72 Uncommon Tumors of Soft Tissue 1022 William Ahrens, Michael Livingston, Robert Lopez, Anthony Crimaldi, and Jeffrey Kneisl Section 13 Pediatric Cancers 73 Introduction to Rare Pediatric Malignancies 1077 Alberto S. Pappo and Gregory H. Reaman 74 Uncommon Tumors of the Gastrointestinal Tract in Children 1080 Alberto S. Pappo and Wayne L. Furman 75 Rare Pediatric Malignancies of the Head and Neck 1096 Cynthia E. Herzog, Michael E. Kupferman, Winston Huh and Anita Mahajan 76 Uncommon Pediatric Tumors of the Thorax 1105 Yoav H. Messinger, Kris Ann P. Schultz, and Louis P. Dehner 77 Uncommon Adrenal Tumors in Children and Adolescents 1134 Raul C. Ribeiro, Carlos Rodriguez???Galindo, Emilia M. Pinto, Gerard P. Zambetti, and Bonald C. Figueiredo 78 Uncommon Pediatric Genitourinary Tumors 1146 Hans Pohl and Jeffrey S. Dome 79 Uncommon Pediatric Brain Tumors 1160 Robert C. Castellino, Matthew Schniederjan, and Tobey J. MacDonald 80 Malignant Tumors of the Skin and Subcutaneous Tissue in Children 1180 Alberto S. Pappo and Carlos Rodriguez???Galindo 81 Rare Hematological Malignancies in Children 1198 Abby M. Green and Stephen P. Hunger Index 000