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Contributors, xi
Preface, xxi
Abbreviations, xxiii
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 Cancer
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 A. 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 G. 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 Reva K. Basho, Helenice Gobbi, Bryan T. Hennessy, Michael Z. Gilcrease, A. Bapsi Chakravarthy, Gildy Babiera, Wei Yang, Vicente Valero, Gabriel N. Hortobagyi, Stacy L. Moulder, and Ingrid 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, Matthew 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, Brian P. Rubin, Douglas B. Flieder, 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 Kris Ann P. 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 J. 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 J. Crimaldi, and Jeffrey Kneisl
Section 13 Pediatric Cancers
73 Introduction to Rare Pediatric Malignancies, 1069 Alberto S. Pappo and Gregory H. Reaman
74 Uncommon Tumors of the Gastrointestinal Tract in Children, 1072 Alberto S. Pappo and Wayne L. Furman
75 Rare Pediatric Malignancies of the Head and Neck, 1088 Cynthia E. Herzog, Michael E. Kupferman, Winston Huh, and Anita Mahajan
76 Uncommon Pediatric Tumors of the Thorax, 1097 Yoav H. Messinger, Kris Ann P. Schultz, and Louis P. Dehner
77 Uncommon Adrenal Tumors in Children and Adolescents, 1126 Raul C. Ribeiro, Carlos Rodriguez-Galindo, Emilia M. Pinto, Gerard P. Zambetti, and Bonald C. Figueiredo
78 Uncommon Pediatric Genitourinary Tumors, 1138 Hans Pohl and Jeffrey S. Dome
79 Uncommon Pediatric Brain Tumors, 1152 Robert C. Castellino, Matthew Schniederjan, and Tobey J. MacDonald
80 Malignant Tumors of the Skin and Subcutaneous Tissue in Children, 1172 Alberto S. Pappo and Carlos Rodriguez-Galindo
81 Rare Hematological Malignancies in Children, 1190 Abby M. Green and Stephen P. Hunger
Index, 1203
Mohammad Abu Zaid, MD Assistant Professor Medicine Bone Marrow and Blood Stem Cell Transplantation Indiana University Melvin and Bren Simon Cancer Center Indianapolis, Indiana, USA
Manmeet S. Ahluwalia, MD, FACP Dean and Diane Miller Family Endowed Chair in Neuro-Oncology Head of Operations Director, Brain Metastasis Research Program; Associate Director, Clinical Trials The Rose Ella Burkhardt Brain Tumor and Neuro-Oncology Center Neurological Institute Cleveland Clinic; Associate Professor Department of Medicine Cleveland Clinic Lerner College of Medicine of Case Western Reserve University Cleveland, Ohio, USA
William Ahrens, MD Director of Bone and Soft Tissue Pathology Department of Pathology Carolinas Medical Center; Carolinas Pathology Group, Charlotte, North Carolina, USA
Jonathan Aicardi, DO Medical Student Touro University College of Osteopathic Medicine San Francisco, California, USA
Humaid Al-Shamsi, MD Assistant Professor of Gastrointestinal Medical Oncology The University of Texas MD Anderson Cancer Center Houston, Texas, USA
Vijayalakshmi Ananthanarayanan, MD Assistant Professor Department of Pathology Loyola University Medical Center Maywood, Illinois, USA
Mara Antonoff, MD Assistant Professor Departments of Thoracic and Cardiovascular Surgery The University of Texas MD Anderson Cancer Center Houston, Texas, USA
Ana Aparicio, MD Associate Professor The University of Texas MD Anderson Cancer Center Houston, Texas, USA
Manju Aron, MD Assistant Professor of Clinical Pathology Department of Pathology Keck School of Medicine at USC USC Norris Comprehensive Cancer Center Los Angeles, California, USA
John A. Arrington, MD Senior Member Department of Diagnostic Imaging H. Lee Moffitt Cancer Center and Research Institute; Professor of Radiology and Oncologic Sciences USF Morsani College of Medicine University of South Florida Tampa, Florida, USA
Joseph M. Aulino, MD Assistant Professor, Neuroradiology Department of Radiology and Radiological Sciences Vanderbilt-Ingram Cancer Center Vanderbilt University Medical Center Nashville, Tennessee, USA
Rita Axelrod, MD Professor Department of Medical Oncology Kimmel Cancer Center Thomas Jefferson University Philadelphia, Pennsylvania, USA
Gildy Babiera, MD Associate Professor Department of Surgical Oncology The University of Texas MD Anderson Cancer Center Houston, Texas, USA
Sunil Badve, MD, FRCPath Professor of Pathology and Laboratory Medicine Department of Pathology Indiana University Indianapolis, Indiana, USA
Leslie K. Ballas, MD Assistant Clinical Professor Department of Radiation Oncology Keck School of Medicine at USC USC Norris Comprehensive Cancer Center Los Angeles, California, USA
Voichita Bar-Ad, MD Associate Professor Department of Radiation Oncology Kimmel Cancer Center Thomas Jefferson University Philadelphia, Pennsylvania, USA
Gene H. Barnett, MD, FACS Associate Dean for Faculty Affairs Cleveland Clinic Lerner College of Medicine of Case Western Reserve University; The Rose Ella Burkhardt Chair in Neurosurgical Oncology; Professor and Director Rose Ella Burkhardt Brain Tumor & Neuro-Oncology Center Cleveland Clinic Neurological Institute Department of Neurosurgery Cleveland, Ohio, USA
Paul M. Barr, MD Associate Professor of Medicine and Oncology Department of Medicine James P. Wilmot Cancer Center Institute University of Rochester Rochester, New York, USA
Reva K. Basho, MD Assistant Professor Division of Medicine The University of Texas MD Anderson Cancer Center Houston, Texas, USA
Jaspreet S. Batra, MD Research Associate Department of Urology Weill Cornell Medical College of Cornell University New York, New York, USA Rachid Baz, MD Associate Member Department of Malignant Hematology H. Lee Moffitt Cancer Center and Research Institute; Assistant Professor Department of Oncologic Sciences and Medicine USF Morsani College of Medicine University of South Florida Tampa, Florida, USA
Joaquim Bellmunt, MD, PhD Associate Professor of Genitourinary Medical Oncology Dana-Farber Cancer Institute Boston, Massachusetts, USA
Caroline C. Billingsley, MD Assistant Professor/Gynecologic Oncologist Department of Obstetrics and Gynecology Division of Gynecologic Oncology University of Cincinnati Medical Center Cincinnati, Ohio, USA
James F. Bishop, AO, MD, MMED, MBBS, FRACP, FRCPA Executive Director Victorian Comprehensive Cancer Centre Parkville; Professor of Cancer Medicine Department of Medicine, Dentistry and Health Sciences University of Melbourne Melbourne Victoria, Australia
Marie M. Bismark, MBChB, LLB, MBHL, MPH, FAFPHM Consumer Advisory Panel Walter and Eliza Hall Institute of Medical Research Consumer Advisory Panel; Associate Professor Melbourne School of Population and Global Health University of Melbourne Melbourne, Victoria, Australia
Destin Black, MD Director Division of Gynecologic Oncology Department of Obstetrics and Gynecology Louisiana State University Health Science Center Shreveport, Louisiana, USA
Charles D. Blanke, MD, FACP, FASCO Professor of Medicine Division of Hematology and Medical Oncology Knight Cancer Institute Portland, Oregon, USA
Priscilla K. Brastianos, MD Assistant Professor in Medicine Harvard Medical School; Director, Central Nervous System Metastasis Program Divisions of Hematology/Oncology and Neuro-Oncology Departments of Medicine and Neurology Massachusetts General Hospital Boston, Massachusetts, USA
Jason Brayer, MD, PhD Assistant Member Department of Malignant Hematology & Immunology Program H. Lee Moffitt Cancer Center & Research Institute; Instructor USF Morsani College of Medicine University of South Florida Tampa, Florida, USA
Vance Broach, MD Gynecologic Oncology Fellow Department of Surgery, Gynecology Service Memorial Sloan Kettering Cancer Center New York, New York, USA
Andrew S. Brohl, MD Assistant Member Departments of Sarcoma and Cutaneous Oncology H. Lee Moffitt Cancer Center and Research Institute Tampa, Florida, USA
Jubilee Brown, MD Professor and Associate Director Division of Gynecologic Oncology Department of Obstetrics & Gynecology Levine Cancer Institute Carolinas HealthCare System Charlotte, North Carolina, USA
Earle F. Burgess, MD Chief, Section of GU Medical Oncology Department of Solid Tumor Oncology and Investigational Therapeutics Levine Cancer Institute Carolinas HealthCare System Charlotte, North Carolina, USA
Nicholas A. Butowski, MD Professor of Neuro-Oncology Department of Neurological Surgery University of California San Francisco, California, USA
Richard D. Carvajal, MD Associate Professor of Medicine Director, Experimental Therapeutics and Melanoma Service Herbert Irving Comprehensive Cancer Center Columbia University Medical Center New York, New York, USA
Robert C. Castellino, MD Professor of Pediatrics Pediatric Neuro-Oncology Aflac Cancer and Blood Disorders Center Children's Healthcare of Atlanta; Department of Pediatrics Emory University School of Medicine Atlanta, Georgia, USA
Brandyn A. Castro, MD Resident Physician Department of Neurological Surgery University of Chicago Chicago, Illinois, USA; Research Associate Departments of Medicine and Neurological Surgery Massachusetts General Hospital Boston, Massachusetts, USA
Charles Catton, MD, FRCPC Professor Department of Radiation Oncology University of Toronto; Radiation Oncologist Princess Margaret Cancer Centre Toronto, Ontario, Canada
Jimmy J. Caudell, MD, PhD Associate Member Department of Radiation Oncology H. Lee Moffitt Cancer Center and Research Institute Tampa, Florida, USA
A. Bapsi Chakravarthy, MD Assistant Professor Department of Radiation Oncology Vanderbilt University School of Medicine Nashville, Tennessee, USA
Philip R. Chapman, MD Associate Professor Neuroradiology Section Department of Radiology University of Alabama Birmingham, Alabama, USA
Sarah F. Cherry, BA, LLB(hons), QLTT(UK) Consumer Advisory Panel Walter and Eliza Hall Institute of Medical Research; Barrister, the Victorian Bar Victoria, Australia
Maria M. Choudhary, MD Resident Department of Internal Medicine Cole Eye Institute Cleveland Clinic Cleveland, Ohio, USA
Toni K. Choueiri, MD Medical...
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