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The editors
Derek Raghavan, MD, PhD, FACP, FRACP, FASCO. President, Levine Cancer Institute, Carolinas HealthCare System; Professor of Medicine, University of North Carolina School of Medicine, Charlotte, North Carolina, 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.
Charles D. Blanke, MD, FACP, FASCO. Professor of Medicine, Division of Hematology and Medical Oncology, Knight Cancer Institute, Portland, Oregon, USA.
Jubilee Brown, MD. Professor and Associate Director of Gynecologic Oncology, Levine Cancer Institute, Carolinas HealthCare System, Charlotte, North Carolina, USA
Edward S. Kim, MD, FACP. Chair, Department of Solid Tumor Oncology and Investigational Therapeutics; Donald S. Kim Distinguished Chair for Cancer Research, Levine Cancer Institute, Carolinas HealthCare System, Charlotte, North Carolina, USA.
Gregory H. Reaman, MD. Professor of Pediatrics, George Washington University School of Medicine and Health Sciences; Center for Cancer and Blood Disorders, Children’s National Health System, Washington, District of Columbia, USA.
Mikkael A. Sekeres, MD, MS. Director, Leukemia Program and Professor of Medicine, Cleveland Clinic, Cleveland, Ohio, USA.
Contributors, xi
Preface, xxi
Abbreviations, xxiii
Section 1 General Principles of Care of Uncommon Cancers
1 A Structured Approach to Uncommon Cancers, 1Derek Raghavan
2 What Patients Need to Know: Information Needs and Information Sources of Patients Diagnosed with Rare Cancers, 6Dana L. Ladd
3 Involving the Public in Rare Cancer Care and Research: Shaping the Future for Rare and Uncommon Cancers, 12Jack 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, 19Guillermo De Velasco, Sabina Signoretti, Brian I. Rini, and Toni K. Choueiri
5 Uncommon Cancers of the Bladder, 41Humaid Al‐Shamsi, Donna E. Hansel, Joaquim Bellmunt, and Arlene O. Siefker‐Radtke
6 Urethral Cancer, 54Tanya B. Dorff, Leslie K. Ballas, Anne K. Schuckman,Manju Aron, and David I. Quinn
7 Uncommon Cancers of the Prostate, 68Scott T. Tagawa, Jaspreet S. Batra, Brian D. Robinson, and Ana Aparicio
8 Rare Tumors of the Testis and Paratesticular Tissues, 97Robert Huddart, Anna Patrikidou, and Stephen Hazell
9 Penile Carcinoma, 122J. 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, 134Madalina Tuluc, Voichita Bar‐Ad, David Cognetti, Jennifer Johnson, and Rita Axelrod
11 Rare Tumors of the Larynx, 152Kenneth J. Niermann, Jill Gilbert, Kyle Mannion, Joseph M. Aulino, Kim Ely, and Barbara A. Murphy
12 Nasopharyngeal Carcinoma in Nonendemic Populations, 165Lachlan McDowell, Annette Lim, and June Corry
13 Esthesioneuroblastoma, 196Christine H. Chung, Gary L. Gallia, Bruce M. Wenig, John A. Arrington, and Jimmy J. Caudell
Section 4 Thoracic Tumors
14 Thymoma and Thymic Carcinoma, 214Mohammad Abu Zaid, Kenneth Kesler, Jessica Smith, Sunil Badve, and Patrick J. Loehrer Sr
15 Primary Sarcomas of the Lung, 248Vinod Ravi, Mara Antonoff, Hannah Wingate, Wei‐Lien Wang, and Kelly K. Hunt
16 Mesothelioma, 266Anna K. Nowak, John G. Edwards, and Jenette Creaney
17 Primary Melanoma of the Lung, 285Louise A. Jackett, Richard A. Scolyer, James F. Bishop, and John F. Thompson
18 Large Cell Neuroendocrine Carcinoma, 293Nanna H. Sulai, Edward S. Kim, and Vijayalakshmi Ananthanarayanan
19 Lung Adenocarcinoma: Current Classifications and Uncommon Mutations, 305Kathryn F. Mileham, Jennifer L. Ersek, Edward S. Kim, and Ignacio I. Wistuba
20 Primary Adenoid Cystic Carcinoma of the Lung, 315Charles B. Simone II, John G. Devlin, Sunil Singhal, and Corey J. Langer
Section 5 Breast Cancers
21 Metaplastic Breast Cancer, 327Reva 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, 338Melinda E. Sanders, Ingrid A. Mayer, and A. Bapsi Chakravarthy
23 Breast Sarcoma, 345Mai‐Kim Gervais, Rebecca A. Gladdy, Abha Gupta, Rita Kandel, and Charles Catton
24 Adenoid Cystic Carcinoma of the Breast, 357Antoinette R. Tan and Chad A. Livasy
25 Breast Cancer in Men, 364Suleiman Alfred Massarweh and George W. Sledge Jr
Section 6 Gastrointestinal Tumors
26 Uncommon Tumors of the Esophagus, 377Cristina H. Hajdu, Robert Rosser, and Lawrence Leichman
27 Uncommon Cancers of the Stomach, 395Elizabeta Popa, Felice Schnoll‐Sussman, Arun Jesudian, Govind Nandakumar, and Manish A. Shah
28 Uncommon Cancers of the Small Bowel, 416Kanwal Raghav, Matthew H. G. Katz, and Michael J. Overman
29 Uncommon Cancers of the Pancreas, 429Maria Diab and Philip A. Philip
30 Uncommon Hepatobiliary Tumors, 444Anthony El‐Khoueiry
31 Unusual Tumors of the Colon, Rectum, and Anus, 458Diane Reidy‐Lagunes, Christopher H. Lieu, Wells A. Messersmith, and Karyn Goodman
32 Gastrointestinal Stromal Tumors, 470Margaret von Mehren, Brian P. Rubin, Douglas B. Flieder, and Chandrajit P. Raut
Section 7 Gynecological Cancers
33 Surgery for Uncommon Gynecological Cancers, 493R. Wendel Naumann
34 Histologies That Cross Organ Types, 508Amanda Jackson and Preetha Ramalingam
35 Germ Cell Tumors of the Ovary, 518Daniela E. Matei, A. Lindsay Frazier, Jean A. Hurteau, and Farzana D. Pashankar
36 Sex Cord-Stromal Tumors of the Ovary, 533Jubilee Brown, Isabelle Ray‐Coquard, and Kris Ann P. Schultz
37 Clear Cell Carcinoma of the Ovary, 550Keiichi Fujiwara, Katsutoshi Oda, Masashi Takano, and Noriomi Matsumura
38 Mucinous Carcinoma of the Ovary, 557Caroline C. Billingsley and David M. Gershenson
39 Low‐Grade Serous Carcinoma of the Ovary, 564Amanda Nickles Fader and Anais Malpica
40 Borderline Tumors of the Ovary, 572Erin K. Crane and Premal H. Thaker
41 Fallopian Tube and Peritoneal Cancers, 582Destin Black, Kara Long Roche, and Ginger J. Gardner
42 Nonendometrioid Endometrial Carcinomas and Uterine Sarcomas, 591Matthew A. Powell
43 Tumors of the Cervix, 618Krishnansu S. Tewari and Bradley J. Monk
44 Tumors of the Vulva and Vagina, 643Vance Broach and Mario M. Leitao Jr
45 Gestational Trophoblastic Disease, 653John Lurain, Michael Seckl, and Julian Schink
46 Targeted Therapy in Gynecological Malignancy, 663Behrouz Zand, Shannon N. Westin, and Robert L. Coleman
Section 8 Endocrine Tumors
47 Adrenal Neoplasms, 681Ashley A. Stewart and Ellen Shannon Story
48 Uncommon Cancers of the Thyroid, 713Bhavana Konda and Manisha H. Shah
49 Parathyroid Carcinoma, 730Colleen Veloski and John A. Ridge
Section 9 Hematological Malignancies
50 Rare Acute Leukemias, 740B. Douglas Smith and Jonathan Webster
51 Unusual Myelodysplastic and Myelodysplastic/ Myeloproliferative Neoplasms, 752Eric Padron and Rami Komrokji
52 Rare B Cell Lymphoproliferative Disorders, 760Andrew G. Evans and Paul M. Barr
53 Uncommon Presentations of Plasma Cell Dyscrasias, 776Jason Brayer, Mohamad A. Hussein, and Rachid Baz
54 Rare Bone Marrow Failure Conditions, 793Amy S. Duffield and Amy E. DeZern
55 Myeloproliferative Neoplasms: Chronic Myelogenous Leukemia, Polycythemia Vera, Essential Thrombocythemia, and Primary Myelofibrosis, 816Raoul Tibes and Ruben A. Mesa
56 Rare T Cell Lymphomas, 830Aleksandr Lazaryan and John Sweetenham
Section 10 Neurological Tumors
57 Primary Melanotic Tumors of the Central Nervous System, 840L. Burt Nabors, Rong Li, Cheryl A. Palmer, and Philip R. Chapman
58 Comprehensive Management of Chordoma, 847Varun R. Kshettry, Jennifer S. Yu, Vyshak A. Venur, Manmeet S. Ahluwalia, and Pablo F. Recinos
59 Atypical and Malignant Meningiomas, 856Brandyn A. Castro, Melissa M. J. Chua, and Priscilla K. Brastianos
60 Primary Central Nervous System Lymphoma, 871Macarena I. de la Fuente and Lisa M. DeAngelis
61 Choroid Plexus Tumors, 883Jonathan Aicardi and Nicholas A. Butowski
62 Diffuse Low‐Grade Gliomas, 888Nancy Ann Oberheim Bush, Daniel Yang, Jennifer Clarke, and Daphne Haas‐Kogan
63 High‐Grade Gliomas, 899Ashley L. Sumrall
64 Ependymomas, 907Roberta Rudà, Mark R. Gilbert, and Riccardo Soffietti
65 Medulloblastoma in Adults, 917Anna M. Kenney, Robert C. Castellino, Paul L. Moots, Christopher D. Corso, and Minesh P. Mehta
66 Pineal Parenchymal Tumors, 939Yosuke Kitagawa, Patrick Y. Wen, and Shota Tanaka
67 Craniopharyngiomas, 949Danilo Silva, Mayur Sharma, Gazanfar Rahmathulla, and Gene H. Barnett
68 Ophthalmic Cancers, 964Maria M. Choudhary and Arun D. Singh
Section 11 Cutaneous Malignancies
69 Unusual Cutaneous Malignancies, 976Charles T. Darragh, Lee E. Wheless, William G. Stebbins, Jeffrey P. Zwerner, Anthony J. Crimaldi, Thomas Olencki, and Laura Y. McGirt
70 Dermatofibrosarcoma Protuberans, 994Carrie Luu, Jane L. Messina, Andrew S. Brohl, and Vernon K. Sondak
71 Unusual Melanomas, 1002Morganna Freeman, Richard D. Carvajal, Mark Faries, and Omid Hamid
Section 12 Musculoskeletal Tumors
72 Uncommon Tumors of Soft Tissue, 1022William Ahrens, Michael Livingston, Robert Lopez, Anthony J. Crimaldi, and Jeffrey Kneisl
Section 13 Pediatric Cancers
73 Introduction to Rare Pediatric Malignancies, 1069Alberto S. Pappo and Gregory H. Reaman
74 Uncommon Tumors of the Gastrointestinal Tract in Children, 1072Alberto S. Pappo and Wayne L. Furman
75 Rare Pediatric Malignancies of the Head and Neck, 1088Cynthia E. Herzog, Michael E. Kupferman, Winston Huh, and Anita Mahajan
76 Uncommon Pediatric Tumors of the Thorax, 1097Yoav H. Messinger, Kris Ann P. Schultz, and Louis P. Dehner
77 Uncommon Adrenal Tumors in Children and Adolescents, 1126Raul C. Ribeiro, Carlos Rodriguez‐Galindo, Emilia M. Pinto, Gerard P. Zambetti, and Bonald C. Figueiredo
78 Uncommon Pediatric Genitourinary Tumors, 1138Hans Pohl and Jeffrey S. Dome
79 Uncommon Pediatric Brain Tumors, 1152Robert C. Castellino, Matthew Schniederjan, and Tobey J. MacDonald
80 Malignant Tumors of the Skin and Subcutaneous Tissue in Children, 1172Alberto S. Pappo and Carlos Rodriguez‐Galindo
81 Rare Hematological Malignancies in Children, 1190Abby M. Green and Stephen P. Hunger
Index, 1203
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