The fifth edition of the only comprehensive text dealing exclusively with rare or infrequently encountered malignancies in adults and children is an essential resource for any clinical oncologist.
Encompasses all the information needed to diagnose and manage uncommon cancers, an area where advice and guidance is typically scarce
Fully revised with new material and an evidence-based, teach-by-example approach
Provides insight on real-world decision making in the clinical setting
Edited and authored by a highly experienced and senior team of medical oncologists, radiation oncologists, and other specialists, giving a balanced and complete overview
Extensively illustrated in full color throughout, including heat maps to show gene expression
Contents
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
Author Information
Derek Raghavan MD, PhD FACP FRACP FASCO
President of the Levine Cancer Institute and Professor of Medicine, Carolinas HealthCare System, Charlotte, NC, USA
Manmeet S. Ahluwalia MD, FACP
Director, Brain Metastasis Research Program and Dean and Diane Miller Family Endowed Chair in Neuro-Oncology at the Cleveland Clinic Taussig Cancer Institute and Neurologic Institute, Cleveland, OH, USA
Charles D. Blanke MD, FACP FASCO
Professor of Medicine, Knight Cancer Institute and Oregon Health and Sciences University and Chair, SWOG, Portland, Oregon, USA
Jubilee Brown MD
Associate Director of Gynecologic Oncology, Levine Cancer Institute of the Carolinas HealthCare System, Charlotte, NC USA
Edward S. Kim MD, FACP
Chair of Solid Tumor Oncology and Investigational Therapeutics and the Donald S. Kim Distinguished Chair for Cancer Research at the Levine Cancer Institute, Carolinas HealthCare System, Charlotte, NC, USA
Gregory H. Reaman MD
Associate Director, Office of Hematology and Oncology Products, CDER, U.S. FDA and Founding Chair of the Children's Oncology Group, Professor of Pediatrics, George Washington University School of Medicine and Health Sciences and Children's National Health System, Washington DC, DC, USA

Mikkael A. Sekeres MD, MS
Director of the Leukemia Program at the Cleveland Clinic Taussig Cancer Institute, Cleveland, OH, US