Textbook of Uncommon Cancer

An uncommon tumor presents a difficult challenge to the clinician as advice on diagnosis, treatment and therapy is scarce. Rather than spend extended periods of time researching each new presentation of an uncommon cancer, Textbook of Uncommon Cancer provides the clinician with all the information and advice needed in one comprehensive guide.

The fifth edition of this authoritative and unique text has been fully revised with new material and an evidence–based, teach–by–example approach in order to deliver unparalleled insight on real–world decision–making in the clinical setting. Where evidence is scarce, the authors provide paradigms on how they address the relevant issues from first principles. Contributions are made by highly experienced and senior teams of medical oncologists, radiation oncologists, and other specialists, giving a balanced and complete overview on how to address each malignancy. New to this edition are;

An increased focus on gene expressions to refine diagnosis The inclusion of heat maps to show this gene expression An increased focus on explaining histopathology

Textbook of Uncommon Cancer enables readers to improve their quality of care by providing expert collation of limited evidence–based and clinical data. Beautifully designed with full color throughout, this is the ideal resource for the clinician inexperienced with treating rare tumors.

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

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, USA