The editorsDerek 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, USAEdward 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, xiPreface, xxiAbbreviations, xxiiiSection 1 General Principles of Care of Uncommon Cancers1 A Structured Approach to Uncommon Cancers, 1Derek Raghavan2 What Patients Need to Know: Information Needs and Information Sources of Patients Diagnosed with Rare Cancers, 6Dana L. Ladd3 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. BismarkSection 2 Genitourinary Cancers4 Uncommon Tumors of the Kidney, 19Guillermo De Velasco, Sabina Signoretti, Brian I. Rini, and Toni K. Choueiri5 Uncommon Cancers of the Bladder, 41Humaid Al‐Shamsi, Donna E. Hansel, Joaquim Bellmunt, and Arlene O. Siefker‐Radtke6 Urethral Cancer, 54Tanya B. Dorff, Leslie K. Ballas, Anne K. Schuckman,Manju Aron, and David I. Quinn7 Uncommon Cancers of the Prostate, 68Scott T. Tagawa, Jaspreet S. Batra, Brian D. Robinson, and Ana Aparicio8 Rare Tumors of the Testis and Paratesticular Tissues, 97Robert Huddart, Anna Patrikidou, and Stephen Hazell9 Penile Carcinoma, 122J. Ryan Mark, Stephen B. Riggs, David L. Graham,Derek R. McHaffie, Chad A. Livasy, and Earle F. BurgessSection 3 Head and Neck Cancer10 Uncommon Tumors of the Oral Cavity and Adjacent Structures, 134Madalina Tuluc, Voichita Bar‐Ad, David Cognetti, Jennifer Johnson, and Rita Axelrod11 Rare Tumors of the Larynx, 152Kenneth J. Niermann, Jill Gilbert, Kyle Mannion, Joseph M. Aulino, Kim Ely, and Barbara A. Murphy12 Nasopharyngeal Carcinoma in Nonendemic Populations, 165Lachlan McDowell, Annette Lim, and June Corry13 Esthesioneuroblastoma, 196Christine H. Chung, Gary L. Gallia, Bruce M. Wenig, John A. Arrington, and Jimmy J. CaudellSection 4 Thoracic Tumors14 Thymoma and Thymic Carcinoma, 214Mohammad Abu Zaid, Kenneth Kesler, Jessica Smith, Sunil Badve, and Patrick J. Loehrer Sr15 Primary Sarcomas of the Lung, 248Vinod Ravi, Mara Antonoff, Hannah Wingate, Wei‐Lien Wang, and Kelly K. Hunt16 Mesothelioma, 266Anna K. Nowak, John G. Edwards, and Jenette Creaney17 Primary Melanoma of the Lung, 285Louise A. Jackett, Richard A. Scolyer, James F. Bishop, and John F. Thompson18 Large Cell Neuroendocrine Carcinoma, 293Nanna H. Sulai, Edward S. Kim, and Vijayalakshmi Ananthanarayanan19 Lung Adenocarcinoma: Current Classifications and Uncommon Mutations, 305Kathryn F. Mileham, Jennifer L. Ersek, Edward S. Kim, and Ignacio I. Wistuba20 Primary Adenoid Cystic Carcinoma of the Lung, 315Charles B. Simone II, John G. Devlin, Sunil Singhal, and Corey J. LangerSection 5 Breast Cancers21 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. Mayer22 Tubular Carcinoma of the Breast, 338Melinda E. Sanders, Ingrid A. Mayer, and A. Bapsi Chakravarthy23 Breast Sarcoma, 345Mai‐Kim Gervais, Rebecca A. Gladdy, Abha Gupta, Rita Kandel, and Charles Catton24 Adenoid Cystic Carcinoma of the Breast, 357Antoinette R. Tan and Chad A. Livasy25 Breast Cancer in Men, 364Suleiman Alfred Massarweh and George W. Sledge JrSection 6 Gastrointestinal Tumors26 Uncommon Tumors of the Esophagus, 377Cristina H. Hajdu, Robert Rosser, and Lawrence Leichman27 Uncommon Cancers of the Stomach, 395Elizabeta Popa, Felice Schnoll‐Sussman, Arun Jesudian, Govind Nandakumar, and Manish A. Shah28 Uncommon Cancers of the Small Bowel, 416Kanwal Raghav, Matthew H. G. Katz, and Michael J. Overman29 Uncommon Cancers of the Pancreas, 429Maria Diab and Philip A. Philip30 Uncommon Hepatobiliary Tumors, 444Anthony El‐Khoueiry31 Unusual Tumors of the Colon, Rectum, and Anus, 458Diane Reidy‐Lagunes, Christopher H. Lieu, Wells A. Messersmith, and Karyn Goodman32 Gastrointestinal Stromal Tumors, 470Margaret von Mehren, Brian P. Rubin, Douglas B. Flieder, and Chandrajit P. RautSection 7 Gynecological Cancers33 Surgery for Uncommon Gynecological Cancers, 493R. Wendel Naumann34 Histologies That Cross Organ Types, 508Amanda Jackson and Preetha Ramalingam35 Germ Cell Tumors of the Ovary, 518Daniela E. Matei, A. Lindsay Frazier, Jean A. Hurteau, and Farzana D. Pashankar36 Sex Cord-Stromal Tumors of the Ovary, 533Jubilee Brown, Isabelle Ray‐Coquard, and Kris Ann P. Schultz37 Clear Cell Carcinoma of the Ovary, 550Keiichi Fujiwara, Katsutoshi Oda, Masashi Takano, and Noriomi Matsumura38 Mucinous Carcinoma of the Ovary, 557Caroline C. Billingsley and David M. Gershenson39 Low‐Grade Serous Carcinoma of the Ovary, 564Amanda Nickles Fader and Anais Malpica40 Borderline Tumors of the Ovary, 572Erin K. Crane and Premal H. Thaker41 Fallopian Tube and Peritoneal Cancers, 582Destin Black, Kara Long Roche, and Ginger J. Gardner42 Nonendometrioid Endometrial Carcinomas and Uterine Sarcomas, 591Matthew A. Powell43 Tumors of the Cervix, 618Krishnansu S. Tewari and Bradley J. Monk44 Tumors of the Vulva and Vagina, 643Vance Broach and Mario M. Leitao Jr45 Gestational Trophoblastic Disease, 653John Lurain, Michael Seckl, and Julian Schink46 Targeted Therapy in Gynecological Malignancy, 663Behrouz Zand, Shannon N. Westin, and Robert L. ColemanSection 8 Endocrine Tumors47 Adrenal Neoplasms, 681Ashley A. Stewart and Ellen Shannon Story48 Uncommon Cancers of the Thyroid, 713Bhavana Konda and Manisha H. Shah49 Parathyroid Carcinoma, 730Colleen Veloski and John A. RidgeSection 9 Hematological Malignancies50 Rare Acute Leukemias, 740B. Douglas Smith and Jonathan Webster51 Unusual Myelodysplastic and Myelodysplastic/ Myeloproliferative Neoplasms, 752Eric Padron and Rami Komrokji52 Rare B Cell Lymphoproliferative Disorders, 760Andrew G. Evans and Paul M. Barr53 Uncommon Presentations of Plasma Cell Dyscrasias, 776Jason Brayer, Mohamad A. Hussein, and Rachid Baz54 Rare Bone Marrow Failure Conditions, 793Amy S. Duffield and Amy E. DeZern55 Myeloproliferative Neoplasms: Chronic Myelogenous Leukemia, Polycythemia Vera, Essential Thrombocythemia, and Primary Myelofibrosis, 816Raoul Tibes and Ruben A. Mesa56 Rare T Cell Lymphomas, 830Aleksandr Lazaryan and John SweetenhamSection 10 Neurological Tumors57 Primary Melanotic Tumors of the Central Nervous System, 840L. Burt Nabors, Rong Li, Cheryl A. Palmer, and Philip R. Chapman58 Comprehensive Management of Chordoma, 847Varun R. Kshettry, Jennifer S. Yu, Vyshak A. Venur, Manmeet S. Ahluwalia, and Pablo F. Recinos59 Atypical and Malignant Meningiomas, 856Brandyn A. Castro, Melissa M. J. Chua, and Priscilla K. Brastianos60 Primary Central Nervous System Lymphoma, 871Macarena I. de la Fuente and Lisa M. DeAngelis61 Choroid Plexus Tumors, 883Jonathan Aicardi and Nicholas A. Butowski62 Diffuse Low‐Grade Gliomas, 888Nancy Ann Oberheim Bush, Daniel Yang, Jennifer Clarke, and Daphne Haas‐Kogan63 High‐Grade Gliomas, 899Ashley L. Sumrall64 Ependymomas, 907Roberta Rudà, Mark R. Gilbert, and Riccardo Soffietti65 Medulloblastoma in Adults, 917Anna M. Kenney, Robert C. Castellino, Paul L. Moots, Christopher D. Corso, and Minesh P. Mehta66 Pineal Parenchymal Tumors, 939Yosuke Kitagawa, Patrick Y. Wen, and Shota Tanaka67 Craniopharyngiomas, 949Danilo Silva, Mayur Sharma, Gazanfar Rahmathulla, and Gene H. Barnett68 Ophthalmic Cancers, 964Maria M. Choudhary and Arun D. SinghSection 11 Cutaneous Malignancies69 Unusual Cutaneous Malignancies, 976Charles T. Darragh, Lee E. Wheless, William G. Stebbins, Jeffrey P. Zwerner, Anthony J. Crimaldi, Thomas Olencki, and Laura Y. McGirt70 Dermatofibrosarcoma Protuberans, 994Carrie Luu, Jane L. Messina, Andrew S. Brohl, and Vernon K. Sondak71 Unusual Melanomas, 1002Morganna Freeman, Richard D. Carvajal, Mark Faries, and Omid HamidSection 12 Musculoskeletal Tumors72 Uncommon Tumors of Soft Tissue, 1022William Ahrens, Michael Livingston, Robert Lopez, Anthony J. Crimaldi, and Jeffrey KneislSection 13 Pediatric Cancers73 Introduction to Rare Pediatric Malignancies, 1069Alberto S. Pappo and Gregory H. Reaman74 Uncommon Tumors of the Gastrointestinal Tract in Children, 1072Alberto S. Pappo and Wayne L. Furman75 Rare Pediatric Malignancies of the Head and Neck, 1088Cynthia E. Herzog, Michael E. Kupferman, Winston Huh, and Anita Mahajan76 Uncommon Pediatric Tumors of the Thorax, 1097Yoav H. Messinger, Kris Ann P. Schultz, and Louis P. Dehner77 Uncommon Adrenal Tumors in Children and Adolescents, 1126Raul C. Ribeiro, Carlos Rodriguez‐Galindo, Emilia M. Pinto, Gerard P. Zambetti, and Bonald C. Figueiredo78 Uncommon Pediatric Genitourinary Tumors, 1138Hans Pohl and Jeffrey S. Dome79 Uncommon Pediatric Brain Tumors, 1152Robert C. Castellino, Matthew Schniederjan, and Tobey J. MacDonald80 Malignant Tumors of the Skin and Subcutaneous Tissue in Children, 1172Alberto S. Pappo and Carlos Rodriguez‐Galindo81 Rare Hematological Malignancies in Children, 1190Abby M. Green and Stephen P. HungerIndex, 1203

The editorsDerek 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, USAEdward 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, xiPreface, xxiAbbreviations, xxiiiSection 1 General Principles of Care of Uncommon Cancers1 A Structured Approach to Uncommon Cancers, 1Derek Raghavan2 What Patients Need to Know: Information Needs and Information Sources of Patients Diagnosed with Rare Cancers, 6Dana L. Ladd3 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. BismarkSection 2 Genitourinary Cancers4 Uncommon Tumors of the Kidney, 19Guillermo De Velasco, Sabina Signoretti, Brian I. Rini, and Toni K. Choueiri5 Uncommon Cancers of the Bladder, 41Humaid Al‐Shamsi, Donna E. Hansel, Joaquim Bellmunt, and Arlene O. Siefker‐Radtke6 Urethral Cancer, 54Tanya B. Dorff, Leslie K. Ballas, Anne K. Schuckman,Manju Aron, and David I. Quinn7 Uncommon Cancers of the Prostate, 68Scott T. Tagawa, Jaspreet S. Batra, Brian D. Robinson, and Ana Aparicio8 Rare Tumors of the Testis and Paratesticular Tissues, 97Robert Huddart, Anna Patrikidou, and Stephen Hazell9 Penile Carcinoma, 122J. Ryan Mark, Stephen B. Riggs, David L. Graham,Derek R. McHaffie, Chad A. Livasy, and Earle F. BurgessSection 3 Head and Neck Cancer10 Uncommon Tumors of the Oral Cavity and Adjacent Structures, 134Madalina Tuluc, Voichita Bar‐Ad, David Cognetti, Jennifer Johnson, and Rita Axelrod11 Rare Tumors of the Larynx, 152Kenneth J. Niermann, Jill Gilbert, Kyle Mannion, Joseph M. Aulino, Kim Ely, and Barbara A. Murphy12 Nasopharyngeal Carcinoma in Nonendemic Populations, 165Lachlan McDowell, Annette Lim, and June Corry13 Esthesioneuroblastoma, 196Christine H. Chung, Gary L. Gallia, Bruce M. Wenig, John A. Arrington, and Jimmy J. CaudellSection 4 Thoracic Tumors14 Thymoma and Thymic Carcinoma, 214Mohammad Abu Zaid, Kenneth Kesler, Jessica Smith, Sunil Badve, and Patrick J. Loehrer Sr15 Primary Sarcomas of the Lung, 248Vinod Ravi, Mara Antonoff, Hannah Wingate, Wei‐Lien Wang, and Kelly K. Hunt16 Mesothelioma, 266Anna K. Nowak, John G. Edwards, and Jenette Creaney17 Primary Melanoma of the Lung, 285Louise A. Jackett, Richard A. Scolyer, James F. Bishop, and John F. Thompson18 Large Cell Neuroendocrine Carcinoma, 293Nanna H. Sulai, Edward S. Kim, and Vijayalakshmi Ananthanarayanan19 Lung Adenocarcinoma: Current Classifications and Uncommon Mutations, 305Kathryn F. Mileham, Jennifer L. Ersek, Edward S. Kim, and Ignacio I. Wistuba20 Primary Adenoid Cystic Carcinoma of the Lung, 315Charles B. Simone II, John G. Devlin, Sunil Singhal, and Corey J. LangerSection 5 Breast Cancers21 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. Mayer22 Tubular Carcinoma of the Breast, 338Melinda E. Sanders, Ingrid A. Mayer, and A. Bapsi Chakravarthy23 Breast Sarcoma, 345Mai‐Kim Gervais, Rebecca A. Gladdy, Abha Gupta, Rita Kandel, and Charles Catton24 Adenoid Cystic Carcinoma of the Breast, 357Antoinette R. Tan and Chad A. Livasy25 Breast Cancer in Men, 364Suleiman Alfred Massarweh and George W. Sledge JrSection 6 Gastrointestinal Tumors26 Uncommon Tumors of the Esophagus, 377Cristina H. Hajdu, Robert Rosser, and Lawrence Leichman27 Uncommon Cancers of the Stomach, 395Elizabeta Popa, Felice Schnoll‐Sussman, Arun Jesudian, Govind Nandakumar, and Manish A. Shah28 Uncommon Cancers of the Small Bowel, 416Kanwal Raghav, Matthew H. G. Katz, and Michael J. Overman29 Uncommon Cancers of the Pancreas, 429Maria Diab and Philip A. Philip30 Uncommon Hepatobiliary Tumors, 444Anthony El‐Khoueiry31 Unusual Tumors of the Colon, Rectum, and Anus, 458Diane Reidy‐Lagunes, Christopher H. Lieu, Wells A. Messersmith, and Karyn Goodman32 Gastrointestinal Stromal Tumors, 470Margaret von Mehren, Brian P. Rubin, Douglas B. Flieder, and Chandrajit P. RautSection 7 Gynecological Cancers33 Surgery for Uncommon Gynecological Cancers, 493R. Wendel Naumann34 Histologies That Cross Organ Types, 508Amanda Jackson and Preetha Ramalingam35 Germ Cell Tumors of the Ovary, 518Daniela E. Matei, A. Lindsay Frazier, Jean A. Hurteau, and Farzana D. Pashankar36 Sex Cord-Stromal Tumors of the Ovary, 533Jubilee Brown, Isabelle Ray‐Coquard, and Kris Ann P. Schultz37 Clear Cell Carcinoma of the Ovary, 550Keiichi Fujiwara, Katsutoshi Oda, Masashi Takano, and Noriomi Matsumura38 Mucinous Carcinoma of the Ovary, 557Caroline C. Billingsley and David M. Gershenson39 Low‐Grade Serous Carcinoma of the Ovary, 564Amanda Nickles Fader and Anais Malpica40 Borderline Tumors of the Ovary, 572Erin K. Crane and Premal H. Thaker41 Fallopian Tube and Peritoneal Cancers, 582Destin Black, Kara Long Roche, and Ginger J. Gardner42 Nonendometrioid Endometrial Carcinomas and Uterine Sarcomas, 591Matthew A. Powell43 Tumors of the Cervix, 618Krishnansu S. Tewari and Bradley J. Monk44 Tumors of the Vulva and Vagina, 643Vance Broach and Mario M. Leitao Jr45 Gestational Trophoblastic Disease, 653John Lurain, Michael Seckl, and Julian Schink46 Targeted Therapy in Gynecological Malignancy, 663Behrouz Zand, Shannon N. Westin, and Robert L. ColemanSection 8 Endocrine Tumors47 Adrenal Neoplasms, 681Ashley A. Stewart and Ellen Shannon Story48 Uncommon Cancers of the Thyroid, 713Bhavana Konda and Manisha H. Shah49 Parathyroid Carcinoma, 730Colleen Veloski and John A. RidgeSection 9 Hematological Malignancies50 Rare Acute Leukemias, 740B. Douglas Smith and Jonathan Webster51 Unusual Myelodysplastic and Myelodysplastic/ Myeloproliferative Neoplasms, 752Eric Padron and Rami Komrokji52 Rare B Cell Lymphoproliferative Disorders, 760Andrew G. Evans and Paul M. Barr53 Uncommon Presentations of Plasma Cell Dyscrasias, 776Jason Brayer, Mohamad A. Hussein, and Rachid Baz54 Rare Bone Marrow Failure Conditions, 793Amy S. Duffield and Amy E. DeZern55 Myeloproliferative Neoplasms: Chronic Myelogenous Leukemia, Polycythemia Vera, Essential Thrombocythemia, and Primary Myelofibrosis, 816Raoul Tibes and Ruben A. Mesa56 Rare T Cell Lymphomas, 830Aleksandr Lazaryan and John SweetenhamSection 10 Neurological Tumors57 Primary Melanotic Tumors of the Central Nervous System, 840L. Burt Nabors, Rong Li, Cheryl A. Palmer, and Philip R. Chapman58 Comprehensive Management of Chordoma, 847Varun R. Kshettry, Jennifer S. Yu, Vyshak A. Venur, Manmeet S. Ahluwalia, and Pablo F. Recinos59 Atypical and Malignant Meningiomas, 856Brandyn A. Castro, Melissa M. J. Chua, and Priscilla K. Brastianos60 Primary Central Nervous System Lymphoma, 871Macarena I. de la Fuente and Lisa M. DeAngelis61 Choroid Plexus Tumors, 883Jonathan Aicardi and Nicholas A. Butowski62 Diffuse Low‐Grade Gliomas, 888Nancy Ann Oberheim Bush, Daniel Yang, Jennifer Clarke, and Daphne Haas‐Kogan63 High‐Grade Gliomas, 899Ashley L. Sumrall64 Ependymomas, 907Roberta Rudà, Mark R. Gilbert, and Riccardo Soffietti65 Medulloblastoma in Adults, 917Anna M. Kenney, Robert C. Castellino, Paul L. Moots, Christopher D. Corso, and Minesh P. Mehta66 Pineal Parenchymal Tumors, 939Yosuke Kitagawa, Patrick Y. Wen, and Shota Tanaka67 Craniopharyngiomas, 949Danilo Silva, Mayur Sharma, Gazanfar Rahmathulla, and Gene H. Barnett68 Ophthalmic Cancers, 964Maria M. Choudhary and Arun D. SinghSection 11 Cutaneous Malignancies69 Unusual Cutaneous Malignancies, 976Charles T. Darragh, Lee E. Wheless, William G. Stebbins, Jeffrey P. Zwerner, Anthony J. Crimaldi, Thomas Olencki, and Laura Y. McGirt70 Dermatofibrosarcoma Protuberans, 994Carrie Luu, Jane L. Messina, Andrew S. Brohl, and Vernon K. Sondak71 Unusual Melanomas, 1002Morganna Freeman, Richard D. Carvajal, Mark Faries, and Omid HamidSection 12 Musculoskeletal Tumors72 Uncommon Tumors of Soft Tissue, 1022William Ahrens, Michael Livingston, Robert Lopez, Anthony J. Crimaldi, and Jeffrey KneislSection 13 Pediatric Cancers73 Introduction to Rare Pediatric Malignancies, 1069Alberto S. Pappo and Gregory H. Reaman74 Uncommon Tumors of the Gastrointestinal Tract in Children, 1072Alberto S. Pappo and Wayne L. Furman75 Rare Pediatric Malignancies of the Head and Neck, 1088Cynthia E. Herzog, Michael E. Kupferman, Winston Huh, and Anita Mahajan76 Uncommon Pediatric Tumors of the Thorax, 1097Yoav H. Messinger, Kris Ann P. Schultz, and Louis P. Dehner77 Uncommon Adrenal Tumors in Children and Adolescents, 1126Raul C. Ribeiro, Carlos Rodriguez‐Galindo, Emilia M. Pinto, Gerard P. Zambetti, and Bonald C. Figueiredo78 Uncommon Pediatric Genitourinary Tumors, 1138Hans Pohl and Jeffrey S. Dome79 Uncommon Pediatric Brain Tumors, 1152Robert C. Castellino, Matthew Schniederjan, and Tobey J. MacDonald80 Malignant Tumors of the Skin and Subcutaneous Tissue in Children, 1172Alberto S. Pappo and Carlos Rodriguez‐Galindo81 Rare Hematological Malignancies in Children, 1190Abby M. Green and Stephen P. HungerIndex, 1203

The editorsDerek 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, USAEdward 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, xiPreface, xxiAbbreviations, xxiiiSection 1 General Principles of Care of Uncommon Cancers1 A Structured Approach to Uncommon Cancers, 1Derek Raghavan2 What Patients Need to Know: Information Needs and Information Sources of Patients Diagnosed with Rare Cancers, 6Dana L. Ladd3 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. BismarkSection 2 Genitourinary Cancers4 Uncommon Tumors of the Kidney, 19Guillermo De Velasco, Sabina Signoretti, Brian I. Rini, and Toni K. Choueiri5 Uncommon Cancers of the Bladder, 41Humaid Al‐Shamsi, Donna E. Hansel, Joaquim Bellmunt, and Arlene O. Siefker‐Radtke6 Urethral Cancer, 54Tanya B. Dorff, Leslie K. Ballas, Anne K. Schuckman,Manju Aron, and David I. Quinn7 Uncommon Cancers of the Prostate, 68Scott T. Tagawa, Jaspreet S. Batra, Brian D. Robinson, and Ana Aparicio8 Rare Tumors of the Testis and Paratesticular Tissues, 97Robert Huddart, Anna Patrikidou, and Stephen Hazell9 Penile Carcinoma, 122J. Ryan Mark, Stephen B. Riggs, David L. Graham,Derek R. McHaffie, Chad A. Livasy, and Earle F. BurgessSection 3 Head and Neck Cancer10 Uncommon Tumors of the Oral Cavity and Adjacent Structures, 134Madalina Tuluc, Voichita Bar‐Ad, David Cognetti, Jennifer Johnson, and Rita Axelrod11 Rare Tumors of the Larynx, 152Kenneth J. Niermann, Jill Gilbert, Kyle Mannion, Joseph M. Aulino, Kim Ely, and Barbara A. Murphy12 Nasopharyngeal Carcinoma in Nonendemic Populations, 165Lachlan McDowell, Annette Lim, and June Corry13 Esthesioneuroblastoma, 196Christine H. Chung, Gary L. Gallia, Bruce M. Wenig, John A. Arrington, and Jimmy J. CaudellSection 4 Thoracic Tumors14 Thymoma and Thymic Carcinoma, 214Mohammad Abu Zaid, Kenneth Kesler, Jessica Smith, Sunil Badve, and Patrick J. Loehrer Sr15 Primary Sarcomas of the Lung, 248Vinod Ravi, Mara Antonoff, Hannah Wingate, Wei‐Lien Wang, and Kelly K. Hunt16 Mesothelioma, 266Anna K. Nowak, John G. Edwards, and Jenette Creaney17 Primary Melanoma of the Lung, 285Louise A. Jackett, Richard A. Scolyer, James F. Bishop, and John F. Thompson18 Large Cell Neuroendocrine Carcinoma, 293Nanna H. Sulai, Edward S. Kim, and Vijayalakshmi Ananthanarayanan19 Lung Adenocarcinoma: Current Classifications and Uncommon Mutations, 305Kathryn F. Mileham, Jennifer L. Ersek, Edward S. Kim, and Ignacio I. Wistuba20 Primary Adenoid Cystic Carcinoma of the Lung, 315Charles B. Simone II, John G. Devlin, Sunil Singhal, and Corey J. LangerSection 5 Breast Cancers21 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. Mayer22 Tubular Carcinoma of the Breast, 338Melinda E. Sanders, Ingrid A. Mayer, and A. Bapsi Chakravarthy23 Breast Sarcoma, 345Mai‐Kim Gervais, Rebecca A. Gladdy, Abha Gupta, Rita Kandel, and Charles Catton24 Adenoid Cystic Carcinoma of the Breast, 357Antoinette R. Tan and Chad A. Livasy25 Breast Cancer in Men, 364Suleiman Alfred Massarweh and George W. Sledge JrSection 6 Gastrointestinal Tumors26 Uncommon Tumors of the Esophagus, 377Cristina H. Hajdu, Robert Rosser, and Lawrence Leichman27 Uncommon Cancers of the Stomach, 395Elizabeta Popa, Felice Schnoll‐Sussman, Arun Jesudian, Govind Nandakumar, and Manish A. Shah28 Uncommon Cancers of the Small Bowel, 416Kanwal Raghav, Matthew H. G. Katz, and Michael J. Overman29 Uncommon Cancers of the Pancreas, 429Maria Diab and Philip A. Philip30 Uncommon Hepatobiliary Tumors, 444Anthony El‐Khoueiry31 Unusual Tumors of the Colon, Rectum, and Anus, 458Diane Reidy‐Lagunes, Christopher H. Lieu, Wells A. Messersmith, and Karyn Goodman32 Gastrointestinal Stromal Tumors, 470Margaret von Mehren, Brian P. Rubin, Douglas B. Flieder, and Chandrajit P. RautSection 7 Gynecological Cancers33 Surgery for Uncommon Gynecological Cancers, 493R. Wendel Naumann34 Histologies That Cross Organ Types, 508Amanda Jackson and Preetha Ramalingam35 Germ Cell Tumors of the Ovary, 518Daniela E. Matei, A. Lindsay Frazier, Jean A. Hurteau, and Farzana D. Pashankar36 Sex Cord-Stromal Tumors of the Ovary, 533Jubilee Brown, Isabelle Ray‐Coquard, and Kris Ann P. Schultz37 Clear Cell Carcinoma of the Ovary, 550Keiichi Fujiwara, Katsutoshi Oda, Masashi Takano, and Noriomi Matsumura38 Mucinous Carcinoma of the Ovary, 557Caroline C. Billingsley and David M. Gershenson39 Low‐Grade Serous Carcinoma of the Ovary, 564Amanda Nickles Fader and Anais Malpica40 Borderline Tumors of the Ovary, 572Erin K. Crane and Premal H. Thaker41 Fallopian Tube and Peritoneal Cancers, 582Destin Black, Kara Long Roche, and Ginger J. Gardner42 Nonendometrioid Endometrial Carcinomas and Uterine Sarcomas, 591Matthew A. Powell43 Tumors of the Cervix, 618Krishnansu S. Tewari and Bradley J. Monk44 Tumors of the Vulva and Vagina, 643Vance Broach and Mario M. Leitao Jr45 Gestational Trophoblastic Disease, 653John Lurain, Michael Seckl, and Julian Schink46 Targeted Therapy in Gynecological Malignancy, 663Behrouz Zand, Shannon N. Westin, and Robert L. ColemanSection 8 Endocrine Tumors47 Adrenal Neoplasms, 681Ashley A. Stewart and Ellen Shannon Story48 Uncommon Cancers of the Thyroid, 713Bhavana Konda and Manisha H. Shah49 Parathyroid Carcinoma, 730Colleen Veloski and John A. RidgeSection 9 Hematological Malignancies50 Rare Acute Leukemias, 740B. Douglas Smith and Jonathan Webster51 Unusual Myelodysplastic and Myelodysplastic/ Myeloproliferative Neoplasms, 752Eric Padron and Rami Komrokji52 Rare B Cell Lymphoproliferative Disorders, 760Andrew G. Evans and Paul M. Barr53 Uncommon Presentations of Plasma Cell Dyscrasias, 776Jason Brayer, Mohamad A. Hussein, and Rachid Baz54 Rare Bone Marrow Failure Conditions, 793Amy S. Duffield and Amy E. DeZern55 Myeloproliferative Neoplasms: Chronic Myelogenous Leukemia, Polycythemia Vera, Essential Thrombocythemia, and Primary Myelofibrosis, 816Raoul Tibes and Ruben A. Mesa56 Rare T Cell Lymphomas, 830Aleksandr Lazaryan and John SweetenhamSection 10 Neurological Tumors57 Primary Melanotic Tumors of the Central Nervous System, 840L. Burt Nabors, Rong Li, Cheryl A. Palmer, and Philip R. Chapman58 Comprehensive Management of Chordoma, 847Varun R. Kshettry, Jennifer S. Yu, Vyshak A. Venur, Manmeet S. Ahluwalia, and Pablo F. Recinos59 Atypical and Malignant Meningiomas, 856Brandyn A. Castro, Melissa M. J. Chua, and Priscilla K. Brastianos60 Primary Central Nervous System Lymphoma, 871Macarena I. de la Fuente and Lisa M. DeAngelis61 Choroid Plexus Tumors, 883Jonathan Aicardi and Nicholas A. Butowski62 Diffuse Low‐Grade Gliomas, 888Nancy Ann Oberheim Bush, Daniel Yang, Jennifer Clarke, and Daphne Haas‐Kogan63 High‐Grade Gliomas, 899Ashley L. Sumrall64 Ependymomas, 907Roberta Rudà, Mark R. Gilbert, and Riccardo Soffietti65 Medulloblastoma in Adults, 917Anna M. Kenney, Robert C. Castellino, Paul L. Moots, Christopher D. Corso, and Minesh P. Mehta66 Pineal Parenchymal Tumors, 939Yosuke Kitagawa, Patrick Y. Wen, and Shota Tanaka67 Craniopharyngiomas, 949Danilo Silva, Mayur Sharma, Gazanfar Rahmathulla, and Gene H. Barnett68 Ophthalmic Cancers, 964Maria M. Choudhary and Arun D. SinghSection 11 Cutaneous Malignancies69 Unusual Cutaneous Malignancies, 976Charles T. Darragh, Lee E. Wheless, William G. Stebbins, Jeffrey P. Zwerner, Anthony J. Crimaldi, Thomas Olencki, and Laura Y. McGirt70 Dermatofibrosarcoma Protuberans, 994Carrie Luu, Jane L. Messina, Andrew S. Brohl, and Vernon K. Sondak71 Unusual Melanomas, 1002Morganna Freeman, Richard D. Carvajal, Mark Faries, and Omid HamidSection 12 Musculoskeletal Tumors72 Uncommon Tumors of Soft Tissue, 1022William Ahrens, Michael Livingston, Robert Lopez, Anthony J. Crimaldi, and Jeffrey KneislSection 13 Pediatric Cancers73 Introduction to Rare Pediatric Malignancies, 1069Alberto S. Pappo and Gregory H. Reaman74 Uncommon Tumors of the Gastrointestinal Tract in Children, 1072Alberto S. Pappo and Wayne L. Furman75 Rare Pediatric Malignancies of the Head and Neck, 1088Cynthia E. Herzog, Michael E. Kupferman, Winston Huh, and Anita Mahajan76 Uncommon Pediatric Tumors of the Thorax, 1097Yoav H. Messinger, Kris Ann P. Schultz, and Louis P. Dehner77 Uncommon Adrenal Tumors in Children and Adolescents, 1126Raul C. Ribeiro, Carlos Rodriguez‐Galindo, Emilia M. Pinto, Gerard P. Zambetti, and Bonald C. Figueiredo78 Uncommon Pediatric Genitourinary Tumors, 1138Hans Pohl and Jeffrey S. Dome79 Uncommon Pediatric Brain Tumors, 1152Robert C. Castellino, Matthew Schniederjan, and Tobey J. MacDonald80 Malignant Tumors of the Skin and Subcutaneous Tissue in Children, 1172Alberto S. Pappo and Carlos Rodriguez‐Galindo81 Rare Hematological Malignancies in Children, 1190Abby M. Green and Stephen P. HungerIndex, 1203