The American Cancer Society’s Oncology in Practice Clinical Management The American Cancer Society is a global grassroots force of nearly million volunteers dedicated to saving lives, celebrating lives, and leading the fight for a world without cancer From breakthrough research, to free lodging near treatment, a live helpline, free rides to treatment, and convening powerful activists to create awareness and impact, the Society is the only organization attacking cancer from every angle For more information go to www.cancer.org The American Cancer Society’s Oncology in Practice Clinical Management Edited by The American Cancer Society Atlanta, Georgia, USA This edition first published 2018 © 2018 The American Cancer Society All rights reserved No part of this publication may be reproduced, stored in a retrieval system, or transmitted, in any form or by any means, electronic, mechanical, photocopying, recording or otherwise, except as permitted by law Advice on how to obtain permission to 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American Cancer Society, editor Title: The American Cancer Society’s Oncology in Practice : clinical management / edited by American Cancer Society Other titles: American Cancer Society textbook of clinical oncology (2018) | Textbook of clinical oncology Description: Hoboken, NJ : Wiley, 2018 | Includes bibliographical references and index | Identifiers: LCCN 2017027177 (print) | LCCN 2017028088 (ebook) | ISBN 9781118592076 (pdf ) | ISBN 9781118591963 (epub) | ISBN 9781118517642 (cloth) Subjects: | MESH: Neoplasms Classification: LCC RC263 (ebook) | LCC RC263 (print) | NLM QZ 200 | DDC 616.99/4–dc23 LC record available at https://lccn.loc.gov/2017027177 Cover design by Wiley Cover image: © Tendo/Shutterstock Set in 9.5/11.5 pt Warnock by SPi Global, Pondicherry, India 10 9 8 7 6 5 4 3 2 1 v Contents Editorial Board and Reviewers ix List of Contributors x Introduction xviii Section | Thoracic Cancers 1 Lung Cancer Suresh S Ramalingam and Fadlo R Khuri Other Thoracic Malignancies 22 Brandon H Tieu, Mehee Choi, Kyle Robinson, and Charles R Thomas, Jr Section | Digestive System Cancers 33 Esophageal Cancer 35 Ravi Shridhar, Khaldoun Almhanna, Sarah E Hoffe, Matthew Biagioli, Domenico Coppola, and Kenneth L. Meredith Gastric Adenocarcinoma 54 Roger H Kim, Quyen D Chu, and Benjamin D Li Small Bowel Cancer (Excluding Gastrointestinal Stromal Tumors and Carcinoid) 69 Alireza Hamidian Jahromi, Roger H Kim, Quyen D Chu, and Benjamin D Li Adenocarcinoma of the Pancreas 82 Quyen D Chu, Erkut Borazanci, Roger H Kim, and Guillermo Sangster Liver Cancers 100 Celina Ang, Sonia Reichert, and Randall F Holcombe Biliary Tract Cancers/Cholangiocarcinomas 117 Celina Ang, Sonia Reichert, and Randall F Holcombe Colon and Rectal Cancer 124 Lauren Kosinski, Ben George, Kiran K Turaga, Candice A Johnstone, and Mohammad Mahmoud 10 Anal Cancer 149 Cathy Eng, Ravi Shridhar, Emily Chan, Nataly Silva, Liana Tsikitis, Susan Hedlund, Michael D Chuong, and Charles R Thomas, Jr 11 Gastrointestinal Stromal Tumors 163 Rian M Hasson Charles, Stanley W Ashley, and Chandrajit P Raut vi Contents Section | Head and Neck 175 12 Oral Cavity and Oropharyngeal Cancer 177 Avinash V Mantravadi and Michael G Moore 13 Salivary Gland Cancer 192 Daniel Brickman and Neil D Gross 14 Larynx Cancer 201 Emma B Holliday, Blaine D Smith, Neil D Gross, Clifton D Fuller, and David I Rosenthal 15 Nasal and Paranasal Sinus Cancer 211 Emma B Holliday, Michael E Kupferman, Clifton D Fuller, and Ehab Hanna 16 Nasopharyngeal Cancer 218 Jamie M Pawlowski, Emma B Holliday, and Clifton D Fuller Section | Urinary System 225 17 Renal Cell Carcinoma 227 Jonathan Mathias and Brian Rini 18 Bladder Cancer and Other Urothelial Sites 237 Michael C Risk, Ayman Soubra, and Badrinath R Konety Section | Female Reproductive Cancer 251 19 Ovarian, Fallopian Tube, and Primary Peritoneal Cancer 253 Michael L Pearl, Erin E Stevens, and Joyce Varughese 20 Uterine Corpus Cancer 268 Mario Javier Pineda and John R Lurain 21 Cervical Cancer 288 Merieme Klobocista, Mark H Einstein, and Carolyn D Runowicz 22 Vaginal Cancer 304 Christina Gauthreaux, Anna Kuan‐Celarier, and Carolyn D Runowicz 23 Vulvar Cancer 310 Carolyn D Runowicz 24 Gestational Trophoblastic Disease 318 Alok Pant and John R Lurain Section | Male Reproductive Cancers 329 25 Testicular Cancer 331 Costantine Albany, Nasser Hanna, and Lawrence H Einhorn 26 Prostate Cancer 344 Bobby C Liaw and William K Oh 27 Penile Cancer 363 David Bowes and Juanita Crook Contents Section | Breast Cancer 375 28 Breast Cancer, Including Brief Discussion of Male Breast Cancer 377 Elisavet Paplomata and Ruth O’Regan Section | Hematologic Cancers 397 29 Myeloid Malignancies 399 Joshua F Zeidner, Darshan Roy, Alexander Perl, and Ivana Gojo 30 Lymphoid Leukemias in Adults 422 Nilanjan Ghosh, Jocelyn L Wozney, and Michael R Grunwald 31 Hodgkin Lymphoma in Adults 434 Satish Shanbhag and Richard Ambinder 32 Non‐Hodgkin Lymphoma in Adults 444 Loretta J Nastoupil, Jean L Koff, Leon Bernal‐Mizrachi, and Christopher R Flowers 33 Multiple Myeloma 463 Giada Bianchi and Kenneth C Anderson Section | Skin Cancer 485 34 Melanoma 487 Justin M Ko, Alan C Geller, and Susan M Swetter 35 Non‐Melanoma Skin Cancers 502 H William Higgins, II and Martin A Weinstock Section 10 | Endocrine Cancers 519 36 Thyroid Cancer 521 Maria E Cabanillas, Steven P Weitzman, Ramona Dadu, Ted Gansler, and Mark Zafereo 37 Adrenal Cortical Carcinoma and Pheochromocytoma 532 Robert Dreicer, Moshe C Ornstein, Kriti Mittal, Jordan Reynolds, Joseph Klink, Christopher Przybycin, and Jorge A Garcia 38 Pituitary Tumors 542 Adriana G Ioachimescu and Nelson M Oyesiku 39 Gastroenteropancreatic Neuroendocrine Tumors 552 Jonathan Strosberg Section 11 | Cancer of the Nervous System and Eye 571 40 Central Nervous System and Peripheral Nerves 573 D Ryan Ormond, Alexandros Bouras, Michael Moore, Matthew Gary, Paula Province Warren, Roshan Prabhu, Kathleen M Egan, Srikant Rangaraju, Christina Appin, Constantinos Hadjipanayis, Burt Nabors, Alfredo Voloschin, and Jeffrey J Olson 41 Malignant Tumors of the Eye 608 Devron H Char and Tia B Cole vii viii Contents Section 12 | Bone and Soft Tissue Tumors 617 42 Sarcomas of Bone in Adults 619 Mrinal Gounder, Yoshiya Yamada, and Nicola Fabbri 43 Sarcoma of Soft Tissue 631 Mrinal Gounder, Vinod Ravi, Yoshiya Yamada, Richard Carvajal, and Aimee Crago Section 13 | Cancer of Unknown Primary, Paraneoplastic Syndromes, and Peritoneal Carcinomatosis 645 44 Cancer of Unknown Primary Site 647 John D Hainsworth and F Anthony Greco 45 Paraneoplastic Syndromes 661 Lorraine C Pelosof and David E Gerber 46 Peritoneal Surface Malignancies 675 Kiran K Turaga Section 14 | Pediatric and Adolescent Oncology 683 47 Pediatric Cancers 685 Stephanie B Dixon, Lisa M Force, Pratiti Bandopadhayay, Peter Manley, Carlos Rodriguez‐Galindo, Lewis B. Silverman, and Karen J Marcus Index 729 742 Index k K‐RAS mutations lung cancer pancreatic cancer 83 Kaposi sarcoma (KS) 514–515 conjunctival 609 keratoacanthoma (KAC) 508–509 clinical features 509, 509 diagnosis 509 incidence 508 pathology 509 risk factors 508 subtypes 509 treatment 509 ketoconazole, Cushing disease 549 KIT mutations acute myeloid leukemia 404 gastrointestinal stromal tumors 163–165, 170, 639 melanoma 489 testicular cancer 331 Klatskin tumors 117 Klinefelter syndrome 331 l Lambert–Eaton myasthenic syndrome (LEMS) 664, 665, 668 lanreotide, neuroendocrine tumors 559–560 antiproliferative effect 560 combined therapy 560–561 laparoscopic treatment adrenal cancer 536 colon cancer 130 endometrial carcinoma 275–276 gastric cancer 60 gastrointestinal stromal tumors 168 see also diagnostic laparoscopy laparoscopic‐assisted digital gastrectomy (LADG) 60 lapatinib, breast cancer 387, 390–391 large cell carcinoma, lung laryngectomy 203–207 larynx cancer 201–208, 203 diagnosis 202 follow‐up 207–208 incidence 201 metastatic disease 202 mortality 201 pathology 202 prevention 201–202 risk factors 201 staging 202, 204–205 survivorship 207–208 treatment 202–207 advanced cancer 206–207 early stage glottic cancer 203–205 early stage subglottic cancer 206 early stage supraglottic cancer 206 laser surgery eye malignancies 612 larynx cancer 206 oropharyngeal cancer 188 vulvar intraepithelial neoplasia 313 leiomyosarcoma 280, 281–282, 638–639 see also soft tissue sarcoma lenalidomide mantle cell lymphoma 454 multiple myeloma 470, 473, 474–475 maintenance therapy 476 myelodysplastic syndromes 402 lentigo maligna melanoma 489, 490, 491, 491 lenvatinib, thyroid cancer 528, 529 leptomeningeal disease (LMD) 597–598 clinical features 597 diagnosis 598, 598 treatment 598 letrozole, breast cancer 387–388, 389 leukemia see acute myeloid leukemia (AML); chronic myeloid leukemia (CML); lymphoid leukemias leukocytoclastic vasculitis 667, 669 leukoplakia 180 levonorgestrel intrauterine device, endometrial hyperplasia management 269 Leydig cell tumors (LCTs) ovarian 264 testicular 334 lichen planus 180 limbic encephalitis (LE) 665 lipiodol, hepatocellular carcinoma 107 liposarcoma 631, 639 subtypes 632 see also soft tissue sarcoma lips 178 see also oral cavity/oropharyngeal cancer liver cancer see hepatocellular carcinoma (HCC); intrahepatic bile duct cancer liver metastases 138 chemotherapy 139 metastasectomy 139–140 liver transplantation cholangiocarcinoma 119 hepatocellular carcinoma 106 Milan criteria 106 metastatic neuroendocrine tumors 563 Li–Fraumeni syndrome 578, 632, 712, 714 lobectomy lung cancer 10 thyroid cancer 524 lumbar puncture, pediatric ALL 686 lung cancer 3–17 adenocarcinoma 4, diagnosis 5–6 elderly patients 15–16 follow‐up 17 incidence 3 metastases 6 radiation therapy 11 mortality 3 nonsmall cell (NSCLC) 4, 11–16 palliative therapy 11, 16–17 paraneoplastic syndromes pathology 4–5 molecular 5, risk factors 3–4 screening 6 small cell (SCLC) 4–5, 16–17 squamous cell carcinoma 4, staging 6, 7–8 lymph node map 9 survivorship 17 treatment 8 maintenance therapy 12–13, 14 radiation therapy 10–11 salvage therapy 13, 13, 17 surgery 9–10, 17 systemic therapy 11–17, 11 targeted therapy 13–16, 14 lymph nodes gastric 58, 59, 59 lung cancer staging management see specific types of cancer neck 205 see also sentinel lymph node biopsy lymphadenectomy bladder cancer 243 cervical cancer 293, 295, 297 colorectal cancer 129–130 gastric cancer 58–60, 60 gastrointestinal stromal tumors 168 Index Merkel cell carcinoma 512 pancreatic cancer 90 penile cancer 365–366, 370 renal cell carcinoma 230 testicular cancer 336, 338 thyroid cancer 527 urothelial carcinoma 245 vaginal cancer 307–308 vulvar cancer 315 see also neck dissection lymphedema, chronic, soft tissue sarcoma association 631–632 lymphoblastic lymphoma 694 lymphoid leukemias 422–429 acute lymphoblastic (ALL) 422–424 see also specific types lymphoma Burkitt 78, 442, 454 colorectal 124 diffuse large B‐cell (DLBCL) 78, 454–456 allogeneic stem cell transplantation 456 epidemiology 454–455 first‐line treatment 455 pathophysiology 455 second‐line therapy 455–456 intraocular 614, 614 mantle cell (MCL) 453–454 marginal zone (MZL) 452–453 gastric 452–453 nodal 453 nongastric 453 splenic 453 peripheral T‐cell (PTCL) 456 salivary gland 195 small bowel 69–70 pathology 71 staging 75, 76 treatment 78 small lymphocytic (SLL) 452 see also follicular lymphoma; Hodgkin lymphoma; non‐Hodgkin lymphoma (NHL); primary CNS lymphoma (PCNSL) Lynch syndrome 71, 125, 245 diagnostic criteria 125 endometrial carcinoma association 269–270 m magnetic resonance imaging (MRI) adrenal cancer 535 anal cancer 150 astrocytoma 579–580 functional imaging 581, 581 bladder cancer 239–240 bone sarcomas 620 breast cancer 379, 381 cholangiocarcinoma 118 gastrointestinal stromal tumors 164, 165 glioma 713 hepatocellular carcinoma 102 lung cancer nasopharyngeal cancer 219 neuroendocrine tumors 557 oral cavity/oropharyngeal cancers 182, 182 pancreatic cancer 85 penile cancer 365 renal cell carcinoma 228 salivary gland cancer 195 sinonasal cancer 212, 212 small bowel cancer 73 soft tissue sarcoma 633 spinal tumors 591 magnetic resonance spectroscopy (MRS), astrocytoma 580–581 male breast cancer 391 malignant bowel obstruction 679 malignant peripheral nerve sheath tumor (MPNST) 587–588 malignant peritoneal mesothelioma 675 malignant pleural mesothelioma (MPM) 25–28 diagnosis 26 follow‐up 28 incidence 25 pathology 25–26 risk factors 25 staging 26, 27 treatment 26–28 chemotherapy 28 radiation therapy 26–28 surgery 26 malnutrition, colorectal cancer 142 mammalian target of rapamycin (mTOR) inhibitors hepatocellular carcinoma 110 mantle cell lymphoma 454 neuroendocrine tumors 561 renal cell carcinoma 231–232 MammaPrint® prognostic score 384 mammography 379 mandibular invasion 186 mantle cell lymphoma (MCL) 453–454 allogeneic stem cell transplantation 454 first‐line treatment 453–454 relapsed disease 454 MAP kinase pathway, hepatocellular carcinoma 110 marginal zone lymphoma (MZL) 452–453 gastric 452–453 nodal 453 nongastric 453 Masaoka‐Koga staging, thymic malignancies 23, 23 mastectomy 384–385 prophylactic 378 mastocytosis 413 maxillofacial prosthodontics 187 MC1R variants, melanoma association 488 medulloblastoma 574, 586–587, 714–716 classification 714–715 diagnosis 715 etiology and risk factors 714 follow‐up and late effects 716 incidence 714 presentation 715 staging 715, 715 treatment 715–716 megestrol acetate, endometrial hyperplasia 269 MEK inhibitor therapy, melanoma 497 melanoma 487–498 choroidal 611, 612 conjunctival 609 diagnosis 492, 492 follow‐up 497–498 incidence 487 iris 613, 613 mortality 487 pathology 489–491 histopathology 491 signaling pathways 489 subtypes 489–490, 490, 490, 491 prognostic factors 487–488 clinical factors 488 melanoma subtype 488 tumor thickness 487 risk factors 487, 488–489 staging 492–493 survivorship 498 treatment 493–497 adjuvant systemic therapy 494–495 743 744 Index melanoma (cont’d ) advanced disease 495–497, 495 chemotherapy 495, 496 immunomodulatory drugs 496 immunotherapy 494–495, 496–497 metastatic disease 494 surgery 493–494 uveal 611–612, 612, 613 vaccines 495 vaginal 306, 306 vulvar 312 treatment 315 melphalan, multiple myeloma 474, 475 meningioma 574, 589 Simpson grading scale 592, 592 spinal 592 Merkel cell carcinoma (MCC) 509–513 clinical features 510 diagnosis 510 incidence 509–510 metastatic disease 510 mortality 510 pathology 510 recurrence 512–513 risk factors 510 staging 510, 511–512 treatment 510–513 mesothelioma see malignant peritoneal mesothelioma; malignant pleural mesothelioma (MPM) metastases bone 11 brain 11, 594–596 clinical features 594–595 diagnosis 595 prophylactic cranial irradiation 17 treatment 595–596, 596 choroidal 613–614 liver 138, 140 chemotherapy 139 metastasectomy 139–140 peritoneal 675–677 pulmonary 140 salivary gland 195 spinal 593–594 clinical findings 593 diagnosis 593–594 treatment 594 metastasizing mixed tumors 194 metastatic disease adrenal cancer 536 anal cancer 150, 154–157 inguinal lymph node management 154 breast cancer 389–391, 652–653 HER2‐negative 391 HER2‐positive 390–391 HR‐positive 389–390 cervical cancer 290, 293, 295, 297 cholangiocarcinoma 120 colorectal cancer 137–138 liver metastases 138, 139–140, 140 palliative and end‐of‐life care 142 treatment planning 139–140, 140 endometrial cancer 273–274, 278–279 patterns of metastatic dissemination 276 gastrointestinal stromal tumors 164, 170–171 gestational trophoblastic disease 322 intracranial 594–596 larynx cancer 202 lung cancer radiation therapy 11 melanoma 494 Merkel cell carcinoma 510 nasopharyngeal cancer 221 neuroblastoma 698–699 neuroendocrine tumors 553, 555, 563 oral cavity/oropharyngeal cancer 179 osteosarcoma 696 pancreatic cancer 92–94 penile cancer 370–371 prostate cancer 352–358 renal cell carcinoma 230 retinoblastoma 711 sinonasal cancer 211–212 soft tissue sarcoma 634, 638–639 testicular cancer 332, 336–338 thyroid cancer 528, 529 vulvar cancer 312 methotrexate acute lymphoblastic leukemia 423 breast cancer 385 gestational trophoblastic disease 322–324 intraocular lymphoma 614 osteosarcoma 626 primary CNS lymphoma 597 microcystins 101 microRNAs adrenal cancer 533 non‐Hodgkin lymphoma 447 microsatellite instability, colorectal cancer 126 microwave ablation, hepatocellular carcinoma 107 midostaurin, acute myeloid leukemia 407 Milan criteria for liver transplantation 106 minimal residual disease (MRD) testing, pediatric ALL 686 minimally invasive adenocarcinoma (MIA) 4 mitomycin C (MMC) anal cancer 150–153 bladder cancer 241 eye malignancies 608 mitotane adrenal cancer 536–537 Cushing disease 549 mitoxantrone, prostate cancer 353, 354, 355 Model for End State Liver Disease (MELD) score 106 Mohs micrographic surgery melanoma 493 penile cancer 367 skin cancer (non‐melanoma) 504, 508, 509, 510, 513 molar pregnancy see hydatidiform mole monoclonal antibodies, multiple myeloma 473, 474 monoclonal B‐cell lymphocytosis (MBL) 425 monoclonal gammopathy of undetermined significance (MGUS) 463–464, 467 mucinous tumors appendiceal 676 endometrial carcinoma 270–271 ovarian 255–256, 257 mucoepidermoid carcinoma (MEC) 193 mucosal melanoma (MM) 212 mucosal‐associated lymphoid tissue (MALT) 78, 452–453 multidetector computed tomography (MDCT) hepatocellular carcinoma 102 pancreatic cancer 84–85, 84 multiple endocrine neoplasia (MEN) 522, 528, 555–556 adrenal adenoma and 543 multiple myeloma (MM) 463–478 diagnosis 464, 466, 467–469, 468 Index genetics 465, 465 histopathology 464–465 incidence 463 mortality 463 pathogenesis 463–464, 472 presentation 465–467, 466 prevention 463 risk factors 463 staging 469, 469 survivorship 477–478 treatment 469–478, 471 allogeneic stem cell transplantation 476 ancillary therapy 477 autologous stem cell transplantation 475–476 histone deacetylase inhibitors 473, 474 immunomodulatory drugs 472, 473 maintenance therapy 476 monoclonal antibodies 473, 474 non‐transplant candidates 474–475 proteasome inhibitors 470–474, 473 recurrent disease 476–477 transplant candidates 475–476 MUTYH‐associated polyposis 70 myasthenia gravis (MG) 22, 665, 668 Myc mutations, non‐Hodgkin lymphoma 447, 454 MYCN amplification medulloblastoma 715 neuroblastoma 699, 700 myelodysplastic syndromes (MDS) 399, 400–403 classification 400–401, 401, 405 etiology 400 genetics 400 incidence 400 pathogenesis 400 pediatric 689 presentation 400 risk stratification/prognosis 401, 402 therapy 401–403 allogeneic stem cell transplantation 403 combination chemotherapy 403 hypomethylating agents 402–403 immunosuppression 402 supportive care 401–402 myelofibrosis 415 primary (PMF) 412, 413 therapy 415 myeloid malignancies 399–415 myeloproliferative neoplasms (MPN) 399, 410–413 classification 413 epidemiology 412–413 genetics 413 pathogenesis 413 presentation 412–413 therapy 414–415, 414 see also chronic myeloid leukemia (CML) myoepithelial carcinoma 194 n N‐acetyltransferase (NAT2) 237 nasal cavity cancer see sinonasal cancer nasopharyngeal cancer (NPC) 218–221 diagnosis 219 follow‐up 221 incidence 218 metastatic disease 221 mortality 218 pathology 218 prevention 218 risk factors 218 staging 219–220, 219 treatment 220–221 NCCN staging, pancreatic cancer 86, 87 necitumumab, lung cancer 14–15 neck dissection larynx cancer 207 oral cavity/oropharyngeal cancer 186–188 salivary gland cancer 198 sinonasal cancer 214 thyroid cancer 528 necrolytic migratory erythema (NME) 555 nelarabine, acute lymphoblastic leukemia 424 Nelson syndrome 549 neorectum construction 132, 135 nephrectomy 229–230 partial 229 nephroblastoma see Wilm’s tumor (WT) nephroureterectomy 245 neuroblastoma 698–701 diagnosis 699 follow‐up and late effects 701 incidence 698 presentation 698–699 staging 699–700, 700 treatment 700–701 neuroendocrine cancer of unknown primary site 648, 651, 655 neuroendocrine tumors (NETs), gastroenteropancreatic 124, 552–564 biology 555–556 classification 552–553 clinical features 553–555 appendiceal tumors 554 colorectal tumors 554 gastroduodenal tumors 553–554 midgut tumors 553 nonfunctioning pancreatic NETs 555 pancreatic tumors 554–555 diagnosis 556–558 imaging 557–558 immunohistochemical markers 556 serum and urine markers 556–557 genetic syndromes 555–556 incidence 555 metastatic disease 553, 555, 563 prognosis 564 staging 553 treatment 558–563 localized tumors 558–559 systemic therapy 559–563 targeted therapies 562–563 neurofibroma 587 spinal 593 neurofibromatosis (NF) type and 2 578, 587 soft tissue sarcoma association 632, 712 nevoid basal cell carcinoma syndrome 502 NF‐κB activation, non‐Hodgkin lymphoma 447 nilotinib, chronic myeloid leukemia 411, 412 nivolumab adrenal cancer 538 hepatocellular carcinoma 110 Hodgkin lymphoma 439 lung cancer 13, 13, 15 melanoma 496–497 renal cell carcinoma 231 745 746 Index nodal marginal zone lymphoma 453–454 nodular melanoma 489, 490, 490 non‐Hodgkin lymphoma (NHL) 70, 444–456 allogeneic stem cell transplantation 452–456 B‐cell 445–447, 449–456 cells of origin 447, 448 classification 444–445, 445, 692, 693 diagnosis 448–449, 693 epigenetic changes 446–447 follow‐up 456 genetics 446–447 affected signaling pathways 447 incidence 444, 692 management by subtype 449–456 follicular lymphoma (FL) 449–452 mantle cell lymphoma 453–454 marginal zone lymphomas 452–453 pediatric 692–695 treatment 694 prognostic indices 450 staging 694, 694 survivorship 456 T‐cell 447–448, 456 cells of origin 448 see also primary CNS lymphoma (PCNSL) non‐melanoma skin cancers atypical fibroxanthoma (AFX) 513–514 basal cell carcinoma (BCC) 502–505 clinical features 504–505, 504 diagnosis 504 incidence 502 pathology 504 risk factors 502–503 treatment 504–505 dermatofibrosarcoma protuberans (DFSP) 513 Kaposi sarcoma (KS) 514–515 keratoacanthoma (KAC) 508–509, 509 melanoma see melanoma Merkel cell carcinoma (MCC) 509–513 squamous cell carcinoma (SSC) 505–508 clinical features 505–506, 505 diagnosis 506 incidence 505 pathology 508 risk factors 505 staging 506–508, 506–507 treatment 508 nonalcoholic fatty liver disease (NAFLD) 100 nongastric marginal zone lymphoma 453 nonsmall cell lung cancer (NSCLC) 4 chemotherapy 12–16 early stage disease 16 locally advanced disease 16 maintenance therapy 12–13 salvage therapy 13, 17 elderly patients 16 radiation therapy 10–11 surgical treatment 9–10, 17 see also lung cancer nonsteroidal anti‐inflammatory drugs (NSAIDs), Barret esophagus prevention 41 nuclear and cytoplasmic neuronal antigens (NCNA) 664 nucleophosmin‐1 (NPM1) mutations, acute myeloid leukemia 404 o obesity breast cancer association 377–378 ovarian cancer association 254 renal cell carcinoma association 227 obinituzumab chronic lymphocytic leukemia 427 follicular lymphoma 452 occupational exposure bladder cancer association 237 lung cancer association multiple myeloma association 463 salivary gland cancer association 192 sinonasal cancer association 211 soft tissue sarcoma association 631 OctreoScan® 557–558 octreotide, neuroendocrine tumors 559–560 antiproliferative effect 560 combined therapy 560–561 radiolabelled octreotide 562–563 ofatumumab, chronic lymphocytic leukemia 427 Okuda staging system, hepatocellular carcinoma 104 olaparib, ovarian cancer 262 oligodendroglioma 575, 584 omacetaxine, chronic myeloid leukemia 412 omeprazole 41 oncocytoma 228 Oncotype DX® assay 384 ophthalmic tumors see eye malignancies opsoclonus/myoclonus syndrome 699 oral cavity/oropharyngeal cancer 177–189 anatomy 178–179 lymphatic drainage 179, 180 oral cavity 178, 179 oropharynx 178–179, 180 biopsy 182 etiology 177–178 evaluation 181–182 imaging 182, 182 physical examination 181–182, 181 follow‐up 189 incidence 177 metastatic disease 179 mortality 177 pathogenesis 180–181 pathology 180 presentation 181 prevention 178 risk factors 177 staging 182–185, 183–186 survivorship 189 treatment 185–189 chemotherapy 189 radiation therapy 188–189 surgery 186–188, 187, 188 see also salivary gland cancer oral contraception, gestational trophoblastic disease 322 oral prosthodontics 187 orbital tumors 609–610 therapy 611 orchiectomy 332–336, 338 organ transplantation melanoma association 488 non‐melanoma skin cancer association 502, 505 ornithine decarboxylase (ODC) 543 oropharyngeal cancer see oral cavity/ oropharyngeal cancer orthotopic liver transplantation (OLT) cholangiocarcinoma 119 hepatocellular carcinoma 106 osimertinib, lung cancer 14 osteosarcoma 619, 620 diagnosis 696 Index etiology and risk factors 695 follow‐up and late effects 696–697 incidence 695 metastatic disease 696 pathology 695 pediatric 695–697 presentation 695–696 therapy 626–627, 696 see also bone sarcoma OVA‐1 blood test 254 ovarian cancer 253–260 imaging 255 incidence 253 mortality 253 palliative care 262 pathology 255–256 simultaneous endometrial and ovarian tumors 271 peritoneal metastases 677 post‐treatment surveillance 260–261, 260 presentation 254–255 recurrence 261–262, 261 risk factors 253–254 age 253 genetic factors 254 HRT 254 obesity 254 reproductive factors 253–254 screening 254 staging 255, 256 stromal tumors 254 treatment 256–260 chemotherapy 259–260, 261–262, 261 cytoreductive surgery 258–259, 260 maintenance therapy 260 neoadjuvant therapy 259–260 surgery 256–259 targeted therapies 260 ovarian germ cell tumors (OGCTs) 254, 262–264 classification 258, 262 late effects 263–264 pathology 262 persistence, progression or recurrence 263 presentation 262 recurrence 263 surveillance 263 treatment 262–263 chemotherapy 263–264 surgery 262–263 ovarian suppression, breast cancer 390 ovarian tumor classification 257–258 overlap MDS/MPN neoplasms 399, 413–414 oxaliplatin cervical cancer 295 colorectal cancer 137, 138 germ cell tumors 339–340 pancreatic cancer 94 p paclitaxel breast cancer 385, 386–387 cervical cancer 295 endometrial carcinoma 278, 280 lung cancer 12, 16 nab‐paclitaxel 12 metastatic anal cancer 157 ovarian cancer 259 maintenance therapy 260 penile cancer 370–371 testicular cancer 338, 339–340 Paget disease, extramammary 313 pain management lung cancer 11 pancreatic cancer 94 palbociclib, breast cancer 389, 390 palliative therapy cholangiocarcinoma 119, 120 colorectal cancer 142 gastric cancer 63 leptomeningeal disease 598 lung cancer 11, 16–17 ovarian cancer 262 pancreatic cancer 94–95 penile cancer 370–371 prostate cancer 357–358 renal cell carcinoma 232 small bowel cancer 78 pamidronate breast cancer metastases 391 multiple myeloma bone disease 477 pancreastatin 557 pancreatic adenocarcinoma 82–95 biology 83 clinical presentation 83–84 familial form 82–83 imaging 84–86, 84, 85 incidence 82 mortality 82 palliative care 94–95 risk factors 82–83 staging 86, 86, 87 surgical treatment 84 treatment 86–94 adjuvant therapy 87–89, 88 borderline resectable cancer 89–90, 90 localized and resectable cancer 87–89, 88 locally advanced cancer (LAPC) 91–92, 93, 94 metastatic disease 92–94 neoadjuvant therapy 89–92, 89 pancreatic enzyme replacement 95 pancreatic intraepithelial neoplasm (PanIN) 83 pancreatic neuroendocrine tumors 554–555 treatment 558 pancreaticoduodenectomy 91, 91, 92, 119 pancreaticojejunostomy 91 pancreatitis, hereditary 82 panitumumab, cholangiocarcinoma 120 panobinostat, multiple myeloma 473, 474 Pap smear test 289, 290 papillary urothelial neoplasm of low malignant potential (PUNLMP) 238, 242 paraganglioma 587 spinal 593 paranasal sinus cancer see sinonasal cancer paraneoplastic cerebellar degeneration (PCD) 665 paraneoplastic pemphigus (PNP) 667 paraneoplastic syndromes 661 constitutional 661 dermatologic and rheumatologic 667–668, 668 endocrine 662–664, 663 hematologic 668–669, 670 neurologic 664–668, 665–666 Paris staging system, gastrointestinal lymphoma 75, 76 Parkinson disease, melanoma association 488 parotid glands 195 see also salivary gland cancer parotidectomy 197–198 pazopanib gastrointestinal stromal tumors 170 ovarian cancer 260 renal cell carcinoma 231 soft tissue sarcoma 639 747 748 Index PC leukemia 467 pediatric cancers 685–716 acute lymphoblastic leukemia (ALL) 685–688 classification 686 CNS‐directed treatment 688 diagnosis 686 etiology and risk factors 685–686 follow‐up and late effects 688 incidence 685 prognostic factors 686–687 risk‐stratified therapy 687 treatment phases 687–688 acute myeloid leukemia (AML) 688–691 classification 690, 690 diagnosis 690 etiology and risk factors 689 follow‐up and late effects 691 incidence 689 presentation 689 treatment 690–691 Ewing sarcoma 697–698 classification 697 diagnosis 697–698 etiology and risk factors 697 follow‐up and late effects 698 incidence 697 presentation 697 treatment 698 germ cell tumors 706–708 diagnosis 707 etiology and risk factors 706 follow‐up and late effects 708 incidence 706 pathology 706 presentation 707 treatment 707–708 glioma 712–714 classification 712–713 diagnosis 713 etiology 712 follow‐up and late effects 714 incidence 712 presentation 713 treatment 713–714 Hodgkin lymphoma 691–692 classification 691 etiology and risk factors 691 follow‐up and late effects 692 incidence 691 presentation 691 treatment 691–692 medulloblastoma 714–716 classification 714–715 diagnosis 715 etiology and risk factors 714 follow‐up and late effects 716 incidence 714 presentation 715 staging 715, 715 treatment 715–716 neuroblastoma 698–701 diagnosis 699 follow‐up and late effects 701 incidence 698 presentation 698–699 staging 699–700, 700 treatment 700–701 non‐Hodgkin lymphoma 692–695 anaplastic large cell lymphoma 694–695 Burkitt lymphoma 693, 694 classification 692, 693 diffuse large B‐cell lymphoma 694 etiology and risk factors 692 follow‐up and late effects 695 incidence 692 lymphoblastic lymphoma 694 presentation and diagnosis 693 staging 693–694, 694 treatment 694–695 osteosarcoma 695–697 diagnosis 696 etiology and risk factors 695 follow‐up and late effects 696–697 incidence 695 pathology 695 presentation 695–696 treatment 696 retinoblastoma 708–712 etiology and risk factors 708–709 follow‐up and late effects 711–712 incidence 708 pathology 709 presentation and diagnosis 709 staging 709, 710 treatment 709–711 rhabdomyosarcoma 704–706 diagnosis 704–705 follow‐up and late effects 706 incidence 704 pathology 704 staging 705, 705, 706 treatment 706–707 Wilm’s tumor 701–704 associated syndromes 701–702, 702 classification 702 etiology and risk factors 701–702 follow‐up and late effects 703–704 incidence 701 presentation and diagnosis 702–703 staging 703, 703 treatment 703 pegvisomant, acromegaly 548 pembrolizumab cholangiocarcinoma 120 lung cancer 13, 13, 15 melanoma 496 pemetrexed lung cancer 12–13 maintenance therapy 12–13 salvage therapy 13 mesothelioma 28 see also chemotherapy penile cancer 363–371 diagnosis 364–365 etiology 363–364 follow‐up 371 histopathology 364 incidence 363 molecular pathology 364 mortality 363 prevention 364 quality of life impact 371 recurrence 371 staging 365–366, 365–366 treatment 366–371 early nonsuperficial lesions 366, 367–370 metastatic disease 370–371 palliative therapy 370–371 radiotherapy 367–370 superficial lesions 366–367 surgery 367 pentostatin, hairy cell leukemia 429 peptide receptor radiotherapy (PRRT), neuroendocrine tumors 562–563 percutaneous ethanol ablation, hepatocellular carcinoma 107 peretinoin, hepatocellular carcinoma 107 perihilar tumors 117 perineural tumor invasion 588 peripheral nerve tumors 587–588 Index peripheral T‐cell lymphoma (PTCL) 456 peritoneal cancer index (PCI) 676, 677, 679 peritoneal malignancies 675–679 diagnosis 677–678 malignant bowel obstruction 679 primary 675 carcinomatosis 653, 675 malignant mesothelioma 675 sarcomatosis 675 secondary 675–677 colorectal carcinomas 676 epithelial appendiceal neoplasms (EAN) 676 gastric cancer 676–677 ovarian cancer 677 treatment 678–679, 678 pertuzumab, breast cancer 390 petrosphenoidal syndrome of Jacod 219 Peutz‐Jegher’s syndrome 71 pharyngeal cancer see nasopharyngeal cancer (NPC); oral cavity/ oropharyngeal cancer pheochromoctyoma 532–538 diagnosis 534–535 imaging 535 immunohistochemistry 533 pathology 533 risk factors 532 staging 535 treatment 536–538 chemotherapy 537 surgery 536 targeted therapy 538 Philadelphia (Ph) chromosome 410, 423, 687 phimosis 363 phlebotomy, polycythemia vera 414 photocoagulation, retinoblastoma 709–710 photodynamic therapy (PDT), cholangiocarcinoma 119 pilocytic astrocytoma 584, 592, 712 pineal region tumors 586 pioglitazone exposure, bladder cancer association 238 pituitary apoplexy 544 pituitary gland 544 pituitary tumors 542–549 adenomas 542, 543 atypical 544 familial 543 carcinomas 542 classification 543 diagnosis 544–545 acromegaly 544–545 Cushing disease 545 nonfunctioning adenomas (NFPA) 544 prolactinomas 544 rare functioning tumors 545 incidence 542 incidentaloma 544 metastases 542 mortality 542 pathogenesis 543 pathology 543–544 risk factors 543 treatment 546–549 aggressive adenomas 549 radiation therapy 546–549 surgery 545–546, 547 placental site trophoblastic tumor (PSTT) 319, 320 treatment 325 see also gestational trophoblastic disease (GTD) plasma cells, multiple myeloma 463–464 plasma cell dyscrasia 468 plasmacytoma 467 platelet‐derived growth factor receptor (PDGFR) mutations CNS tumors 579 gastrointestinal stromal tumors 163–164, 165, 639 pleomorphic xanthoastrocytoma 584 pleural effusions lung cancer mesothelioma 26 pneumonectomy 10 pneumonitis, radiation‐related 11 POEMS syndrome 469 polycythemia vera (PV) 412 therapy 414 polymorphous adenocarcinoma 193 polymyalgia rheumatica (PMR) 668 polyps cervical 290 gastrointestinal 125 pomalidomide, multiple myeloma 470, 473 ponatinib acute lymphoblastic leukemia 424 chronic myeloid leukemia 412 portal vein embolization 119 portal vein resection 90 positron emission tomography (PET) adrenal cancer 535 anal cancer 150 astrocytoma 580 bladder cancer 240 bone sarcomas 620 breast cancer 381 cancer of unknown primary site 650, 651 cervical cancer 293 esophageal cancer 36, 43, 45 gastric cancer 57 gastrointestinal stromal tumors 164 Hodgkin lymphoma 436, 437, 691 lung cancer 6, 10 melanoma follow‐up 497–498 metastatic skin cancer (non‐melanoma) 510 multiple myeloma 465 nasopharyngeal cancer 219 neuroendocrine tumors 558 oral cavity/oropharyngeal cancers 182 pancreatic cancer 85, 85 peritoneal malignancies 677 small bowel cancer 73–74, 74 soft tissue sarcoma 633 testicular cancer 332 Predictor Analysis of Microarray (PAM) 50 384 pregnancy, cervical cancer 295–296 primary acquired melanosis (PAM) 609 primary CNS lymphoma (PCNSL) 596–597 diagnosis 597, 597 presentation 597 treatment 597 primary cutaneous neuroendocrine carcinoma see Merkel cell carcinoma (MCC) primary myelofibrosis (PMF) 412, 413 therapy 415 primary peritoneal cancer 253–264 presentation 254–255 staging 255, 256 treatment 256 chemotherapy 259 primitive neuroectodermal tumors (PNETs) 586, 640 proctectomy 130–131, 134 postproctectomy syndrome 142–143 749 750 Index progesterone receptor expression, breast cancer 379–380, 384 progestin therapy endometrial carcinoma 279 recurrent disease 279 endometrial hyperplasia 269 prolactinoma 542 diagnosis 544 treatment 546 prolymphocytic leukemia (PLL) 428–429 prophylactic cranial irradiation (PCI) 17 prostate cancer 344–358 diagnosis 346–347 incidence 344, 345 metastatic disease 352–358 chemohormonal therapy 352–353 metastatic disease prostate cancer bone‐directed therapy 357–358 chemotherapy 353–354, 355 immunotherapy 356–357, 357 radionuclide therapy 357, 357 secondary hormonal therapy 354–356 molecular markers 345 mortality 344 recurrence 351 risk factors 344–346 risk stratification 349 screening 346, 346 staging 347–348, 348 prognostic stage groups 349 subtypes 347, 347 treatment 348–358 active surveillance 348–349 androgen deprivation therapy (ADT) 349, 350–351, 352 radiation therapy 349–350 radical prostatectomy 349–351 salvage therapy 351–352 prostate‐specific antigen (PSA) 344, 346, 348, 351 cancer of unknown primary site 649, 653 prostatectomy 349–351 prostatic urothelial carcinoma 245–246 proteasome inhibitors, multiple myeloma 470–474, 473 pseudomyxoma peritonei 256 pulmonary metastases 140 pure red cell aplasia (PRCA) 669, 670 pylorus‐sparing Whipple procedure 91, 92 q 5q‐syndrome 400 quizartinib, acute myeloid leukemia 407 r radiation exposure see ionizing radiation exposure radiation therapy acromegaly 548 anal cancer 150–153, 157–158 inguinal lymph node management 154 intensity‐modulated therapy (IMRT) 154, 155, 156 techniques 157–158 treatment breaks 158 astrocytoma 581–582 bone sarcoma 624–626 pediatric patients 696 radiotherapy as cause 619 brain metastases 11, 595, 596 prophylactic cranial irradiation 17 breast cancer 385 cervical cancer 296 cholangiocarcinoma 120 CNS tumors 577 colorectal cancer 132–134, 135 external beam RT 133 particle RT 134 diffuse large B‐cell lymphoma 455 endometrial carcinoma 276 postoperative 276–277 recurrent disease 279–280 esophageal cancer 43 Ewing sarcoma 698 eye malignancies 609, 612, 614 follicular lymphoma 451, 452 glioblastoma 582, 583 gliomas 713–714 hepatocellular carcinoma 108 Hodgkin lymphoma 436, 437–438 long‐term complications 440–441 pediatric patients 692 larynx cancer 203–207, 207, 208 lung cancer 10–11 palliative 11 SCLC 17 marginal zone lymphomas 453 medulloblastoma 715 mesothelioma 26–28 nasopharyngeal cancer 220 oral cavity/oropharyngeal cancer 188–189 pancreatic cancer 87–89 penile cancer 367–370, 369 pituitary tumors 546–549 prostate cancer 349–350 renal cell carcinoma 232 retinoblastoma 710, 711 rhabdomyosarcoma 706 salivary gland cancer 197 selective internal radiotherapy (SIRT) 563 seminoma 334–336 sinonasal cancer 213, 214–215, 215 skin cancer (non‐melanoma) 508, 512, 513 soft tissue sarcoma 636 spinal metastases 594 thymic malignancies 24, 25 thyroid cancer 529 vaginal cancer 307–308 vulvar cancer 315 Wilm’s tumor 703, 704 see also brachytherapy; chemoradiotherapy radiation‐associated sarcomagenesis (RAS) 631 radioactive iodine ablation (RAI), thyroid cancer 527–528 radiofrequency ablation cholangiocarcinoma 119 hepatocellular carcinoma 107 liver metastases 138 renal cell carcinoma 229 radiologic studies, small bowel cancer 71 radionuclide therapy neuroendocrine tumors 562–563 prostate cancer 357, 357 radiosurgery see stereotactic radiosurgery (SRS) radium‐223 therapy, prostate cancer 357 radon exposure, lung cancer association 4 raloxifene, breast cancer risk reduction 378–379 ramucirumab esophageal cancer 49 gastric cancer 63 hepatocellular carcinoma 110 lung cancer 14 Index ranitidine 41 RECIST (Response Evaluation Criteria in Solid Tumors) guidelines 110 rectal cancer follow‐up and surveillance 141 radiation therapy 132–134, 135 external beam RT 133 particle RT 134 recurrence 141 squamous cell carcinoma 124 staging 129, 130 surgical treatment 130–132, 132, 133, 134 neorectum construction 132, 135 quality grading 132 see also colorectal cancer (CRC) rectal carcinoid tumors 554 red meat consumption 70 Reed–Sternberg cells 434, 691 regorafenib, gastrointestinal stromal tumors 170, 639 regorafenib, hepatocellular carcinoma 109 renal cell carcinoma 227–233 follow‐up 232–233 imaging 228, 229 incidence 227 inherited forms 228 metastatic disease 230 molecular pathology 228 mortality 227 palliative therapy 232 presentation 228 recurrence 230 risk factors 227–228 staging 228–229, 230 survivorship 233 treatment 229–232 immunotherapy 231 large tumors 229–230 localized disease 229 systemic therapy 230–231 targeted therapy 230, 231–232, 232, 233 renal cysts 228 RET mutations, thyroid cancer 522 retinoblastoma 708–712 etiology and risk factors 708–709 follow‐up and late effects 711–712 incidence 708 osteosarcoma association 695 pathology 709 presentation and diagnosis 709 staging 709, 710 treatment 709–711 bilateral retinoblastoma 710–711 chemotherapy 710, 711 CNS disease 711 extraocular retinoblastoma 711 focal therapies 709–710 metastatic retinoblastoma 711 radiotherapy 710 surgery 709 unilateral retinoblastoma 710 retromolar trigone 178, 180 see also oral cavity/oropharyngeal cancer retroparotid syndrome of Villaret 219 retroperitoneal lymph node dissection (RPLND) 336, 338 rhabdomyosarcoma 631, 640, 704–706 diagnosis 704–705 follow‐up and late effects 706 incidence 704 pathology 704 staging 705, 705, 706 subtypes 704 treatment 705–706 see also soft tissue sarcoma ribociclib 389 Richter transformation 428 ricolinostat, multiple myeloma 476 rituximab chronic lymphocytic leukemia 427 diffuse large B‐cell lymphoma 455 follicular lymphoma 451–452 maintenance therapy 452 hairy cell leukemia 429 Hodgkin lymphoma 440 intraocular lymphoma 614 mantle cell lymphoma 453–454 marginal zone lymphomas 453 robotic surgery colorectal cancer 130, 132 endometrial carcinoma 276 esophagectomy 42–43 oropharyngeal cancer 188 Rombo syndrome 503 Roux‐en‐Y reconstruction 57–58, 58 ruxolitinib, polycythemia vera 415 s sacral nerve stimulation 143, 159 salivary duct carcinoma 194 salivary gland cancer 192–198, 193 diagnosis 195–197 biopsy 197 examination 195 imaging 195–197 follow‐up 198 incidence 192 mortality 192 pathology 192–195 acinic cell carcinoma 193 adenocarcinoma 194–195 adenoid cystic carcinoma 193 basal cell adenocarcinoma 194 carcinoma ex pleomorphic adenoma 194 epithelial–myoepithelial carcinoma 194 lymphoma 195 metastasizing mixed tumors 194 mucoepidermoid carcinoma 193 myoepithelial carcinoma 194 polymorphous adenocarcinoma 193 salivary duct carcinoma 194 small cell carcinoma 194 squamous cell carcinoma 194 prognosis 198 risk factors 192 staging 196, 197 survivorship 198 treatment 197–198 sarcoma Kaposi (KS) 514–515 conjunctival 609 small cell 640 uterine 280–282 vaginal 305–306, 306 see also bone sarcoma; Ewing sarcoma; soft tissue sarcoma sarcoma botyroides 305, 306 sarcomatoid carcinoma, bladder 239 satraplatin, prostate cancer 354, 355 schwannoma 587 spinal 591–592, 591 screening actionable molecular alterations 656 anal cancer 149 breast cancer 379 cervical cancer 289 cholangiocarcinoma 118 colorectal cancer 126 endometrial carcinoma 269 esophageal cancer 41 hepatocellular carcinoma 101–102, 102 lung cancer ovarian cancer 254 prostate cancer 346, 346 small bowel cancer 71 751 752 Index seizures, brain tumor patients 577 selective estrogen receptor modulators (SERMs) 378 selective internal radiotherapy (SIRT) 563 seminoma 332–333 presentation 334 surveillance 334 treatment 334–336 sentinel lymph node biopsy melanoma 493–494 penile cancer 366 sinonasal cancer 214 skin cancer (non‐melanoma) 507, 508, 510 vulvar cancer 313 serotonin secretion, neuroendocrine tumors 553 diagnostic assays 556–557 serous tumors endometrial 271 ovarian 255, 257 salivary gland 192–193 Sertoli–Leydig cell tumors ovarian 264 testicular 334 sex cord–stromal tumors (SCSTs) ovarian 254, 258, 264 chemotherapy 264 presentation 264 surgery 264 testicular 333, 334 treatment 340 sexual dysfunction anal cancer survivors 158–159 colorectal cancer survivors 143 penile cancer survivors 371 Siewert classification, gastroesophageal junction cancers 36 Simpson–Golabi–Behmel syndrome 702 sinonasal cancer 211–215 diagnosis 212, 212 follow‐up 215 incidence 211 metastatic disease 211–212 mortality 211 pathology 211–212 risk factors 211 staging 212–213, 213 survivorship 215 treatment 213–215 neck management 214 primary tumor surgery 213–214, 214 radiation therapy 214–215, 215 systemic therapy 215 sipuleucel‐T, prostate cancer 356–357, 357 skin cancers see melanoma; non‐ melanoma skin cancers small bowel cancer 69–78 diagnosis 71 CT 71–74, 72, 73 endoscopy 74–75 enteroclysis 72–73 MRI 73 PET‐CT 73–74, 74 radiologic assessment 71 follow‐up 78 incidence 69 mortality 69 pathology 71 risk factors 69–71 inherited conditions 70–71 lifestyle factors 69–70 underlying conditions 70 screening 71 staging 75, 75, 76 survivorship 78 treatment 75–78 chemoradiation 76–78, 77 chemotherapy 76, 78 palliative therapy 78 surgery 76 small cell carcinoma bladder 247 cervical 290 salivary gland 194 small cell lung cancer (SCLC) 4–5 chemotherapy 16–17 radiation therapy 17 recurrence 16 see also lung cancer small cell sarcoma 640 small lymphocytic lymphoma (SLL) 452 small renal masses (SRMs) see renal cell carcinoma smoking bladder cancer association 237, 238 gastric cancer association 54 larynx cancer association 201 liver cancer association 101 lung cancer association 3, 17 cessation importance 17 oral cavity/oropharyngeal cancer association 177 pancreatic cancer association 82 penile cancer association 364 renal cell carcinoma association 227 small bowel cancer association 69 smooth muscle tumors of uncertain malignant potential (STUMP) 281 soft tissue sarcoma 631–640 diagnosis 632–633 etiology 631–632 genetic syndromes 632 incidence 631 metastatic disease 634, 638–639 mortality 631 natural history 632 pathology 633–634 presentation 632 recurrence 635, 638 staging 634, 634 treatment 634–638 chemotherapy 636–638, 636, 637 radiotherapy 636 surgery 634–635 solitary plasmacytoma 467 somatostatin analog (SSA) therapy, neuroendocrine tumors 559–560 antiproliferative effect 560 combined therapy 560–561 radiolabeled SSAs 562–563 somatostatin‐receptor scintigraphy 557–558 sonidegib, skin cancer 504 sorafenib 109 acute myeloid leukemia 407 hepatocellular carcinoma 107, 108–109 combination therapy 109 response evaluation 110 renal cell carcnoma 231 thyroid cancer 528 speech therapy 187 spinal cord compression 11, 574, 591, 593 spinal tumors 590–593 diagnosis 591 differential diagnosis 590 metastases 593–594 clinical findings 593 diagnosis 593–594 treatment 594 presentation 590–591 treatment 591 see also central nervous system (CNS) tumors; specific tumors splenectomy, myelofibrosis 415 splenic marginal zone lymphoma 453 Index squamous cell carcinoma (SCC) anal 150 bladder 246–247 cancer of unknown primary site 648, 650–651, 654–655 cervical 290 conjunctival 608 endometrial 271 esophageal 35–36 staging 36, 37–40 gastrointestinal 124 larynx 202, 203, 207, 207, 208 lung 4 molecular pathology oral cavity/oropharyngeal 177, 180, 182 penile 364, 366 salivary gland 194 sinonasal 211, 215 skin 505–508, 505 vaginal 305 vulvar 310, 311–312 stem cell transplantation see allogeneic stem cell transplantation (SCT); autologous stem cell transplantation (SCT) stereotactic body radiation therapy (SBRT), lung cancer 10–11 stereotactic needle biopsy, astrocytoma 580 stereotactic radiosurgery (SRS) brain metastases 11, 595–596 Cushing disease 548–549 pituitary tumors 546 Stewart–Treves syndrome 631–632 stomach cancer see gastric adenocarcinoma streptozocin adrenal cancer 537 neuroendocrine tumors 562 stromal tumors, endometrial 281 see also gastrointestinal stromal tumors (GISTs); sex cord– stromal tumors (SCSTs) subacute sensory neuropathy 666 subependymal giant cell astrocytoma 584, 712 sublingual glands 195 see also salivary gland cancer submandibular glands 195 see also salivary gland cancer subungual melanoma 491 sunitinib adrenal cancer 538 gastrointestinal stromal tumors 170, 639 hepatocellular carcinoma 110 neuroendocrine tumors 561 pheochromocytoma 538 renal cell carcinoma 231 thymic carcinoma 24 sunlamp use, melanoma association 488–489 superficial spreading melanoma 489, 490, 490, 491 survivorship, lung cancer 17 swallowing 178–179 therapy 187 see also dysphagia Sweet syndrome 668, 668, 669 synaptophysin 556 syndrome of inappropriate antidiuretic hormone (SIADH) 662, 663 syrinx 592 t T‐cell development 447–448 talimogene laherparepvec (T‐vec), melanoma 497 tamoxifen, breast cancer 387 metastatic disease 390 risk reduction 378–379 tanning bed use, melanoma association 488–489 temozolomide CNS tumors 577 astrocytoma 582, 583 glioblastoma 582 glioma 714 neuroendocrine tumors 462 pituitary adenoma 549 temsirolimus adrenal cancer 538 renal cell carcinoma 232 teratoma 262, 333, 334, 338 pediatric 707 testicular cancer 331–340 diagnosis 332 etiology 331–332 single nucleotide polymorphisms 331–332, 332 incidence 331 metastatic disease 336–338 metastatic patterns 332 prognostic system 337 mortality 331 pathology 332–333 presentation 333–334 recurrent disease 338–340, 338, 339 prognostic factors 339, 339 staging 333 treatment 334–340, 335 advanced disease 336–338 nonseminomatous germ cell tumors 336 postchemotherapy surgery 338 salvage therapy 338–340, 338 seminoma 334–336 testicular dysgenesis 331 thalidomide, multiple myeloma 470, 473, 475 maintenance therapy 476 thoracentesis 26 three‐dimensional conformal radiation therapy (3DCRT), esophageal cancer 47–49 thrombocytosis, paraneoplastic 669, 670 thrombophilia 668 thymectomy 24 thymic carcinoma 22–28 diagnosis 22–23 follow‐up 25 mortality 22 pathology 22 staging 23, 23 subtypes 22 treatment 24 chemotherapy 24 radiation therapy 24, 25 surgery 24 thymoma 22–28 diagnosis 22–23 follow‐up 25 incidence 22 mortality 22 pathology 22 recurrence 25 staging 23, 23 subtypes 22 treatment 24 chemotherapy 24 radiation therapy 24 surgery 24 thyroid cancer 521–529 classification 521–522 diagnosis 523–524, 524 fine needle aspiration 523–525 follow‐up 528, 529 incidence 521 molecular pathology 524 mortality 521 753 754 Index thyroid cancer (cont’d ) presentation 523 risk factors 522–523 demographic factors 522 heritable conditions 522 iodine deficiency 523 ionizing radiation 522–523 staging 524, 525–527 survivorship 528, 529 treatment 524–529 anaplastic thyroid cancer 529 differentiated thyroid cancer 524–528 medullary thyroid cancer 528–529 metastatic disease 528, 529 radioactive iodine ablation 527–528 refractory disease 528 surgery 524–527, 528 TSH suppression 528 thyroid stimulating hormone (TSH) suppression 528 TSHomas 545 thyroid transcription factor (TTF‐1) 4 tivantinib, hepatocellular carcinoma 109 TNM staging adrenal cancer 535 anal cancer 150, 151 bladder cancer 239–240, 240 bone sarcoma 621, 623 breast cancer 381–384, 382–383 cervical cancer 290, 291 colorectal cancer 127–128, 127–128 fallopian tube cancer 255, 256 gastric cancer 56–57, 56 gastrointestinal stromal tumors 165 hepatocellular carcinoma 104, 104 larynx cancer 202, 204–205 lung cancer 6, 7–8 mesothelioma 26, 27 nasopharyngeal cancer 219–220, 219 neuroendocrine tumors 553 oral cavity/oropharyngeal cancers 182–185, 183–185 ovarian cancer 256 pancreatic cancer 86, 86 penile cancer 365–366 pheochromocytoma 535 primary peritoneal cancer 255, 256 prostate cancer 347, 348 renal cell carcinoma 228–229, 230 salivary gland cancer 196, 197 sinonasal cancer 212–213, 213 skin cancer (non‐melanoma) 506–507, 511–512 small bowel cancer 75 soft tissue sarcoma 634, 634 testicular cancer 333 thymic malignancies 23 thyroid cancer 524, 525–527 vaginal cancer 307 vulvar cancer 312, 313 tobacco smoking see smoking tongue 178, 180 surgical treatment 186, 187 see also oral cavity/oropharyngeal cancer tonsillectomy 651 topotecan cervical cancer 295 lung cancer 17 toremifene, breast cancer 390 total mesorectal excision 130, 132 quality grading 132, 132 total parenteral nutrition (TPN), colorectal cancer patients 142 TP53 mutations breast cancer 380–381, 384 CNS tumors 579 ovarian cancer 255 skin cancer 504 vulvar cancer 312 trabectedin, soft tissue sarcoma 638–639 tracheostomy 205 trametinib lung cancer 15 melanoma 497 trans‐arterial chemoembolization 119–120 see also embolic therapies transarterial chemoembolization (TACE) hepatocellular carcinoma 107–108 drug‐eluting bead TACE 108 liver metastases 563 transarterial radioembolization (TARE), hepatocellular carcinoma 108 transient myeloproliferative disorder (TMD) 689 transpupillary thermotherapy, retinoblastoma 710 transurethral resection of bladder tumor (TURBT) 239, 241, 242, 244 transvaginal ultrasound (TVUS) endometrial carcinoma 270 ovarian cancer 254 trastuzumab breast cancer 386, 388 metastatic disease 390 trastuzamab‐DM1 391 esophageal cancer 49 gastric cancer 63 trauma, soft tissue sarcoma association 632 TSHoma 545 tuberous sclerosis 556, 578, 584, 712 tubular carcinoma, breast 379 tumor lysis syndrome 423, 424, 427, 428 tumor markers see biomarkers; specific markers Turcot syndrome 578, 712, 714 tyrosine kinase inhibitor (TKI) therapy acute lymphoblastic leukemia 423–424 acute myeloid leukemia 407 adrenal cancer 538 chronic lymphocytic leukemia 427 chronic myeloid leukemia 411, 412 response monitoring 411 gastrointestinal stromal tumors 166, 170 hepatocellular carcinoma 109 lung cancer 5, 14 neuroendocrine tumors 561 renal cell carcinoma 231 thyroid cancer 528 u ulcerative colitis 125 ultrasound breast cancer 379 endometrial carcinoma 270 gestational trophoblastic disease 321 ovarian cancer 255 testicular cancer 332 see also endoscopic ultrasound (EUS) ultraviolet radiation (UVR) eye malignancy association 608 melanoma association 487, 488 indoor tanning 488–489 non‐melanoma skin cancer association 502, 505 upper urinary tract urothelial carcinoma (UUT‐UCC) 245 urachal adenocarcinoma 246 staging 246 Index ureterectomy 245 urinary bladder cancer see bladder cancer urinary cytology 241 urothelial cell carcinoma (UCC) bladder 237 pathology 238–239 see also bladder cancer prostatic 245–246 upper urinary tract 245 uterine corpus cancer 268 see also endometrial carcinoma uterine sarcoma 280–282 classification 280 uveal melanoma 611–612, 612, 613 v vaccines human papillomavirus 149, 178, 289 melanoma 495 vaginal cancer 304–308 etiology 304 follow‐up 308 incidence 304 mortality 304 pathology 305–306, 306 adenocarcinoma 305 melanoma 306, 306 sarcoma 305–306, 306 squamous cell carcinoma 305 presentation 304–305 prevention 304 routes of spread 306–307, 306 staging 307, 307 treatment 307–308 treatment‐related complications 308 vaginal intraepithelial neoplasia (VAIN) 304–305, 305 diagnosis 305 pathology 305 treatment 307 vandetanib, thyroid cancer 528, 529 vascular endothelial growth factor (VEGF) inhibitors cholangiocarcinoma 120 lung cancer 13–14 renal cell carcinoma 231 vasoactive intestinal peptide (VIP) 555 VIPoma 555 vemurafenib hairy cell leukemia 429 melanoma 497 venetoclax chronic lymphocytic leukemia 427 multiple myeloma 477 venous thromboembolism (VTE) 661 verrucous carcinoma larynx 202 vaginal 305 vulvar 311, 312 vestibular schwannoma (VS) 587 vinblastine, testicular cancer 336–337 vincristine adrenal cancer 537 gestational trophoblastic disease 324 vinflunine, bladder cancer 244 VIPoma 555 vismodegib, skin cancer 504 vitamin C, gastric cancer relationship 54 VLX1570, multiple myeloma 477 vocal cord cancer 202, 203 see also larynx cancer von Hippel–Lindau syndrome (VHL) 228, 556 von Recklinghausen’s neurofibromatosis (NF1) 164 vulvar cancer 310–316 etiology 310, 311 follow‐up 315–316 incidence 310 metastatic disease 312 mortality 310 pathology 311–313 Bartholin gland cancer 312–313 basal cell carcinoma 313 extramammary Paget disease 313 melanoma 312 squamous cell carcinoma 311–312 verrucous carcinoma 312 presentation 311, 311 prevention 310 recurrence 315–316 staging 312, 313 survivorship 316 treatment 314–315 chemotherapy 315 melanoma 315 radiation therapy 315 surgery 314–315, 314 survival rates 315 vulvar intraepithelial neoplasia (VIN) 310, 311 pathology 311 treatment 313 vulvectomy 314, 314 w WAGR syndrome 702 Whipple procedure 91, 91, 119 pylorus‐sparing 91, 92 Whipple triad 554 Wilm’s tumor (WT) 701–704 classification 702 etiology and risk factors 701–702 associated syndromes 701–702, 702 follow‐up and late effects 703–704 incidence 701 presentation and diagnosis 702–703 staging 703, 703 treatment 703 wireless capsule endoscopy 74–75 y 90 Y‐ibritumomab tiuxetan, follicular lymphoma 452 yolk sac tumor (YST) 333 presentation 334 z zoledronic acid breast cancer 388, 391 multiple myeloma bone disease 477 prostate cancer 353, 357–358 renal cell carcinoma 232 Zollinger–Ellison syndrome 555 755 WILEY END USER LICENSE AGREEMENT Go to www.wiley.com/go/eula to access Wiley’s ebook EULA ... annually, lung cancer is the most common cancer in China in both men and women Based on the increasing incidence of cigarette smoking in the developing world, it is estimated that most lung cancers... for developing lung cancer Though the prevalence of cigarette smoking is declining in the US, it is increasing at an alarming rate in developing and third world countries Consequently, the number... in patients with lung cancer The 7th Edition of the American Joint Committee on Cancer (AJCC) and the Union for International Cancer Control (UICC) system introduced in 2010 is in use until the