WHO Classification of Endocrine and Neuroendocrine Tumours

Endocrine and Neuroendocrine Tumours is Volume 10 in the 5th edition of the WHO series on the classification of human tumours. This series (also known as the WHO Blue Books) is regarded as the gold standard for the diagnosis of tumours and comprises a unique synthesis of histopathological diagnosis with digital and molecular pathology. These authoritative and concise reference books provide indispensable international standards for anyone involved in the care of patients with cancer or in cancer research, underpinning individual patient treatment as well as research into all aspects of cancer causation, prevention, therapy, and education.

What’s new in this edition?

The 5th edition, guided by the WHO Classification of Tumours Editorial Board, establishes a single coherent cancer classification presented across a collection of individual volumes organized on the basis of anatomical site (digestive system, breast, soft tissue and bone, etc.) and structured in a systematic manner, with each tumour type listed within a taxonomic classification: site, category, family (class), type, and subtype. In each volume, the entities are now listed from benign to malignant and are described under an updated set of headings, including histopathology, diagnostic molecular pathology, staging, and easy-to-read essential and desirable diagnostic criteria.

Who should read this book?

Pathologists

Endocrinologists

Medical oncologists

Surgical oncologists

Radiation oncologists

Radiologists

Nuclear medicine specialists

Molecular pathologists

Geneticists

Immunologists

Cancer researchers

Epidemiologists

General practitioners

Oncology nurses

Cancer registrars

This volume

Prepared by 175 authors and editors

Contributors from around the world

More than 1100 high-quality images

More than 5100 references

Table of contents

List of abbreviations x
Foreword xi
ICD-O topographical coding 1
ICD-O morphological coding 4
Pituitary tumours 5
Thyroid tumours 7
Parathyroid tumours 9
Adrenal gland tumours 10
Adrenal medulla and extra-adrenal paraganglia tumours 11
Pancreatic neuroendocrine neoplasms 12
Neuroendocrine neoplasms in non-endocrine organs 13
Mesenchymal and stromal tumours 14
Haematolymphoid tumours 16
Germ cell tumours 17
TNM staging of endocrine and neuroendocrine tumours 18
Carcinomas of the thyroid gland 18
Carcinomas of the adrenal cortex 20
Tumours of the pancreas 21
Well-differentiated neuroendocrine tumours of the
gastrointestinal tract 22
1 Introduction to endocrine and neuroendocrine tumours 28
2 Pituitary tumours 33
Introduction 34
Anterior pituitary neuroendocrine tumours (PitNETs)
PitNETs of PIT1 lineage
Somatotroph PitNET/adenoma 40
Mammosomatotroph PitNET/adenoma 45
Lactotroph PitNET/adenoma 48
Thyrotroph PitNET/adenoma 53
Mature plurihormonal PIT1-lineage PitNET/adenoma 55
Immature PIT1-lineage PitNET/adenoma 57
Acidophil stem cell PitNET/adenoma 59
Mixed somatotroph–lactotroph PitNET/adenoma 62
PitNETs of TPIT lineage
Corticotroph PitNET/adenoma 65
PitNETs of SF1 lineage
Gonadotroph PitNET/adenoma 70
PitNETs without distinct lineage differentiation
Null cell PitNET/adenoma 73
Plurihormonal PitNET/adenoma 75
Multiple PitNETs
Multiple synchronous PitNETs/adenomas of
distinct lineages 77
Metastatic PitNETs
Metastatic PitNET 79
Other anterior pituitary tumours
Adamantinomatous craniopharyngioma 82
Papillary craniopharyngioma 86
Pituitary blastoma 89
Posterior pituitary and hypothalamic neoplasms
Pituicyte-derived tumours
Pituicytomas 92
Neuronal tumours
Gangliocytoma and mixed gangliocytoma–PitNET/
adenoma 97
Sellar neurocytoma 100
Other sellar tumours
Meningiomas 103
Chordoma 105
3 Thyroid tumours
Introduction 108
Developmental abnormalities
Thyroglossal duct cyst 113
Other congenital thyroid abnormalities 116
Follicular cell–derived neoplasms
Benign tumours
Thyroid follicular nodular disease 119
Follicular thyroid adenoma 122
Follicular thyroid adenoma with papillary architecture 126
Oncocytic adenoma of the thyroid gland 129
Low-risk neoplasms
Non-invasive follicular thyroid neoplasm with
papillary-like nuclear features 132
Thyroid tumours of uncertain malignant potential 135
Hyalinizing trabecular tumour 138
Malignant neoplasms
Follicular thyroid carcinoma 142
Invasive encapsulated follicular variant of papillary
thyroid carcinoma 147
Papillary thyroid carcinoma 150
Oncocytic carcinoma of the thyroid gland 161
High-grade follicular cell–derived non-anaplastic
thyroid carcinoma 165
Anaplastic thyroid carcinoma 170
Thyroid C cell–derived carcinoma
Medullary thyroid carcinoma 174
Mixed medullary and follicular cell–derived carcinomas
Mixed medullary and follicular cell–derived thyroid
carcinoma 179
Salivary gland–type carcinomas of the thyroid gland
Mucoepidermoid carcinoma 182
Secretory carcinoma 184
Thyroid tumours of uncertain histogenesis
Sclerosing mucoepidermoid carcinoma with
eosinophilia 186
Cribriform morular thyroid carcinoma 189
Thymic tumours within the thyroid
Thymoma 192
Spindle epithelial tumour with thymus-like elements 194
Intrathyroidal thymic carcinoma 196
Embryonal thyroid neoplasms
Thyroblastoma 199
4 Parathyroid tumours 201
Introduction 202
Parathyroid hyperplasia 204
Parathyroid adenoma 207
Parathyroid lipoadenoma 213
Atypical parathyroid tumour 215
Parathyroid carcinoma 218
5 Adrenal gland tumours 223
Introduction 224
Lesions in the adrenal cortex
Adrenal rests 226
Contents
Adrenal cysts 228
Adrenal myelolipoma 230
Adrenal cortical tumours
Congenital adrenal hyperplasia 232
Adrenocortical nodular disease 236
Adrenal cortical adenoma 240
Adrenal cortical carcinoma 246
Sex cord stromal and other tumours
Adrenal sex cord stromal tumour 254
Adenomatoid tumour 256
Adrenal melanoma 258
6 Adrenal medulla and extra-adrenal paraganglia tumours 261
Introduction 262
Neuroblastic tumours
Neuroblastoma 265
Ganglioneuroblastoma, intermixed 271
Ganglioneuroblastoma, nodular 273
Ganglioneuroma 275
Paraganglioma and phaeochromocytoma
Phaeochromocytoma 277
Sympathetic paraganglioma 285
Parasympathetic paraganglioma 288
Composite paraganglion tumours
Composite phaeochromocytomas and
paragangliomas 292
7 Pancreatic neuroendocrine neoplasms 295
Introduction 296
Neuroendocrine neoplasms
Neuroendocrine tumours
Non-functioning (non-syndromic) pancreatic
neuroendocrine tumours 300
Insulinoma 305
Glucagonoma 309
Somatostatinoma 312
Gastrinoma 315
VIPoma 317
Serotonin-producing tumours
with and without carcinoid syndrome 320
Other pancreatic neuroendocrine tumours with
ectopic hormone production 322
Neuroendocrine carcinomas
Pancreatic neuroendocrine carcinoma 325
Mixed neuroendocrine–non-neuroendocrine neoplasms
Mixed ductal adenocarcinoma–neuroendocrine
neoplasms 328
Mixed acinar–neuroendocrine carcinoma 330
8 Neuroendocrine neoplasms in non-endocrine organs 333
Introduction 334
Neuroendocrine neoplasms
Neuroendocrine tumours
Neuroendocrine tumours 337
Neuroendocrine carcinomas
Small cell neuroendocrine carcinoma 339
Large cell neuroendocrine carcinoma 342
Merkel cell carcinoma 346
Mixed neuroendocrine neoplasms
Mixed neuroendocrine–non-neuroendocrine
neoplasms 348
Paraganglioma-like neuroendocrine neoplasms
Composite gangliocytoma/neuroma and
neuroendocrine tumour 351
Cauda equina neuroendocrine tumour (previously
paraganglioma) 354
9 Mesenchymal and stromal tumours 357
Introduction 358
Lipocytic tumours
Lipoma 359
Fibrous tumours
Solitary fibrous tumour 360
Vascular tumours
Benign vascular tumours 362
PEComa 364
Glomangioma and glomus tumour 366
Angiosarcoma 368
Smooth muscle tumours
Leiomyoma 371
Leiomyosarcoma 373
Skeletal muscle tumours
Rhabdomyosarcomas 376
Peripheral nerve sheath tumours
Schwannoma 379
Neurofibroma 381
Granular cell tumour 383
Malignant peripheral nerve sheath tumour 385
10 Haematolymphoid tumours 387
Introduction 388
IgG4-related disease 389
Extranodal marginal zone lymphoma of
mucosa-associated lymphoid tissue 392
Diffuse large B-cell lymphoma NOS 395
Langerhans cell histiocytosis 397
Rosai–Dorfman disease 400
Erdheim–Chester disease 402
Follicular dendritic cell sarcoma 404
11 Germ cell tumours 407
Introduction 408
Germ cell tumours
Germ cell tumours in endocrine organs 409
Teratomas with endocrine differentiation 412
12 Metastasis 415
Introduction 416
Metastasis to the pituitary gland 417
Metastasis to the thyroid gland 419
Metastasis to the adrenal gland 422
13 Genetic tumour syndromes associated with endocrine
lesions and tumours 425
Introduction 426
Multiple endocrine neoplasia type 1 429
Multiple endocrine neoplasia type 2 435
Multiple endocrine neoplasia type 4 440
Multiple endocrine neoplasia type 5 (MAX-related
tumours) 444
Hyperparathyroidism–jaw tumour syndrome 448
Von Hippel–Lindau syndrome 452
SDH-deficient tumour syndromes 457
Neurofibromatosis type 1 463
Carney complex 466
McCune–Albright syndrome 471
DICER1 syndrome 474
Glucagon cell hyperplasia and neoplasia 478
MAFA-related familial insulinomatosis 480
Syndromic familial follicular cell–derived thyroid
tumours 482
Non-syndromic familial follicular cell–derived
thyroid tumours 488
Contributors 493
Declaration of interests 499
IARC/WHO Committee for ICD-O 500
Sources 501
References 507
Subject index 587
Previous volumes in the series 595