List of abbreviations xi
Foreword xii
ICD-O topographical coding 1
ICD-O morphological coding 1
Skin tumours 2
TNM staging of skin tumours 15
Carcinoma of the skin 16
Skin carcinoma of the head and neck 17
Carcinoma of the skin of the eyelid 19
Melanoma of the skin 20
Merkel cell carcinoma of the skin 22
Soft tissue sarcoma of the skin 23
1 Introduction to skin tumours 26
2 Keratinocytic/epidermal tumours 27
Introduction 28
Carcinoma precursors and benign simulants
Premalignant keratoses and precursors
Actinic keratosis 30
Arsenical keratosis 32
PUVA keratosis 34
Squamous cell carcinoma in situ (Bowen disease) 36
Verrucae
Verruca vulgaris 38
Verruca plantaris and verruca palmaris 40
Verruca plana 42
Benign acanthomas/keratoses
Seborrhoeic keratosis 44
Solar lentigo 46
Lichen planus–like keratosis 48
Clear cell acanthoma 50
Large cell acanthoma 52
Warty dyskeratoma 54
Other benign keratoses 56
Epidermal carcinomas
Basal cell carcinomas 58
Superficial basal cell carcinoma 60
Nodular basal cell carcinoma 62
Micronodular basal cell carcinoma 64
Infiltrating basal cell carcinoma 66
Sclerosing/morphoeic basal cell carcinoma 68
Basosquamous carcinoma 70
Basal cell carcinoma with sarcomatoid differentiation 72
Basal cell carcinoma with adnexal differentiation 74
Fibroepithelial basal cell carcinoma 76
Keratoacanthoma
Keratoacanthoma 78
Squamous cell carcinomas 82
Verrucous squamous cell carcinoma 84
Acantholytic squamous cell carcinoma 86
Lymphoepithelial carcinoma 88
Clear cell squamous cell carcinoma 90
Spindle cell squamous cell carcinoma 92
Squamous cell carcinoma with sarcomatoid
differentiation 94
Neuroendocrine neoplasms
Neuroendocrine carcinomas
Merkel cell carcinoma 95
3 Melanocytic neoplasms 99
Introduction 101
Genomic landscape of melanoma 106
Melanocytic neoplasms in intermittently sun-exposed skin
Naevi
Junctional, compound, and dermal naevi 110
Simple lentigo and lentiginous melanocytic naevus 113
Dysplastic naevus 115
Naevus spilus 119
Special-site naevi (of the breast, axilla, scalp, and ear) 121
Halo naevus 123
Meyerson naevus 125
Recurrent naevus 126
Combined naevus 128
Melanocytomas
WNT-activated deep penetrating / plexiform
melanocytoma (naevus) 130
Pigmented epithelioid melanocytoma 132
BAP1-inactivated melanocytoma 134
MITF pathway–activated melanocytic tumours 136
Melanoma in intermittently sun-exposed skin
Low-CSD melanoma (including superficial
spreading melanoma) 138
Melanocytic neoplasms in chronically sun-exposed skin
Melanoma in chronically sun-exposed skin
High-CSD melanoma (including lentigo maligna
melanoma) 141
Desmoplastic melanoma 144
Spitz tumours
Spitz naevi
Pigmented spindle cell naevus (Reed naevus) 147
Spitz naevus 149
Spitz melanocytoma
Spitz melanocytoma (atypical Spitz tumour) 152
Spitz melanoma
Spitz melanoma 155
Melanocytic tumours in acral skin
Acral naevi
Acral naevus 158
Acral melanomas
Acral melanoma 160
Melanocytic neoplasms of the genitalia and mucosa
Mucosal and genital naevi
Melanosis 163
Genital naevus 165
Mucosal melanoma
Mucosal melanoma 167
Blue naevus and related neoplasms
Blue naevi and melanocytoses
Naevus of Ito and naevus of Ota 169
Congenital dermal melanocytosis 171
Blue naevus 173
Melanomas arising from blue naevus
Melanoma arising in blue naevus 176
Contents

Congenital melanocytic tumours
Congenital naevi
Congenital melanocytic naevus 178
Proliferative nodules in congenital melanocytic naevus 180
Melanomas arising in congenital naevi
Melanoma arising in giant congenital naevus 182
Ocular and CNS melanocytic tumours
Conjunctival melanocytic tumours
Conjunctival naevus 184
Conjunctival melanocytic intraepithelial lesions 186
Conjunctival melanoma 189
Uveal melanocytic tumours
Uveal melanocytoma 191
Uveal melanoma 193
CNS melanocytic tumours
Diffuse meningeal melanocytic neoplasms:
Melanocytosis and melanomatosis 196
Circumscribed meningeal melanocytic
neoplasms: Melanocytoma and melanoma 199
Nodular, naevoid, and metastatic melanomas
Nodular and other melanomas
Nodular melanoma 202
Naevoid melanoma 204
Dermal melanoma 206
Metastatic melanomas
Melanoma metastatic to the skin 208
Melanoma metastatic to other organs 210
4 Appendageal tumours 213
Introduction 215
Tumours with apocrine and eccrine differentiation
Benign apocrine and eccrine tumours
Hidrocystoma/cystadenoma 217
Syringoma 219
Poroma 221
Syringofibroadenoma 223
Hidradenoma 225
Spiradenoma 227
Cylindroma 229
Tubular adenoma 231
Syringocystadenoma papilliferum 233
Mixed tumour 235
Myoepithelial tumour 237
Malignant apocrine and eccrine tumours
Adnexal adenocarcinoma NOS 239
Microcystic adnexal carcinoma 241
Cribriform tumour (previously carcinoma) 243
Porocarcinoma 245
NUT carcinoma 247
Malignant neoplasms arising from spiradenoma,
cylindroma, or spiradenocylindroma 249
Malignant mixed tumour 252
Hidradenocarcinoma 254
Endocrine mucin-producing sweat gland carcinoma 256
Mucinous carcinoma 258
Digital papillary adenocarcinoma 260
Adenoid cystic carcinoma 262
Apocrine carcinoma 264
Squamoid eccrine ductal carcinoma 266
Syringocystadenocarcinoma papilliferum 268
Secretory carcinoma 270
Signet-ring cell / histiocytoid carcinoma 272
Tumours with follicular differentiation
Benign tumours with follicular differentiation
Trichoblastoma 274
Pilomatricoma 277
Melanocytic matricoma 279
Trichilemmoma 281
Trichoadenoma 283
Trichofolliculoma 285
Pilar sheath acanthoma 287
Tumour of the follicular infundibulum 288
Trichodiscoma and fibrofolliculoma 290
Malignant tumours with follicular differentiation
Proliferating trichilemmal tumour 292
Pilomatrical carcinoma 294
Trichoblastic carcinoma/carcinosarcoma 296
Trichilemmal carcinoma 298
Tumours with sebaceous differentiation
Benign tumours with sebaceous differentiation
Sebaceoma 300
Sebaceous adenoma 302
Malignant tumours with sebaceous differentiation
Sebaceous carcinoma 304
Site-specific appendageal tumours
Benign site-specific appendageal tumours
Hidradenoma papilliferum 307
Fibroadenoma and phyllodes tumour of
anogenital mammary-like glands 309
Malignant site-specific appendageal tumours
Mammary Paget disease 311
Extramammary Paget disease 313
Adenocarcinoma of mammary gland type 315
5 Tumours of the nail unit 317
Nail bed epithelial tumours
Onychomatricoma 318
Onychopapilloma 320
Ungual fibrokeratoma 322
Onychocytic matricoma 324
Subungual keratoacanthoma 326
6 Tumours of haematopoietic and lymphoid origin 329
Introduction 331
Myeloid proliferations and neoplasms
Mastocytosis
Mastocytosis 335
Mast cell sarcoma 339
Secondary cutaneous involvement in myeloid neoplasms
Cutaneous involvement in myeloid neoplasms,
including myeloid sarcoma 341
Histiocytic or dendritic cell neoplasms
Plasmacytoid dendritic cell neoplasms
Mature plasmacytoid dendritic cell proliferation
associated with myeloid neoplasm 344
Blastic plasmacytoid dendritic cell neoplasm 346
Langerhans cell neoplasms
Langerhans cell histiocytosis 348
Histiocytic tumours
Xanthelasma 352
Juvenile xanthogranuloma 354
Reticulohistiocytosis 357
Rosai–Dorfman disease 360
Erdheim–Chester disease 363
ALK-positive histiocytosis 365
Histiocytic sarcoma 367
Other dendritic cell neoplasms
Indeterminate dendritic cell tumour 369
Cutaneous B-cell lymphoproliferative disorders and neoplasms
Reactive B-cell–rich lymphoid proliferations 371
Primary cutaneous mature B-cell neoplasms
Primary cutaneous marginal zone lymphoma 374
Primary cutaneous follicle centre lymphoma 377
Primary cutaneous diffuse large B-cell lymphoma,
leg type 380
Intravascular large B-cell lymphoma 383Cutaneous involvement in primarily extracutaneous
B-cell lymphomas and leukaemias
Mantle cell lymphoma 385
Burkitt lymphoma 387
Chronic lymphocytic leukaemia / small
lymphocytic lymphoma 389
B-lymphoblastic leukaemia/lymphoma 391
Lymphomatoid granulomatosis 393
Cutaneous T-cell and NK-cell lymphoproliferative disorders
and neoplasms
Reactive T-cell–rich lymphoid proliferations 396
Primary cutaneous T-cell disorders
Primary cutaneous CD4-positive small or medium
T-cell lymphoproliferative disorder 399
Mycosis fungoides 402
Primary cutaneous CD30-positive T-cell lympho-
proliferative disorder: Lymphomatoid papulosis 407
Primary cutaneous CD30-positive T-cell
lymphoproliferative disorder: Primary cutaneous
anaplastic large cell lymphoma 411
Subcutaneous panniculitis-like T-cell lymphoma 414
Primary cutaneous gamma/delta T-cell lymphoma 416
Primary cutaneous acral CD8-positive T-cell
lymphoproliferative disorder 419
Primary cutaneous CD8-positive aggressive
epidermotropic cytotoxic T-cell lymphoma 422
Primary cutaneous peripheral T-cell lymphoma NOS 425
Sézary syndrome 427
EBV-positive lymphoproliferative diseases of childhood
Severe mosquito bite allergy 430
Hydroa vacciniforme lymphoproliferative disorder 432
Secondary cutaneous involvement in T-cell and NK-cell
lymphomas and leukaemias
Anaplastic large cell lymphoma 434
Nodal T follicular helper cell lymphoma,
angioimmunoblastic type 437
T-prolymphocytic leukaemia 439
T-lymphoblastic leukaemia/lymphoma 442
Adult T-cell leukaemia/lymphoma 444
Extranodal NK/T-cell lymphoma 446
Other cutaneous lymphoproliferative disorders
Lymphoproliferative disorders and lymphomas
associated with immunodeficiency and dysregulation
Introduction 449
EBV-positive mucocutaneous ulcer 450
Inborn error of immunity–associated
lymphoproliferative disorders 453
7 Soft tissue tumours 455
Introduction 457
Adipocytic tumours
Naevus lipomatosus superficialis 459
Lipoma 461
Angiolipoma 463
Spindle cell / pleomorphic lipoma 465
Atypical lipomatous tumour 467
Pleomorphic liposarcoma 469
Fibroblastic, myofibroblastic, and fibrohistiocytic tumours
Benign fibroblastic, myofibroblastic, and fibrohistiocytic
neoplasms
Fibroma of tendon sheath 471
Calcifying aponeurotic fibroma 473
Sclerotic fibroma 475
Nuchal-type fibroma 476
Gardner fibroma 478
Pleomorphic fibroma 479
Elastofibroma 481
Desmoplastic fibroblastoma 482
Fibrous papule 483
Fibroblastic connective tissue naevus 485
Fibro-osseous tumour of digits 487
Dermatofibroma (fibrous histiocytoma) 488
Superficial fibromatosis 491
Inclusion body fibromatosis 493
Plexiform fibrohistiocytic tumour 495
Superficial acral fibromyxoma 497
Cutaneous myxoma (superficial angiomyxoma) 499
Dermatomyofibroma 501
Multinucleate cell angiohistiocytoma 503
Plaque-like CD34-positive dermal fibroma 505
Nodular fasciitis 507
EWSR1::SMAD3-rearranged fibroblastic tumour 509
Intermediate fibroblastic, myofibroblastic,
and fibrohistiocytic neoplasms
Dermatofibrosarcoma protuberans 511
Myxoinflammatory fibroblastic sarcoma 515
Malignant fibroblastic, myofibroblastic,
and fibrohistiocytic neoplasms
Myxofibrosarcoma 517
Vascular tumours
Haemangiomas
Cherry haemangioma 519
Sinusoidal haemangioma 520
Microvenular haemangioma 521
Hobnail haemangioma 522
Glomeruloid haemangioma 524
Papillary haemangioma 525
Spindle cell haemangioma 526
Epithelioid haemangioma 528
Tufted haemangioma 530
Angiokeratoma 532
Infantile haemangioma 534
Congenital non-progressive haemangiomas:
Rapidly involuting congenital haemangioma and
non-involuting congenital haemangioma 536
Lobular capillary haemangioma 538
Poikilodermatous plaque-like haemangioma 540
Acquired elastotic haemangioma 542
Verrucous venous malformation 543
Arteriovenous malformation 545
Other benign vascular tumours
Lymphangioma (superficial lymphatic malformation) 547
Cutaneous epithelioid angiomatous nodule 549
Postradiation atypical vascular lesion 551
Intermediate vascular neoplasms
Pseudomyogenic haemangioendothelioma 553
Epithelioid haemangioendothelioma 555
Hobnail haemangioendotheliomas 557
Composite haemangioendothelioma 559
Kaposi sarcoma 561
Malignant vascular sarcomas
Cutaneous angiosarcoma 566
Pericytic and perivascular tumours
Benign pericytic and perivascular neoplasms
Glomus tumour 568
Myopericytoma 571
Myofibroma and myofibromatosis 573
Angioleiomyoma 575
Smooth muscle tumours
Benign smooth muscle neoplasms
Smooth muscle hamartoma 577
Cutaneous leiomyomas 579
EBV-associated smooth muscle tumour 581
Intermediate smooth muscle neoplasms
Atypical intradermal smooth muscle neoplasm 583Neural tumours
Benign neural neoplasms
Dermal hyperneury / epithelial sheath neuroma 585
Solitary circumscribed neuroma 587
Dermal nerve sheath myxoma 589
Perineurioma 591
Neurofibroma 593
Schwannoma 596
Granular cell tumour 598
Hybrid nerve sheath tumours 601
Malignant neural neoplasms
Malignant peripheral nerve sheath tumour 603
Tumours of uncertain differentiation
Benign neoplasms of uncertain differentiation
Cellular neurothekeoma 606
Epithelioid fibrous histiocytoma 608
Non-neural granular cell tumour 610
PEComa 612
Intermediate neoplasms of uncertain differentiation
Angiomatoid fibrous histiocytoma 614
NTRK-rearranged spindle cell neoplasm 616
Atypical fibroxanthoma 618
Superficial CD34-positive fibroblastic tumour 621
Malignant neoplasms of uncertain differentiation
Pleomorphic dermal sarcoma 623
Epithelioid sarcoma 625
CRTC1::TRIM11 cutaneous tumour 627
Dermal clear cell sarcoma 629
Ewing sarcoma 631
8 Metastases to skin 633
Metastases to skin 634
Cancer of unknown primary site 637
9 Genetic tumour syndromes associated with
skin malignancies 641
Introduction 642
Familial melanoma 645
BAP1-related tumour predisposition syndrome 648
Xeroderma pigmentosum 650
Naevoid basal cell carcinoma syndrome
(Gorlin syndrome) 653
Carney complex 656
Muir–Torre syndrome 658
Brooke–Spiegler and related syndromes 660
Contributors 663
Declaration of interests 672
IARC/WHO Committee for ICD-O 673
Sources 674
References 682
Subject index 752
Previous volumes in the series 762