- Paediatric tumours (5th ed.)
- 1. Forewords and Introductions
WHO Classification of Tumours: Editorial Board
How to cite this volume
Foreword with changes from the book, including corrigenda
ICD-O coding of paediatric tumours
Introduction to paediatric tumours
- 2. Haematolymphoid disorders
Haematolymphoid disorders: Introduction
Myeloid neoplasms
Myeloproliferative neoplasms
Chronic myeloid leukaemia (CML)
Myelodysplastic/myeloproliferative neoplasms
Juvenile myelomonocytic leukaemia
Myelodysplastic syndromes
Refractory cytopenia of childhood
Myelodysplastic syndrome with excess blasts
Acute myeloid leukaemia (AML) and related neoplasms
Acute myeloid leukaemias (AMLs) defined by differentiation
Acute myeloid leukaemias (AMLs) with recurrent genetic abnormalities
Myeloid neoplasms and proliferations associated with antecedent or predisposing conditions
Myeloid neoplasms associated with germline predisposition
Myeloid proliferations associated with Down syndrome
Mast cell neoplasia
Mastocytosis
Lymphoid neoplasms
Precursor lymphoid neoplasms
B-cell lymphoblastic leukaemia/lymphoma
T-cell and NK-cell lymphoblastic leukaemia/lymphoma
Mature B-cell neoplasms
Primary mediastinal large B-cell lymphoma
Diffuse large B-cell lymphoma NOS
EBV-positive diffuse large B-cell lymphoma NOS
Large B-cell lymphoma with IRF4 rearrangement
Paediatric-type follicular lymphoma
Paediatric nodal marginal zone lymphoma
ALK-positive large B-cell lymphoma
Lymphomatoid granulomatosis
Plasmablastic lymphoma
Mediastinal grey zone lymphoma
Burkitt lymphoma
Large B-cell lymphoma with 11q aberration
Mature T/NK-cell neoplasms
Peripheral T-cell lymphoma NOS
Aggressive NK-cell leukaemia
Primary cutaneous T-cell neoplasms
Mycosis fungoides
Primary cutaneous CD30-positive T-cell lymphoproliferative disorders
Subcutaneous panniculitis-like T-cell lymphoma
Anaplastic large cell lymphoma
Anaplastic large cell lymphoma, ALK-positive
Hepatosplenic T-cell lymphoma
Hepatosplenic T-cell lymphoma
EBV-positive lymphoproliferative diseases of childhood
Systemic EBV-positive T-cell lymphoma of childhood
Hydroa vacciniforme lymphoproliferative disorder
Hodgkin lymphoma
Classic Hodgkin lymphoma
Nodular lymphocyte-predominant Hodgkin lymphoma
Histiocytic and dendritic cell neoplasms
Langerhans cell histiocytosis and related disorders
Immunodeficiency-associated lymphoproliferative disorders
Primary immunodeficiency-associated lymphoproliferative disorders
Posttransplant lymphoproliferative disorders
HIV-associated lymphoproliferative disorders
- 3. CNS tumours
CNS tumours: Introduction
Gliomas, glioneuronal & neuronal tumours
Gliomas, glioneuronal tumours, and neuronal tumours: Introduction
Paediatric-type diffuse low-grade gliomas
Diffuse astrocytoma, MYB- or MYBL1-altered
Angiocentric glioma
Polymorphous low-grade neuroepithelial tumour of the young
Diffuse low-grade glioma, MAPK pathway-altered
Paediatric-type diffuse high-grade gliomas defined by H3 status
Diffuse midline glioma, H3 K27-altered
Diffuse hemispheric glioma, H3 G34-mutant
Diffuse paediatric-type high-grade glioma, H3-wildtype and IDH-wildtype
Infant-type hemispheric glioma
Circumscribed astrocytic gliomas
Pilocytic astrocytoma
High-grade astrocytoma with piloid features
Pleomorphic xanthoastrocytoma
Subependymal giant cell astrocytoma
Astroblastoma, MN1-altered
Glioneuronal and neuronal tumours
Ganglioglioma
Desmoplastic infantile ganglioglioma / desmoplastic infantile astrocytoma
Dysembryoplastic neuroepithelial tumour
Diffuse glioneuronal tumour with oligodendroglioma-like features and nuclear clusters
Diffuse leptomeningeal glioneuronal tumour
Multinodular and vacuolating neuronal tumour
Ependymal tumours
Ependymal tumours: Introduction
Supratentorial ependymoma
Supratentorial ependymoma, ZFTA fusion-positive
Supratentorial ependymoma, YAP1 fusion-positive
Posterior fossa ependymoma
Posterior fossa group A (PFA) ependymoma
Posterior fossa group B (PFB) ependymoma
Spinal ependymoma, MYCN-amplified
Myxopapillary ependymoma
Choroid plexus tumours
Choroid plexus papilloma
Atypical choroid plexus papilloma
Choroid plexus carcinoma
CNS embryonal tumours
Medulloblastoma: Introduction
Medulloblastomas, molecularly defined
Medulloblastoma, WNT-activated
Medulloblastoma, SHH-activated and TP53-wildtype
Medulloblastoma, SHH-activated and TP53-mutant
Medulloblastoma, non-WNT/non-SHH
Medulloblastoma, histologically defined
Medulloblastoma, histologically defined
Other CNS embryonal tumours
Other CNS embryonal tumours: Introduction
Atypical teratoid/rhabdoid tumour
Cribriform neuroepithelial tumour
Embryonal tumour with multilayered rosettes
CNS neuroblastoma, FOXR2-activated
CNS tumour with BCOR internal tandem duplication
CNS embryonal tumour NEC/NOS
Pineal region tumours
Pineal region tumours
Pineal tumours: Introduction
Pineoblastoma
Melanocytic tumours
Melanocytic tumours: Introduction
Diffuse meningeal melanocytic neoplasms: Melanocytosis and melanomatosis
Tumours of the sellar region
Tumours of the sellar region: Introduction
Pituitary endocrine tumours
Pituitary adenoma / pituitary neuroendocrine tumour
Pituitary blastoma
Craniopharyngiomas
Adamantinomatous craniopharyngioma
- 4. Peripheral neuroblastic tumours
Peripheral neuroblastic tumours: Introduction
Ganglioneuroma
Ganglioneuroblastoma, intermixed
Neuroblastoma
Ganglioneuroblastoma, nodular (and other composite neuroblastic tumours)
- 5. Eye tumours
Eye tumours: Introduction
Conjunctival neoplasms
Hamartomas
Epibulbar choristoma
Epibulbar osseous choristoma
Phakomatous choristoma
Melanocytic neoplasms
Conjunctival junctional, compound, and subepithelial naevi
Inflamed juvenile conjunctival naevus
Uveal neoplasms
Diffuse choroidal neurofibroma and ganglioneuroma
Lisch nodule (iris hamartoma)
Retinal and neuroepithelial tumours
Retinocytoma
Retinoblastoma
Medulloepithelioma
Optic nerve tumours
Pilocytic astrocytoma and other gliomas of the optic nerve
- 6. Soft tissue and bone tumours
Soft tissue and bone tumours: Introduction
Soft tissue tumours
Adipocytic tumours
Lipomatosis
Lipoblastoma/lipoblastomatosis
Liposarcoma
Fibroblastic and myofibroblastic tumours
Fasciitis/myositis
Fibrodysplasia ossificans progressiva
Fibroma of tendon sheath
Gardner fibroma
Fibrous hamartoma of infancy
Lipofibromatosis
Inclusion body infantile digital fibromatosis
Juvenile hyaline fibromatosis (hyaline fibromatosis syndrome)
Fibromatosis colli
Calcifying aponeurotic fibroma
Sinonasal angiofibroma
Plantar/palmar fibromatoses
Dermatofibrosarcoma protuberans
Desmoid fibromatosis
EWSR1::SMAD3-positive fibroblastic tumour
Infantile fibrosarcoma
Paediatric NTRK-rearranged spindle cell neoplasm
Low-grade fibromyxoid sarcoma / sclerosing epithelioid fibrosarcoma
Inflammatory myofibroblastic tumour
Low-grade myofibroblastic sarcoma
So-called fibrohistiocytic tumours
Fibrous histiocytoma
Plexiform fibrohistiocytic tumour
Tenosynovial giant cell tumour
Vascular tumours
Capillary malformations
Venous malformations
Arteriovenous malformations
Intramuscular vascular anomalies
Lymphatic anomalies
Congenital haemangioma
Infantile haemangioma
Haemangioma of placenta
Lobular capillary haemangioma (pyogenic granuloma)
Epithelioid haemangioma
Tufted angioma and kaposiform haemangioendothelioma
Papillary intralymphatic angioendothelioma and retiform haemangioendothelioma
Pseudomyogenic haemangioendothelioma
Kaposi sarcoma
Epithelioid haemangioendothelioma
Angiosarcoma
Pericytic (perivascular) tumours
Myofibroma and myopericytoma
Glomus tumour and glomuvenous malformation
Smooth muscle tumours
EBV-associated smooth muscle tumour
Skeletal muscle tumours
Rhabdomyoma
Rhabdomyosarcomas
Ectomesenchymoma
Gastrointestinal stromal tumour
Paediatric gastrointestinal stromal tumour
Peripheral nerve sheath tumours
Schwannoma
Neurofibroma
Perineurioma
Hybrid nerve sheath tumour
Granular cell tumour
Solitary circumscribed neuroma
Ectopic meningioma and meningothelial hamartoma
Benign triton tumour / neuromuscular choristoma
Malignant peripheral nerve sheath tumour
Tumours of uncertain derivation
Intramuscular/juxta-articular myxoma
Superficial angiomyxoma
Deep angiomyxoma
Angiomatoid fibrous histiocytoma
Clear cell sarcoma of soft tissue
Alveolar soft part sarcoma
Extrarenal rhabdoid tumour
PEComa
Synovial sarcoma
Epithelioid sarcoma
Myoepithelial tumours of soft tissue
Phosphaturic mesenchymal tumour
Desmoplastic small round cell tumour
Undifferentiated sarcomas
Undifferentiated small round cell sarcomas of soft tissue and bone
Ewing sarcoma
Round cell sarcoma with EWSR1::non-ETS fusions
CIC-rearranged sarcoma
Sarcoma with BCOR genetic alterations
Bone tumours
Osteogenic tumours
Subungual exostosis
Bizarre parosteal osteochondromatous proliferation
Osteoblastoma
Osteoid osteoma
Chondromesenchymal hamartoma of the chest wall
Osteosarcoma
Chondrogenic tumours
Chondroblastoma
Osteochondroma
Chondromyxoid fibroma
Enchondroma and enchondromatosis
Chondrosarcoma
Mesenchymal chondrosarcoma
Other bone tumours
Vascular tumours of bone
Aneurysmal bone cyst
Giant cell tumour of bone
Non-ossifying fibroma
Notochordal tumours
Simple bone cyst
Adamantinoma
Osteofibrous dysplasia
Fibrous dysplasia
- 7. Germ cell tumours
Germ cell tumours: Introduction
Germ cell tumours
Non-invasive germ cell neoplasia
Intratubular germ cell neoplasia (male gonadal)
Gonadoblastoma
Germinoma-family tumours
Germinoma/dysgerminoma/seminoma
Non-germinomatous germ cell tumours
Non-germinomatous germ cell tumours: Introduction
Mature cystic teratoma
Extragonadal teratoma
Monodermal teratomas (female gonadal)
Immature teratoma (female gonadal)
Prepubertal-type testicular teratoma
Postpubertal-type teratoma
Embryonal carcinoma
Yolk sac tumour
Fetus in fetu
Choriocarcinoma (non-gestational)
Malignant mixed germ cell tumours
- 8. Renal and male genital tumours
Renal and male genital tumours: Introduction
Renal tumours
Nephroblastic and related tumours
Paediatric cystic nephroma
Nephroblastoma
Molecularly defined renal tumours
Renal cell carcinoma with MiT translocations
ALK-rearranged renal cell carcinoma
Eosinophilic solid and cystic renal cell carcinoma
SMARCB1-deficient renal medullary carcinoma
Metanephric tumours
Metanephric adenoma
Metanephric adenofibroma
Metanephric stromal tumour
Mesenchymal renal tumours
Ossifying renal tumour of infancy
Mesoblastic nephroma
Clear cell sarcoma of the kidney
Malignant rhabdoid tumour of the kidney
Anaplastic sarcoma of the kidney
Renal Ewing sarcoma
Testicular tumours
Non-germ cell testicular tumours: Introduction
Juvenile granulosa cell tumour of the testis
- 9. Female genital tumours
Non-germ cell tumours of the female genital tract: Introduction
Ovarian tumours
Sex cord stromal tumours
Ovarian fibroma
Sclerosing stromal tumour
Juvenile granulosa cell tumour of the ovary
Sex cord tumour with annular tubules
Papillary cystadenoma
Sertoli-Leydig cell tumour
Gynandroblastoma
Other ovarian tumours
Small cell carcinoma of the ovary, hypercalcaemic type
Lower female genital tract tumours
Epithelial tumours
Mesonephric remnants and hyperplasia
Mullerian papilloma
Condyloma acuminatum
Peritoneal tumours
Mesothelial tumours
Peritoneal inclusion cysts
- 10. Breast tumours
Breast tumours: Introduction
Breast tumours
Fibroepithelial tumours
Juvenile fibroadenoma
Juvenile papillomatosis
- 11. Digestive system tumours
Digestive system tumours: Introduction
Liver tumours
Epithelial tumours
Hepatoblastoma
Fibrolamellar variant of hepatocellular carcinoma
Paediatric hepatocellular carcinoma
Mesenchymal tumours unique to the liver
Mesenchymal hamartoma
Calcifying nested stromal-epithelial tumour
Embryonal sarcoma of the liver
Hepatic congenital haemangioma
Hepatic infantile haemangioma
Hepatic angiosarcoma
Pancreatic tumours
Epithelial tumours
Pancreatoblastoma
Pancreatic acinar cell carcinoma
Solid pseudopapillary neoplasm
Gastrointestinal tumours
Epithelial tumours
Gastroblastoma
Appendiceal neuroendocrine tumours
- 12. Endocrine tumours
Endocrine tumours: Introduction
Thyroid tumours
Thyroid epithelial tumours
Follicular adenoma of the thyroid
Papillary thyroid carcinoma
Medullary thyroid carcinoma
Spindle epithelial tumour with thymus-like elements
Parathyroid tumours
Parathyroid endocrine tumours
Parathyroid adenoma
Adrenal tumours
Adrenocortical tumours
Adrenal cortical tumours
Tumours of the adrenal medulla and extra-adrenal paraganglia
Sympathetic paraganglioma
Parasympathetic paraganglioma (head and neck paraganglioma)
Phaeochromocytoma
Composite phaeochromocytoma/paraganglioma
Neuroendocrine neoplasms
Neuroendocrine tumours
Other neuroendocrine tumours
- 13. Head and neck tumours
Head and neck tumours: Introduction
Benign tumours
Squamous cell papilloma of the larynx
White sponge naevus
Congenital granular cell epulis
Central giant cell granuloma
Odontogenic tumours
Ossifying fibroma
Sinonasal tract myxoma
Nasal dermoid cyst
Nasopharyngeal dermoid
Sinonasal chondromesenchymal hamartoma
Pleomorphic adenoma
Malignant tumours
Mucoepidermoid carcinoma
Acinic cell carcinoma
Sialoblastoma
Nasopharyngeal carcinoma
NUT carcinoma
Melanotic neuroectodermal tumour of infancy
- 14. Thoracic tumours
Lung tumours
Fetal lung interstitial tumour
Congenital peribronchial myofibroblastic tumour
Pleuropulmonary blastoma
Heart tumours
Cardiac rhabdomyoma
- 15. Skin tumours
Skin tumours: Introduction
Hamartomas
Hamartomas
Epithelial neoplasms
Squamous neoplasms
Angiokeratoma
Epidermal naevi
Pilomatricoma
Melanocytic neoplasms
Naevi
Congenital naevi
Junctional, compound, and dermal naevi
Blue naevus
Spitz naevus
Pigmented spindle cell naevus (Reed naevus)
Melanoma
Melanoma
- 16. Chapter 16: Genetic tumour syndromes
Selected tumour predisposition syndromes: Introduction
Syndromes predisposing primarily to neural tumours
Neurofibromatosis type 1
Neurofibromatosis type 2
Tuberous sclerosis
Naevoid basal cell carcinoma syndrome (Gorlin syndrome)
Retinoblastoma syndrome
Syndromes predisposing primarily to endocrine tumours
Von Hippel-Lindau syndrome
Hereditary phaeochromocytoma-paraganglioma syndromes
Syndromes predisposing primarily to Wilms tumours
WAGR syndrome
Beckwith-Wiedemann and related overgrowth syndromes
Syndromes predisposing primarily to gastrointestinal tumours
Familial adenomatous polyposis
Lynch syndrome
Other syndromes
Xeroderma pigmentosum
Rothmund-Thomson syndrome
Li-Fraumeni syndrome
DICER1 syndrome
BAP1 tumour predisposition syndrome
Constitutional mismatch repair deficiency syndrome
Rhabdoid tumour predisposition syndrome