• Endocrine and Neuroendocrine 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 Endocrine and neuroendocrine tumours
    Introduction to Endocrine and Neuroendocrine tumours


  • 2. Pituitary gland
    Introduction
  • Tumour Type Transcription Factor(s) Hormone(s) Keratin
    (CAM 5.2 or CK18)
    Tumour Subtype Hormone Excess Syndrome
    PIT1 lineage tumours




    Somatotroph tumours PIT1 GH, α-subunit Perinuclear Densely granulated somatotroph tumour Florid acromegaly


    GH Fibrous bodies (>70%) Sparsely granulated somatotroph tumour Subtle acromegaly
    Lactotroph tumours PIT1, ERα PRL (paranuclear) Weak or negative Sparsely granulated lactotroph tumour Hyperprolactinemia


    PRL (diffuse cytoplasmic) Weak or negative Densely granulated lactotroph tumour Hyperprolactinemia
    Mammosomatotroph tumour PIT1, ERα GH (often predominant) PRL, α-subunit Perinuclear
    Acromegaly and hyperprolactinemia
    Thyrotroph tumour PIT1, GATA2/3 α-subunit, βTSH Weak or negative
    Hyperthyroidism
    Mature plurihormonal PIT1-lineage tumour PIT1, ERα, GATA2/3 GH (often predominant), PRL, α-subunit, βTSH Perinuclear
    Acromegaly,
    hyperprolactinemia and hyperthyroidism
    Acidophil stem cell tumour PIT1, ERα PRL (predominant), GH (focal/variable) Scattered fibrous bodies
    Hyperprolactinemia ± subclinical acromegaly
    Immature PIT1-lineage tumour PIT1, ERα GATA2/3 GH, PRL, α-subunit, βTSH Focal/Variable
    Acromegaly
    and/or Hyperprolactinemia
    and/or hyperthyroidism
    TPIT lineage tumours




    Corticotroph tumours TPIT (TBX19), NeuroD1/β2 ACTH and other POMC derivatives Strong Densely granulated corticotroph tumour Florid Cushing disease, often microtumour



    Variable Sparsely granulated corticotroph tumour Subtle Cushing disease, often macrotumour



    Intense ring-like perinuclear Crooke cell tumour Variable,
    Cushing disease but may be unusual
    SF1 lineage tumours




    Gonadotroph tumour SF1, ERα GATA2/3 α-subunit, βFSH, βLH Variable
    Hypogonadism (virtually all)
    Hypergonadism (exceptional)
    Tumours without distinct cell lineage




    Unclassified plurihormonal tumours Multiple combinations Multiple combinations Variable
    Variable
    Null cell tumour None None Variable
    None

    • Anterior neuroendocrine neoplasms
      Pituitary neuroendocrine tumors of PIT1-lineage
      Somatotroph PitNET/adenoma
      Mammosomatotroph PitNET/adenoma
      Lactotroph PitNET/adenoma
      Thyrotroph PitNET/adenoma
      Mature plurihormonal PIT1 lineage PitNET/adenoma
      Immature PIT1-lineage PitNET/adenoma
      Acidophil stem cell PitNET/adenoma
      Mixed somatotroph-lactotroph PitNET/adenoma
      Pituitary neuroendocrine tumors of TPIT lineage
      Corticotroph PitNET/adenoma
      Pituitary neuroendocrine tumors of SF1 lineage
      Gonadotroph PitNET/adenoma
      Pituitary neuroendocrine tumours without distinct lineage differentiation
      Null cell PitNET/adenoma
      Plurihormonal PitNET/adenomas
      Multiple pituitary neuroendocrine tumours
      Multiple synchronous PitNET/adenomas of distinct lineages
      Metastatic pituitary neuroendocrine tumours
      Metastatic PitNET
      Other anterior pituitary tumours
      Adamantinomatous craniopharyngioma
      Papillary craniopharyngioma
      Pituitary blastoma
      Posterior pituitary and hypothalamic neoplasms
      Pituicyte-derived tumours
      Pituicyte tumour family
      Neuronal tumours
      Gangliocytoma and mixed gangliocytoma - PitNET/pituitary adenoma
      Sellar neurocytoma
      Other sellar tumours
      Meningioma
      Chordoma


    • 3. Thyroid gland
      Introduction
      Developmental abnormalities
      Thyroglossal duct cyst
      Other congenital thyroid abnormalities
      Follicular cell-derived neoplasms
      Benign tumours
      Thyroid follicular nodular disease
      Follicular thyroid adenoma
      Follicular thyroid adenoma with papillary architecture
      Oncocytic adenoma of the thyroid
      Low risk neoplasms
      Non-invasive follicular thyroid neoplasm with papillary-like nuclear features
      Thyroid tumours of uncertain malignant potential
      Hyalinizing trabecular tumour of thyroid
      Malignant neoplasms
      Follicular thyroid carcinoma
      Invasive encapsulated follicular variant papillary carcinoma
      Papillary thyroid carcinoma
      Oncocytic carcinoma of the thyroid
      Follicular-derived carcinomas, high-grade
      Anaplastic follicular cell derived thyroid carcinoma
      Thyroid C-cell derived carcinoma
      Medullary thyroid carcinoma
      Mixed medullary and follicular-cell derived carcinomas
      Mixed medullary and follicular cell-derived thyroid carcinoma
      Salivary gland-type carcinomas of the thyroid
      Mucoepidermoid carcinoma of the thyroid
      Secretory carcinoma of salivary gland type
      Thyroid tumours of uncertain histogenesis
      Sclerosing mucoepidermoid carcinoma with eosinophilia
      Cribriform morular thyroid carcinoma
      Thymic tumours within the thyroid
      Thymoma family
      Spindle epithelial tumour with thymus-like elements
      Thymic carcinoma family
      Embryonal thyroid neoplasms
      Thyroblastoma


    • 4. Parathyroid glands
      Introduction
      Parathyroid hyperplasia
      Parathyroid adenoma
      Parathyroid lipoadenoma
      Atypical parathyroid tumour
      Parathyroid carcinoma


    • 5. Adrenal gland
      Introduction
      Adrenal cortex
      Adrenal rests
      Adrenal cysts
      Adrenal myelolipoma
      Adrenal cortical tumours
      Congenital adrenal hyperplasia
      Adrenocortical nodular disease
      Adrenal cortical adenoma
      Adrenal cortical carcinoma
      Sex cord stromal and other tumours
      Adrenal sex cord stromal tumour
      Adenomatoid tumour
      Adrenal melanoma


    • 6. Tumours of the adrenal medulla and extra-adrenal paraganglia
      Introduction
      Neuroblastic tumours
      Neuroblastoma
      Ganglioneuroblastoma, intermixed
      Ganglioneuroblastoma, nodular
      Ganglioneuroma
      Paraganglioma and pheochromocytoma
      Phaeochromocytoma
      Sympathetic paraganglioma
      Parasympathetic paraganglioma
      Composite paraganglion tumours
      Composite phaeochromocytoma and paraganglioma


    • 7. Neuroendocrine pancreas
      Neuroendocrine neoplasms
      Introduction
      Neuroendocrine tumours
      Non-functioning (non-syndromic) neuroendocrine tumours
      Insulinoma
      Glucagonoma
      Somatostatinoma
      Gastrinoma
      VIPoma
      Serotonin-producing tumours with and without carcinoid syndrome
      Other pancreatic neuroendocrine tumours with ectopic hormone production
      Neuroendocrine carcinomas
      Pancreatic neuroendocrine carcinoma
      Mixed neuroendocrine-non-neuroendocrine neoplasms (MiNENs)
      Mixed ductal adenocarcinoma-neuroendocrine neoplasms
      Mixed acinar neuroendocrine carcinomas


    • 8. Neuroendocrine neoplasms, non-endocrine organs
      Introduction
      Neuroendocrine neoplasms
      Neuroendocrine tumours
      Neuroendocrine tumour
      Neuroendocrine carcinoma
      Small cell neuroendocrine carcinoma
      Large cell neuroendocrine carcinoma
      Merkel cell carcinoma
      Mixed neuroendocrine neoplasms
      Mixed neuroendocrine-non-neuroendocrine neoplasms (MiNENs)
      Paragangliomas-like NEN
      Composite gangliocytoma/neuroma and neuroendocrine tumour (CoGNET)
      Cauda equina neuroendocrine tumour (previously paraganglioma)


    • 9. Mesenchymal and stromal tumours
      Introduction
      Lipocytic tumours
      Lipoma
      Fibrous tumours
      Solitary fibrous tumour
      Vascular tumours
      Benign vascular tumours
      PEComa
      Glomangioma and glomus tumour
      Angiosarcoma
      Smooth muscle tumours
      Leiomyoma
      Leiomyosarcoma
      Skeletal muscle tumours
      Rhabdomyosarcoma family
      Peripheral nerve sheath tumours
      Schwannoma
      Neurofibroma
      Granular cell tumour
      MPNST


    • 10. Haematolymphoid tumours
      Introduction
      IgG4-related disease
      Extranodal marginal zone lymphoma
      Diffuse large B cell lymphoma
      Langerhans cell histiocytosis
      Rosai Dorfman disease
      Erdheim Chester disease
      Follicular dendritic cell sarcoma


    • 11. Germ cell tumours
      Germ cell tumours
      Introduction
      Germ cell tumours in endocrine organs
      Teratomas with endocrine differentiation


    • 12. Metastasis
      Introduction
      Metastasis to the pituitary
      Metastasis to thyroid
      Metastasis to the adrenal


    • 13. Genetic tumour syndromes
      Introduction
      Multiple endocrine neoplasia type 1
      Multiple endocrine neoplasia type 2
      Multiple endocrine neoplasia type 4
      Multiple endocrine neoplasia type 5 (MAX related tumours)
      Hyperparathyroidism jaw tumour syndrome
      Von Hippel Lindau syndrome
      SDH deficient tumour syndromes
      Neurofibromatosis type 1
      Carney complex
      McCune Albright syndrome
      DICER1 syndrome
      Glucagon cell hyperplasia and neoplasia
      MAFA related familial insulinomatosis
      Syndromic familial follicular cell-derived thyroid tumours
      Non-syndromic familial follicular cell-derived thyroid tumours