Appendix A: WHO Classification of Tumors of the Central Nervous System WHO Classification 1979 • Ganglioneuroblastoma • Anaplastic [malignant] gangliocytoma and Zülch KJ (1979) Histological typing of tumours ganglioglioma of the central nervous system. 1st ed. World • Neuroblastoma Health Organization, Geneva • Poorly differentiated and embryonal tumours • Glioblastoma Tumours of Neuroepithelial tissue –– Variants: • Astrocytic tumours –– Glioblastoma with sarcomatous compo- • Astrocytoma nent [mixed glioblastoma and sarcoma] –– fibrillary –– Giant cell glioblastoma –– protoplasmic • Medulloblastoma –– gemistocytic –– Variants: • Pilocytic astrocytoma –– desmoplastic medulloblastoma • Subependymal giant cell astrocytoma [ven- –– medullomyoblastoma tricular tumour of tuberous sclerosis] • Medulloepithelioma • Astroblastoma • Primitive polar spongioblastoma • Anaplastic [malignant] astrocytoma • Gliomatosis cerebri • Oligodendroglial tumours • Oligodendroglioma Tumours of nerve sheath cells • Mixed-oligo-astrocytoma • Neurilemmoma [Schwannoma, neurinoma] • Anaplastic [malignant] oligodendroglioma • Anaplastic [malignant] neurilemmoma [schwan- • Ependymal and choroid plexus tumours noma, neurinoma] • Ependymoma • Neurofibroma –– Myxopapillary • Anaplastic [malignant]neurofibroma [neurofi- –– Papillary brosarcoma, neurogenic sarcoma] –– Subependymoma • Anaplastic [malignant] ependymoma Tumours of meningeal and related tissues • Choroid plexus papilloma • Meningioma • Anaplastic [malignant] choroid plexus papil- –– meningotheliomatous [endotheliomatous, loma syncytial, arachnotheliomatous] • Pineal cell tumour –– fibrous [fibroblastic] • Pineocytoma [pinealocytoma] –– transitional [mixed] • Pineoblastoma [pinealoblastoma] –– psammomatous • Neuronal tumour –– Angiomatous • Gangliocytoma –– haemangioblastic • Ganglioglioma –– haemangiopericytic © Springer-Verlag GmbH Austria, part of Springer Nature 2019 S. Weis et al., Imaging Brain Diseases, https://doi.org/10.1007/978-3-7091-1544-2 2167 2168 Appendix A: WHO Classification of Tumors of the Central Nervous System –– papillary Tumours of the anterior pituitary –– anaplastic [malignant] meningioma • Pituitary adenoma • Meningeal sarcomas • acidophil –– Fibrosarcoma • basophil [mucoid cell] –– Polymorphic cell sarcoma • mixed acidophil-basophil –– Primary meningeal sarcomatosis • chromophobe • Xanthomatous tumours • Pituitary adenocarcinoma –– Fibroxanthoma –– Xanthosarcoma [malignant fibroxanthoma] Local extensions from regional tumours • Primary melanotic tumours • Glomus jugulare tumour [chemodectoma, –– Melanoma paragnglioma] –– Meningeal melanomatosis • Chordoma • others • Chondroma • Chondrosarcoma Primary Malignant Lymphomas • Olfactory neuroblastoma [esthesioneuroblas- toma] Tumours of blood vessel origin • Adenoid cystic carcinoma [cylindroma] • Haemangioblastoma [capillary haemangio- • others blastoma] • Monstrocellular sarcoma Metastatic tumours Unclassified tumours Germ Cell Tumours • Germinoma • Embryonal carcinoma WHO Classification 1993 • Choriocarcinoma • Teratoma Kleihues P, Burger PC, Scheithauer BW: Histo- logical Typing of Tumours of the Central Ner- Other malformative tumours and tumour-like vous System. Springer Verlag, 1993, 2nd edition lesions • Craniopharyngioma Tumours of Neuroepithelial tissue • Rathke’ cleft cyst • Astrocytic tumours • Epidermoid cyst –– Astrocytoma • Dermoid cyst • Colloid cyst of the third ventricle ◦◦ Fibrillary • Enterogenous cyst ◦◦ Protoplasmic • Other cysts ◦◦ Gemistocytic • Lipoma –– Anaplastic (malignant) astrocytoma • Choristoma [pituicytoma, granular cell “myo- –– Glioblastoma blastoma”] ◦◦ Giant cell glioblastoma • Hypothalamic neuronal hamartoma ◦◦ Gliosarcoma • Nasal glial heterotopia [nasal glioma] –– Pilocytic astrocytoma –– Pleomorphic xanthoastrocytoma Vascular malformations –– Subependymal giant cell astrocytoma • Capillary telangiectasia (Tuberous sclerosis) • Cavernous angioma • Oligodendroglial tumours • Arteriovenous malformation –– Oligodendroglioma • Venous malformation –– Anaplastic (malignant) oligodendroglioma • Sturge-Weber disease [cerebrofacial or cere- • Ependymal and choroid plexus tumours brotrigeminal angiomatosis] –– Ependymoma WHO Classification 1993 2169 ◦◦ Cellular • Neurofibroma ◦◦ Papillary –– Circumscribed (solitary) ◦◦ Clear cell –– Plexiform –– Anaplastic (malignant) ependymoma • Malignant peripheral nerve sheath tumour –– Myxopapillary ependymoma (MPNST) –– Subependymoma –– Epitheloid • Mixed gliomas –– MPNST with divergent mesenchymal and/ –– Oligo-astrocytoma or epithelial differentiation –– Anaplastic (malignant) oligo-astrocytoma –– Melanotic –– Others • Chororid plexus tumours Tumours of the Meninges –– Choroid plexus papilloma • Tumours of meningothelial cells –– Choroid plexus carcinoma –– Meningioma • Neuroepithelial tumours of uncertain origin ◦◦ Meningothelial] –– Astroblastoma ◦◦ Fibrous (fibroblastic) –– Polar spongioblastoma ◦◦ Transitional (mixed) –– Gliomatosis cerebri ◦◦ Psammomatous • Neuronal and mixed neuronal-glial tumours ◦◦ Angiomatous –– Gangliocytoma ◦◦ Microcystic –– Dysplastic gangliocytoma of cerebellum ◦◦ Secretory (Lhermitte-Duclos) ◦◦ Clear cell –– Desmoplastic infantile ganglioglioma ◦◦ Chordoid –– Ganglioglioma ◦◦ Lymphoplasma-cyte rich –– Anaplastic (malignant) ganglioglioma ◦◦ Metaplastic –– Central neuroblastoma –– Atypical meningioma –– Paraganglioma of the filum terminale –– Papillary meningioma –– Olfactory neuroblastoma (Aesthesioneuro- –– Anaplastic (malignant) meningioma blastoma) • Mesenchymal, non-meningothelial tumours ◦◦ Olfactory neuroepithelioma –– Benign neoplasm • Pineal parenchymal tumours ◦◦ Osteocartilaginous tumours –– Pineocytoma ◦◦ Lipoma –– Pineoblastoma ◦◦ Fibrous histiocytoma –– Mixed/transitional pineal tumours ◦◦ Others • Embryonal tumours –– Malignant neoplasms –– Medulloepithelioma ◦◦ Hemangiopericytoma –– Neuroblastoma ◦◦ Chondrosarcoma ◦◦ Ganglioneuroblastoma ◦◦ Mesenchymal chondrosarcoma –– Ependymoblastoma ◦◦ Rhabdomyosarcoma –– Primitive neuroectodermal tumours (PNET) ◦◦ Meningeal sarcomas –– Medulloblastoma ◦◦ Others ◦◦ Desmoplastic medulloblastoma –– Primary melanocytic lesions ◦◦ Medullomyoblastoma ◦◦ Diffuse melanosis ◦◦ Melanotic medulloblastoma ◦◦ Melanocytoma ◦◦ Malignant melanoma Tumours of Cranial and Spinal Nerves ◦◦ Meningeal melanomatosis • Schwannoma –– Tumours of uncertain histogenesis –– Cellular ◦◦ Haemangioblastoma (capillary haeman- –– Plexiform gioblastoma) –– Melanotic 2170 Appendix A: WHO Classification of Tumors of the Central Nervous System Lymphomas and Haemopoietic Neoplasms WHO Classification 2000 • Malignant lymphoma • Plasmacytoma Kleihues P, Cavenee WK: World Health Organi- • Granulocytic sarcoma zation Classification of Tumours. Pathology & • Others Genetics. Tumours of the Nervous System. IARC Press, 2000, 3rd edition Germ Cell Tumours • Germinoma Tumours of Neuroepithelial tissue • Embryonal carcinoma • Astrocytic tumours • Yolk sac tumour (endodermal sinus tumour) –– Diffuse astrocytoma • Choriocarcinoma ◦◦ Fibrillary • Teratoma ◦◦ Protoplasmic –– Immature ◦◦ Gemistocytic –– Mature –– Anaplastic astrocytoma –– Teratoma with malignant transformation –– Glioblastoma • Mixed germ cell tumours ◦◦ Giant cell glioblastoma –– Gliosarcoma Cysts and tumour-like lesions –– Pilocytic astrocytoma • Rathke cleft cyst –– Pleomorphic xanthoastrocytoma • Epidermoid cyst –– Subependymal giant cell astrocytoma • Dermoid cyst (Tuberous sclerosis) • Colloid cyst of the third ventricle • Oligodendroglial tumours • Enterogenous cyst –– Oligodendroglioma • Neuroglial cyst –– Anaplastic oligodendroglioma • Granular cell tumour (Choristoma, Pituicytoma) • Mixed gliomas • Hypothalamic neuronal hamartoma –– Oligo-astrocytoma • Nasal glial heterotopias –– Anaplastic oligo-astrocytoma • Plasma cell granuloma • Ependymal and choroid plexus tumours –– Ependymoma Tumours of the Sellar Region ◦◦ Cellular • Pituitary adenoma ◦◦ Papillary • Pituitary carcinoma ◦◦ Clear cell • Craniopharyngioma ◦◦ Tanycytic –– Adamantinomatous –– Anaplastic ependymoma –– Papillary –– Myxopapillary ependymoma –– Subependymoma Local extensions from regional tumours • Choroid plexus tumours • Paraganglioma (chemodectoma) –– Choroid plexus papilloma • Chordoma –– Choroid plexus carcinoma • Chondroma • Glial tumours of uncertain origin • Chondrosarcoma –– Astroblastoma • Carcinoma –– Gliomatosis cerebri –– Chordoid glioma of the 3rd ventricle Metastatic tumours • Neuronal and mixed neuronal-glial tumours Unclassified tumours –– Gangliocytoma WHO Classification 2000 2171 –– Dysplastic gangliocytoma of cerebellum –– Epitheloid (Lhermitte-Duclos) –– MPNST with divergent mesenchymal and/ –– Desmoplastic infantile astrocytoma/gan- or epithelial differentiation glioglioma –– Melanotic –– Dysembryoplastic neuroepithelial tumour –– Melanotic psammomatous –– Ganglioglioma –– Anaplastic ganglioglioma Tumours of the Meninges –– Central neurocytoma • Tumours of meningothelial cells –– Cerebellar liponeurocytoma –– Meningioma –– Paraganglioma of the filum terminale ◦◦ Meningothelial • Neuroblastic tumours ◦◦ Fibrous (fibroblastic) –– Olfactory neuroblastoma (Aesthesioneuro- ◦◦ Transitional (mixed) blastoma) ◦◦ Psammomatous –– Olfactory neuroepithelioma ◦◦ Angiomatous –– Neuroblastoma of the adrenal gland and ◦◦ Microcystic sympathetic nervous system ◦◦ Secretory • Pineal parenchymal tumours ◦◦ Lymphoplasma-cyte rich –– Pineocytoma ◦◦ Metaplastic –– Pineoblastoma ◦◦ Clear cell –– Pineal parenchymal tumour of intermedi- ◦◦ Chordoid ate differentiation ◦◦ Atypical