脑肿瘤who新分类课件

World Health Organization classification of brain tumors: Its significance and problems,Yoichi Nakazato, M.D., Ph.D. Gunma University Graduate School of Medicine Department of Human Pathology The Third Invited Slide Seminar of Neuropathology March 25 26, 2006. Shanghai, China,Principal Lecture,Brain Tumors,Definition： Neoplasms arising in the cranial cavity A large number of histological types and subtypes The histological classification is essential for studies and treatment of brain tumors. Pathological diagnosis of brain tumors Assessment of the histogenesis and grading of malignancy,Glioblastoma showing a butterfly appearance,History of WHO classification,The aim： to provide a standardized system to facilitate communication between researchers worldwide. WHO Collaborating Center for CNS Tumors was established in 1970. The Director was K.J. Zlch. The Committee reviewed 230 cases, and made the WHO classification of tumors of the CNS. The first edition was published in 1979 as a “blue book”. In 1993, the book was revised as the second edition. IARC published the third edition of WHO classification as a book, “Pathology and Genetics of Tumours of the Nervous System” in 2000.,The Preuss Foundation Conference,July 25, 1999 IARC, Lyon, France,“Pathology and Genetics of Tumours of the Nervous System”,1997,2000,Features and advantages of WHO classification,A classification of tumors of the nervous system. Includes 126 types and subtypes. The book is not merely a simple chart of classification, but a compact handbook of tumors. Contains the most up-to-date findings of “Pathology and Genetics” of brain tumors. Has been spread worldwide, and highly admired. Influenced on other classifications.,Clear-cut grading,Astrocytoma G2: occasional nuclear atypia, mitotic activity is generally absent G3: increased cellularity, distinct nuclear atypia, marked mitotic activity G4: marked nuclear atypia, brisk mitotic activity, microvascular proliferation, and necrosis,Controversial points,Does not cover all intracranial tumors Much complicated Vagueness of the assessment Diagnosis depends on the location An inherent issue of the grading Issues specific to each tumor type A rush for new tumor entities,Does not cover all intracranial tumors,A classification of tumors of the nervous system. Entities deleted from the second edition Cysts and Tumor-like lesions, Local extensions from regional tumors, Unclassified tumors, Pituitary adenoma and carcinoma Includes extra-cranial tumors Tumors of the PNS, Adrenal neuroblastoma It is surely scientific, but not practical !,TUMOURS OF NEUROEPITHELIAL TISSUE Astrocytic tumours Diffuse astrocytoma Fibrillary astrocytoma Protoplasmic astrocytoma Gemistocytic astrocytoma Anaplastic astrocytoma Glioblastoma Giant cell glioblastoma Gliosarcoma Pilocytic astrocytoma Pleomorphic xanthoastrocytoma Subependymal giant cell astrocytoma Oligodendroglial tumours Oligodendroglioma Anaplastic oligodendroglioma Mixed gliomas Oligoastrocytoma Anaplastic oligoastrocytoma Ependymal tumours Ependymoma Cellular Papillary Clear cell Tanycytic Anaplastic ependymoma Myxopapillary ependymoma Subependymoma Choroid plexus tumours Choroid plexus papilloma Choroid plexus carcinoma Glial tumours of uncertain origin Astroblastoma Gliomatosis cerebri Chordoid glioma of the 3rd ventricle Neuronal and mixed neuronal-glial tumours Gangliocytoma Dysplastic gangliocytoma of cerebellum (Lhermitte-Duclos) Desmoplastic infantile astrocytoma/ganglioglioma Dysembryoplastic neuroepithelial tumour,Ganglioglioma Anaplastic ganglioglioma Central neurocytoma Cerebellar liponeurocytoma Paraganglioma of the filum terminale Neuroblastic tumours Olfactory neuroblastoma (Aesthesioneuroblastoma) Olfactory neuroepithelioma Neuroblastomas of the adrenal gland and sympathetic nervous system Pineal parenchymal tumours Pineocytoma Pineoblastoma Pineal parenchymal tumour of intermediate differentiation Embryonal tumours Medulloepithelioma Ependymoblastoma Medulloblastoma Desmoplastic medulloblastoma Large cell medulloblastoma Medullomyoblastoma Melanotic medulloblastoma Supratentorial primitive neuro- ectodermal tumour (PNET) Neuroblastoma Ganglioneuroblastoma Atypical teratoid/rhabdoid tumour TUMOURS OF PERIPHERAL NERVES Schwannoma (neurilemmoma, neurinoma) Cellular Plexiform Melanotic Neurofibroma Plexiform,Perineurioma Intraneural perineurioma Soft tissue perineurioma Malignant peripheral nerve sheath tumour (MPNST) Epithelioid MPNST with divergent mesen- chymal and/or epithelial differentiation Melanotic Melanotic psammomatous TUMOURS OF THE MENINGES Tumours of meningothelial cells Meningioma Meningothelial Fibrous (fibroblastic) Transitional (mixed) Psammomatous Angiomatous Microcystic Secretory Lymphoplasmacyte-rich Metaplastic Clear cell Chordoid Atypical Papillary Rhabdoid Anaplastic Mesenchymal, non-meningothelial tumours Lipoma Angiolipoma Hibernoma Liposarcoma (intracranial) Solitary fibrous tumour Fibrosarcoma Malignant fibrous histiocytoma Leiomyoma Leiomyosarcoma Rhabdomyoma Rhabdomyosarcoma,Chondroma Chondrosarcoma Osteoma Osteosarcoma Osteochondroma Haemangioma Epithelioid haemangioendothelioma Haemangiopericytoma Angiosarcoma Kaposi sarcoma Primary melanocytic lesions Diffuse melanocytosis Melanocyioma Malignant melanoma Meningeal melanomatosis Tumours of uncertain histogenesis Haemangioblastoma LYMPHOMAS AND HAEMOPOIETIC NEOPLASMS Malignant lymphomas Plasmacytoma Granulocytic sarcoma GERM CELL TUMOURS Germinoma Embryonal carcinoma Yolk sac tumour Choriocarcinoma Teratoma Mature lmmature Teratoma with malignant transformation Mixed germ cell tumours TUMOURS OF THE SELLAR REGION Craniopharyngioma Adamantinomatous Papillary Granular cell tumour METASTATIC TUMOURS,WHO Classification of Tumours of the Nervous System,Much complicated yet !,Vagueness of the assessment,Criteria for grading is rather subjective Oligodendroglioma anaplastic oligo. Ependymoma anaplastic ependymoma Pineocytoma PPT of intermediate differentiation pineoblastoma Ganglioglioma anaplastic ganglioglioma,Diagnosis depends on the location,Cerebellum medulloblastoma,Frontal lobe supratentorial PNET,Brain stem or spinal cord ?,An inherent issue of grading (1),WHO grading basically depends on the histological type. anaplastic ependymoma, WHO grade II anaplastic ependymoma, WHO grade III anaplastic ependymoma, WHO grade IV,An inherent issue of grading (2),Stepwise assessments against continuous phenomenon, such as atypia or malignancy.,MIB-1 LI in oligodendroglial tumors,Issues specific for each tumor,Does protoplasmic astrocytoma exist? What is Anaplastic pilocytic astrocytoma? Glioblastoma with oligo component? Atypical choroid plexus papilloma? Is ganglioneuroblastoma grade IV? Double standard for meningioma grading.,Protoplasmic astrocytoma,A rare tumor composed of neoplastic astrocytes with few flaccid processes and scant GFAP expression. Often shows mucoid degeneration and microcystic degeneration Does it really exist?,Protoplasmic astrocytoma, H.E.,Protoplasmic astrocytoma, GFAP,What is ?,Anaplastic pilocytic astrocytoma Glioblastoma with oligo component Atypical choroid plexus papilloma,Ganglioneuroblastoma,A rare tumor composed of both primitive appearing and terminally differentiated cells. It is classified into a subtype of supratentorial PNET. Is this tumor grade 4?,Neuroimaging,MRI T1 T1+Gd T2 Left temporal lobe tumor in a 32 years old man with long history of epilepsy,Ganglioneuroblastoma with lobular architecture,Small neuroblastic cells with mitotic figures,A ganglion cell intermixed with neurocytes,NeuN NFP70/200 NFP-1D,MIB-1,15.4%,1.0%,Chromogranin A,Grading of meningioma,In addition to the grading criteria, WHO committee stated that “meningiomas of any subtype or grade with high proliferation index and/or brain invasion” are considered to be high risk group. It is a double standard. Grade 1 meningioma with brain invasion could be G1 and also in high risk group!,Newcomers to brain tumor society,Papillary glioneuronal tumor Rosette-forming glioneuronal of the fourth ventricle Rosetted glioneuronal tumor Neuroblastic tumor with abundant neuropil and true rosettes Isomorphic astrocytoma Pilomyxoid astrocytoma Medulloblastoma with extensive nodularity,Papillary Glioneuronal Tumor,Komori T, et al. reported 9 cases. Am J Surg Pathol 22: 1171-1183, 1998 Adults (11 - 52 y.o.), sex：F5 : M4 Cerebral white matter： temp 5, pariet 2, front 2 Solid and cystic mass, 1.5 - 7 cm in diameter Pseudopapillary arrangement of neuronal and glial cells around blood vessels which show adventitial hyalinosis. Clinically benign.,Papillary glioneuronal tumor,Papillary glioneuronal tumor,GFAP,NeuN,OLIG2,Rosette-forming Glioneuronal Tumor of the 4th Ventricle,Komori T, et al. reported 11 cases. Am J Surg Pathol 26: 582-591, 2002. Age: children - adults，Sex: M4 : F7 Location: within or around the 4th ventricle Histology: 2 components Neurocytes consisting neurocytic/perivascular rosettes Astrocytic component resembled pilocytic astrocytoma Prognosis: fairly good,Rosette-forming Glioneuronal Tumor of the 4th Ventricle,42 y.o., female, multicystic cerebellar mass lesion,Rosette-forming Glioneuronal Tumor of the 4th Ventricle,Rosette-forming Glioneuronal Tumor of the 4th Ventricle,H.E. Synaptophysin GFAP,Rosetted Glioneuronal Tumor,Teo JG, et al. reported 4 cases. Am J Surg Pathol. 23: 502-510, 1999. Age: adults, Sex: F2 : M2, Cerebrum (spinal cord) Rosette-like islands of well differentiated small neurons and neuropil in diffusely infiltrating astrocytoma. Neurons occasionally show high MIB-1 index. Prognosis: unfavorable,Rosetted Glioneuronal Tumor,Infiltrating glioma with rosetted neuropil islands,Rosetted Glioneuronal Tumor,GFAP synaptophysin,Rosetted Glioneuronal Tumor,MIB-1 immunostaining,Pediatric Neuroblastic Brain Tumor Containing Abundant Neuropil and True Rosettes,Eberhart CG, et al. reported 7 cases Pediatr Dev Pathol 3: 346-352, 2000 Age: 1 3 y.o. Sex: F5 : M2, Cerebrum Clusters of small and medium-sized cells in the neuropil-like stroma True rosettes and perivascular pseudorosettes IHC: NFP(+), synaptophysin(+), GFAP() Prognosis: poor,Neuroblastic tumor with abundant neuropil and rosettes,Neuroblastic tumor with abundant neuropil and rosettes,Neuroblastic tumor with abundant neuropil and rosettes,Neu-N NFP,Isomorphic Astrocytoma,Blmcke I, et al. reported 19 cases Acta Neuropathol 107: 381-388, 2004 Diffuse astrocytoma with chronic seizures Infiltration of highly differentiated astrocytes showing low cellularity without mitotic figures IHC: GFAP(+), 1%MIB-1, glial MAP2(), CD34(), p53() Favorable prognosis, WHO grade I,Isomorphic Astrocytoma,MRI-T1 MRI-T1Gd MRI-T2,山形大学脳神経外科症例,Diffuse infiltration of glioma cells in the cortex,Slight hypercellularity of the white matter,GFAP MIB-1,Pilomyxoid Astrocytoma,Tihan T, et al. reported 18 cases JNEN 58: 1061-1068, 1999 Infants and young children Involved the hypothalamic/chiasmat