General Pathology Histogenetic Classification of Neoplasms Neuroectodermal Other

NEOPLASIA – classification HISTOGENETIC v v v mesenchymal epithelial neuroectodermal mixed, teratoma choriocarcinoma mesothelioma

Neuroectodermal Tumours derived from: CNS & PNS located v ganglion cells v glial &

WHO Clasification of Tumours of the Nervous System (WHO 2000) - 1. v Glial

WHO Clasification of Tumours of the Nervous System (WHO 2000) - 2. v Tumours

WHO Clasification of Tumours of the Nervous System (WHO 2000) - 3. v Lymphomas

Gangliocytoma Def. : well differentiated slowly growing neuroepithelial tumour composed of neoplastic mature ganglion

Neuroblastoma (WHO: Neuroblastic tumours of adrenal gland sympathetic nervous system) Def. : childhood embryonal

Paraganglioma Def. : benign neuroendocrine neoplasm arising in specialised neural crest cells associated with

Phaeochromocytoma Def. : benign tumour of of chromaffin cells (intraadrenal paraganglioma) Macro: whittish, solid,

Neurilemmoma (WHO Schwannoma) Def. : a usually encapsulated benign tumour composed of differentiated neoplastic

Neurofibroma Def. : a well demarcated intraneural or diffusely infiltrative extraneural tumour consisting of

Melanocytic Skin Lesions - 1. v Freckle – ephelis – a hyperpigmented macule due

Melanocytic Skin Lesions - 2. v Acquired melanocytic nevi – junctional – dermal –

Man 56 yrs v progressive N 416/02 neurological symptoms v brain CT negative v

Diagnosis Morbus principalis: Melanoma malignum originis ignotae generalisatum. Complicationes: Metastases tumorosae multiplices cerebri, medullae

Diagnosis Causa mortis: Generalisatio tumorosa. Oedema cerebri. Inventus accessorius: Anthracosis pulmonum et lnn. thoracalium.

NEOPLASIA – classification HISTOGENETIC v v v mesenchymal epithelial neuroectodermal mixed, teratoma choriocarcinoma mesotelioma

Other Tumours v v mixed tumours trophoblast choriocarcinoma v teratoma coetaneous – differentiated embryonal

Mixed Tumours Def. : Tumours (benign or malignant) composed of two or more different

Teratomas Def. : Tumours (benign or malignant) composed of two or more different cell

Germ cell tumours – germinoma – embryonal sarcoma – yolc sac tumour – choriocarcinoma

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General Pathology Histogenetic Classification of Neoplasms Neuroectodermal & „Other“ Neoplasms Jaroslava Dušková Inst. Pathol. , 1 st Med. Faculty, Charles Univ. Prague http: //www 1. lf 1. cuni. cz/~jdusk/

NEOPLASIA – classification HISTOGENETIC v v v mesenchymal epithelial neuroectodermal mixed, teratoma choriocarcinoma mesothelioma

Neuroectodermal Tumours derived from: CNS & PNS located v ganglion cells v glial & Schwann cells v mixed (ganglion and glial cells) v gangliocytoma, neuroblastoma gliomas, neurilemmoma glioblastoma, neurog. sarcoma, ganglioma, ganglioneuroma melanocytes pigmented nevi melanoma

WHO Clasification of Tumours of the Nervous System (WHO 2000) - 1. v Glial cell tumours v astrocytic v oligodendroglial v mixed (oligo-astro) v ependymal v choroid plexus v glial tumours of uncertain origin v v Neuronal and mixed neuronal-glial tumours Neuroblastic Pineal parenchymal tumours Embryonal tumours

WHO Clasification of Tumours of the Nervous System (WHO 2000) - 2. v Tumours of peripheral nerves v v v Schwannoma (neurilemmoma) Neurofibroma Perineurioma Malignant peripheral nerve sheet tumour (MPNST) Tumours of the meninges v v v Tumours of meningotelial cells – meningiomas Mesenchymal non-meningothelial tumours Primary melanocytic lesions

WHO Clasification of Tumours of the Nervous System (WHO 2000) - 3. v Lymphomas and haemopoietic neoplasms v malignant v v v Germ cell tumours v v v germinoma embryonal sarcoma yolc sac tumour choriocarcinoma teratoma (mature, immature) Tumours of the sellar region v v v lymphomas plasmocytoma granulocytic sarcoma craniopharyngioma granular cell tumour Metastatic tumours

Gangliocytoma Def. : well differentiated slowly growing neuroepithelial tumour composed of neoplastic mature ganglion cells Age /sex – no special predisposition (diagnosed mostly in childhood – young adults) Incidence: RARE Histogenesis: most probably highly differentiated remnants of embryonal neuroblasts

Neuroblastoma (WHO: Neuroblastic tumours of adrenal gland sympathetic nervous system) Def. : childhood embryonal tumours of migrating neuroectodermal cells derived from the neural crest and destined for the adrenal medulla and sympathetic nervous system Age /sex – 96% in the 1 st decade , no sex predilection Incidence: most common solid extracranial malignant tumours during the first two years of life Histogenesis: see definition Clinic: palpable mass (retroperit, abd. , cervical), X-ray thoracic Macro: soft gray-tan mass, regressive changes Micro: undiff. + differentiating neuroblasts Variants: neuroblastoma (undiff. ), ganglioneuroblastoma intermixed, ganglioneuroblastoma nodular, ganglioneuroma Behaviour: malignant, dependent on age and histology variant

Paraganglioma Def. : benign neuroendocrine neoplasm arising in specialised neural crest cells associated with autonomic ganglia Macro: encapsulated , solid Micro: uniform cells forming compact nests (Zellballen) Biology: benign

Phaeochromocytoma Def. : benign tumour of of chromaffin cells (intraadrenal paraganglioma) Macro: whittish, solid, regressive changes Micro: solid alveolar (Zellballen) Behaviour: benign (15% bilateral, 10% in children, 10% malignant) MEN II and von Hippel-Lindau disease component

Neurilemmoma (WHO Schwannoma) Def. : a usually encapsulated benign tumour composed of differentiated neoplastic Schwann cells Age /sex – all ages, peak 4 -6 th decade , no sex predilection Incidence: common solid head, neck, extremities, INTRACRANIAL intramedullary Histogenesis: see definition Clinic: periph. - palpable asymptomatic mass, intraspinal – pain, intracranial – cerebellopontine lesion symptoms – eharing, tinnitus, facial paresthesias Macro: white, soft – firm, encapsulated, (+nerve) Micro: elongated Schwann cells , palisading, Verocay bodies Variants: Antoni A, B, biphasic, cellular, pigmented, plexiform Behaviour: slowly growing, benign, malignant transformation rare

Neurofibroma Def. : a well demarcated intraneural or diffusely infiltrative extraneural tumour consisting of a mixture of cell types including Schwann cells, perineuriallike cells, and fibroblasts. Multiple in Nerofibromatosis 1. Age /sex – all ages, no sex predilection Incidence: common Clinic: palpable asymptomatic mass, mostly cutaneous nodule (s) ass. with café-au-lait spots Macro: white, firm, circumscribed Micro: elongated Schwann cells , fibroblasts, Wagner-Meissner-like tactile corpuscles Variants: atypical, cellular, plexiform, may be pigmented Behaviour: slowly growing, benign, malignant transformation rare – mostly in plexiform variants

Melanocytic Skin Lesions - 1. v Freckle – ephelis – a hyperpigmented macule due to increased melanin amount in a normal density of melanocytes v Lentigo simplex - small well circumscribed hyperpigmentation due to increased frequency of basal melanocytes v Café-au-lait spots – hyperpigmented keratinocytes. In neurofibromatosis present at birth. v Naevus spilus (congenital) – up to 10 cm in diam. lentiginous and JUNCTIONAL foci v Dermal melanocytoses – blue spots Ota, melanocytic hamartomas) (mongolian, n. Ito, n.

Melanocytic Skin Lesions - 2. v Acquired melanocytic nevi – junctional – dermal – compound – deep dermal (blue) nevus v Congenital melanocytic nevus

Man 56 yrs v progressive N 416/02 neurological symptoms v brain CT negative v CSF – no signs of inflammation – scattered atypical cells – neoplasm? – systemic degeneration?

Diagnosis Morbus principalis: Melanoma malignum originis ignotae generalisatum. Complicationes: Metastases tumorosae multiplices cerebri, medullae spinalis, myocardii et renum. Haemorrhagia lobi frontalis hemisphaerii sin. cerebri. Endocardiosis marantica valvae mitralis. Infarctus recentes aliquot lienis, renum et cerebri.

Diagnosis Causa mortis: Generalisatio tumorosa. Oedema cerebri. Inventus accessorius: Anthracosis pulmonum et lnn. thoracalium. Urocystitis heamorrhagica acuta. Struma colloides nodosa gl. thyreoideae. Cholesterolosis vesicae felleae.

NEOPLASIA – classification HISTOGENETIC v v v mesenchymal epithelial neuroectodermal mixed, teratoma choriocarcinoma mesotelioma

Other Tumours v v mixed tumours trophoblast choriocarcinoma v teratoma coetaneous – differentiated embryonal – nondifferentiated v mesothelioma fibromesothelioma, adenomatoid tu. biphasic (mesot. ca)

Mixed Tumours Def. : Tumours (benign or malignant) composed of two or more different cell lines that are normally present in the place of tumour origin

Teratomas Def. : Tumours (benign or malignant) composed of two or more different cell lines that are NOT normally present in the place of tumour origin

Germ cell tumours – germinoma – embryonal sarcoma – yolc sac tumour – choriocarcinoma – teratoma (mature, immature)