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Pathology of intracranial tumors lecture

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Information about Pathology of intracranial tumors lecture
Health & Medicine

Published on February 18, 2014

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Pathology of intracranial tumours- postgraduate lecture
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Pathology of intracranial tumours- a primer E.E.U. AKANG, MBBS, FMCPath, FWACP Consultant Pathologist, Department of Pathology, University College Hospital, Ibadan, Nigeria National Postgraduate Medical College of Nigeria 2014 Revision Course in Pathology FEBRUARY 14, 2014 LAGOS, NIGERIA

E k’abo! Welcome to Lagos!

Outline • Introduction • Historical background • • Classification of intracranial neoplasms • • Neuroepithelial • neoplasms • • Meningeal neoplasms • • Sellar region neoplasms • Germ cell neoplasms • • Lymphoid-Haematopoietic neoplasms Cranial/spinal nerve neoplasms Metastatic neoplasms Paediatric CNS neoplasms Clinical effects Intraoperative consultation Concluding remarks OBJECTIVES- Student should have a good grasp of the classification, clinical manifestations, gross and microscopic features of common intracranial neoplasms

Introduction 1- Definition Intracranial neoplasms are a diverse group of >130 primary CNS neoplasms, and numerous secondary neoplasms arising via either direct spread from neighbouring structures or haematogenous spread from distant sites Each tumour entity has distinctive biology, treatment and prognosis

Introduction 2- Epidemiology Mortality from CNS tumours in developed and developing nations is 3.6 and 2.9 per 100,000 yearly All intracranial neoplasms are potentially fatal (closed space and infiltrative properties) No known pre-malignant or in situ stages 2/3 of 10 neoplasms are gliomas 2/3 of gliomas are astrocytic 2/3 of adult neoplasms are supratentorial 2/3 of paediatric neoplasms are infratentorial Cerebral/spinal cord tumour ratio is up to 12:1 10 CNS neoplasms only very rarely metastasise Metastatic tumours > 10 CNS neoplasms

Introduction 3- Aetiopathogenesis Mostly sporadic, with cumulative mutations in oncogenes, tumour suppressor, DNA repair and apoptosis genes A few CNS neoplasms are familial (von Hippel Lindau, Turcot, Gorlin, Cowden, NF1, NF2, LiFraumeni) Clonal expansion of tumour stem cells 6

Outline • Introduction • Historical background

Historical background •CNS tumours were believed to be rare in native Africans up until half a century ago •Expanding general/specialist medical services have led to increasing recognition of CNS tumours •Most of the early reports from the sub region have been clinical neurosurgery series

Early African studies •1st CNS tumours reported from Africa were autopsy cases •Strachan, 1934- South Africa- less common in Bantus than other races •Davies, 1957- 6 gliomas among 2162 PMs- not uncommon •Jackson & Okubadejo, 196313 CNS tumours among 3489 PMs- uncommon

Ibadan- 1991-2007 Sahabi 2008, IbadanProgressive increase in CNS tumour rates 356 histologically confirmed CNS neoplasms264 (74.2%) adults & 92 (25.8%) children Gliomas most common, followed by meningiomas Female predominance in meningiomas; Relative decline of both metastases and choriocarcinoma

Outline • Introduction • Historical background • Classification of intracranial neoplasms

Classification and grading of intracranial neoplasms (WHO) Histological groups • Neuroepithelial • Meningeal • Sellar region • Germ cell • LymphoidHaematopoietic • Cranial/spinal nerve • Metastatic WHO grading •Grade 1- slow growing, non-malignant, with longterm survival •Grade 2- relatively slowgrowing, recurrent, progress to higher grade •Grade 3- malignant, recur as higher grade •Grade 4- very aggressive malignant neoplasms

Clinical categorization of CNS neoplasms INTRA-AXIAL Neuroepithelial tumours Metastatic tumours EXTRA-AXIAL • Intradural Meningioma Sellar region tumours CP angle tumours Nerve sheath tumours Metastatic tumours • Extradural Metastatic tumours

Outline • Introduction • Historical background • Classification of intracranial neoplasms • Neuroepithelial neoplasms

Neuroepithelial neoplasms • • • • • • • • Astrocytic tumours Oligoastrocytic tumours Oligodendroglial tumours Ependymal tumours Choroid plexus tumours Other neuroepithelial tumours Tumours of the pineal region Embryonal tumours (medulloblastoma, CNS Primitive Neuroectodermal Tumour/PNET, atypical teratoid/rhabdoid tumour)

Astrocytic neoplasms Grouped by: •Topography (supratentorial vs. infratentorial) •Differentiation (fibrillary, protoplasmic, gemistocytic) •WHO grade 1- (Pilocytic astrocytoma, chordoid glioma, desmoplastic astrocytoma, pituicytoma), 2- (Well-differentiated- low cellularity/ minimal pleomorphism, no vascular proliferation or necrosis), 3- (Anaplastic- or malignant- high cellularity, marked pleomorphism, no vascular proliferation or necrosis) 4- (Glioblastoma- high cellularity, marked pleomorphism with microvascular proliferation and/or necrosis) •Growth pattern (Expansile (WHO grade 1 and PXA- grade 2) vs. Diffuse (WHO grades 2 to 4))

Pilocytic astrocytoma WHO grade 1 neoplasms of childhood Occur in cerebellum, sellar region, brainstem and optic nerve Cerebellar neoplasms have an excellent prognosis Grossly cystic neoplasms with mural nodule Biphasic pattern (solid and microcystic foci with bipolar cells associated with Rosenthal fibres and eosinophil granular bodies)

Astrocytoma, grade 2 Low cellularity, mild pleomorphism, no vascular proliferation and no necrosis May progress to grade 3 astrocytoma Median survival 6 years; peak 5th decade of life Fibrillary astrocytoma Small stellate, elongated cells with fibrillary processes Gemistocytic astrocytoma Large, plump cells with abundant glassy eosinophilic cytoplasm and peripheral nuclei

Anaplastic astrocytoma, grade 3 Acquisition of additional mutations compared to grade 2 astrocytoma May progress to secondary glioblastoma High cellularity, significant pleomorphism, but no microvascular proliferation and no necrosis Median survival times of 2 years and peak in the 5th decade of life

Glioblastoma Grade 4 neoplasm showing cellularity, pleomorphism and vascular proliferation and or necrosis Peak in 6th decade, but any age. Median survival of 1 year

Glioblastoma - 2 molecular pathways 21

Oligodendroglioma Hemispheric gliomas of young/middle aged adults Frontal, temporal, parietal and occipital lobes in ratio 3:2:2:1 Calcification on X-ray/CT Histology shows uniform cells with perinuclear haloes (fried egg) with chicken-wire capillaries. Better prognosis than astrocytoma (mean survival- 20yr grade 2 and 10yr grade 3). Surgery, chemotherapy, and radiotherapy Del 1p/19q has good prognosis

Cancer stem cell theory applied to gliomas (Louis, 2006)

Ependymoma Most sporadic; few familial in type 2 neurofibromatosis Arise from lining of fourth (children) and lateral (adult) ventricles. Commonest spinal cord neoplasms Grade 1- Subependymoma: rare incidental tumour; and myxopapillary ependymoma: cauda equina Grade 2- well differentiated ependymoma Grade 3- anaplastic ependymoma

Ependymoma Histological features: 1. Perivascular pseudorosettes 2. True rosettes with central lumen having a limiting membrane Ciliary basal bodies (blepharoplasts) can be demonstrated on electron microscopy

EpendymomaBony (left) and cartilaginous (right) metaplasia

Anaplastic ependymomaA- Perivascular pseudorosettes, B- ependymal true rosettes, Cpapillary differentiation, D- microvascular proliferation, Epseudopalisading necrosis A C E B D

Choroid plexus neoplasms Choroid plexus papilloma (CPP) Grade 1 childhood tumours of lateral ventricle. Grossly, pink cauliflower like mass Cause hydrocephalus mainly by obstruction of flow, but also rarely, by overproduction of CSF. Histologically are papillary neoplasms that recapitulate normal choroid plexus Atypical CPP- Grade 2 CP carcinoma- Grade 3 Hyperdiploidy, multiple gains

Medulloblastoma Malignant, invasive embryonal cerebellar tumour of children with neuronal differentiation, and tendency to spread via CSF Commonest malignant paediatric CNS neoplasm Most are sporadic but familial cases with nevus basal cell carcinoma (Gorlin) or Turcot syndromes may occur Del 17p and isochromosome 17q PTCH (Gorlin), APC (Turcot) Variants- classical, large cell, anaplastic, desmoplastic

Outline • Introduction • Historical background • Classification of intracranial neoplasms • Neuroepithelial neoplasms • Meningeal neoplasms

Meningioma Origin from meningothelial arachnoid cells of convexities, parafalcine, sphenoid, olfactory and suprasellar regions, optic nerve and choroid plexus Commoner in blacks (30-40%) than Caucasians (10-15%) Females (steroid receptors) Most sporadic; Familial in NF2 Recurrence rate is 11% WHO grade 1- benign (del 22q) Grade 2- atypical (del 14q) Grade 3- malignant (del 1p) Co-express EMA and vimentin

Meningeal neoplasms Sahabi, 2008 Transitional Meningothelial Fibroblastic Psammomatous Secretory Angiomatous Atypical Anaplastic WHO GRADE I II III 45 0 0 21 0 0 8 0 0 5 0 0 3 0 0 2 0 0 0 3 0 0 0 7 Haemangiopericytoma 0 3 1 4 Haemangioblastoma 3 87 0 6 0 8 3 101 HISTOLOGICAL HISTOLOGICAL TYPES SUBTYPES Meningioma Mesenchymal tumours Other TOTAL TOTAL 45 21 8 5 3 2 3 7

Outline • Introduction • Historical background • Classification of intracranial neoplasms • Neuroepithelial neoplasms • Meningeal neoplasms • Sellar region neoplasms

Pituitary adenoma Sellar neoplasms are commoner in Nigeria (20-28%), than other parts of Africa (7.5-13.4%) Pituitary adenomas comprise 2/3 of sellar neoplasms Clinically manifest with 1. Mass effect (headache, vomiting, papilloedema) 2. Bitemporal hemianopsia 3. Pituitary hormone dysfunction (panhypo- or selected hyper- function) Best grouped by immune or by EM Atypical adenoma and pituitary carcinoma are uncommon

Craniopharyngioma Awelimobor, 2011 Majority originate from Rathke cleft remnants. Commonest childhood sellar region tumour. Common in Nigeria and Japan. Benign cystic locally invasive neoplasms with dark oily fluid and calcific material. Epithelial islands with peripheral palisading and central stellate reticulum. Express steroid receptors. Mutation of betacatenin (CTNNB1) gene. Recurrence in 10-62%

Outline • Introduction • Historical background • Classification of intracranial neoplasms • Neuroepithelial neoplasms • Meningeal neoplasms • Sellar region neoplasms • Germ cell neoplasms

Germ cell neoplasms Origin from aberrant migrating germ cells in midline (pineal and suprasellar regions) Rare (except in Taiwan and Japan) Germinoma Teratoma (mature, immature, with malignant transformation) Yolk sac tumour Embryonal carcinoma Choriocarcinoma Mixed germ cell tumour

Outline • Introduction • Historical background • Classification of intracranial neoplasms • Neuroepithelial neoplasms • Meningeal neoplasms • Sellar region neoplasms • Germ cell neoplasms • LymphoidHaematopoietic neoplasms

Lymphoid-haematopoietic neoplasms CNS may be involved in advanced systemic NHL (e.g. Burkitt), Hodgkin disease, plasmacytoma or myeloma Primary CNS lymphoma (PCNSL) occurs in both immunosuppressed and immunocompetent individuals NHL most common (particularly high grade B cell NHL) EBV associated with PCNSL May present as solitary or multiple parenchymal nodules or with diffuse meningeal infiltration

Outline • Introduction neoplasms • Historical background • Cranial/spinal nerve neoplasms • Classification of intracranial neoplasms • Neuroepithelial neoplasms • Meningeal neoplasms • Sellar region neoplasms • Germ cell neoplasms • LymphoidHaematopoietic

Vestibular schwannomaCerebellopontine angle Vestibular part of VIII nerve Sporadic (unilateral in 95% of cases) or familial (bilateral in NF2) Cellular (Antoni A) and loose (Antoni B) areas Antoni A Antoni B

Outline • Introduction neoplasms • Historical background • Cranial/spinal nerve neoplasms • Classification of intracranial neoplasms • Metastatic neoplasms • Neuroepithelial neoplasms • Meningeal neoplasms • Sellar region neoplasms • Germ cell neoplasms • LymphoidHaematopoietic

Metastatic CNS neoplasms Outnumber 10 CNS neoplasms by ratio of 10:1 4-30% of intracranial neoplasms in PM series Primary sources include choriocarcinoma, lung, breast, prostate, soft tissue, lymphoid, GIT and female genital tract malignancies Usually multiple and located at grey-white matter junction

Outline • Introduction neoplasms • Historical background • Cranial/spinal nerve neoplasms • Classification of intracranial neoplasms • Metastatic neoplasms • Neuroepithelial • Paediatric CNS neoplasms neoplasms • Meningeal neoplasms • Sellar region neoplasms • Germ cell neoplasms • LymphoidHaematopoietic

Paediatric CNS neoplasms •ASR is 25-40 per 106 in developed and <15 per 106 in developing nations •Due partly to under ascertainment and partly to racial factors •Previous Nigerian and African series have shown prominence of astrocytoma, medulloblastoma, ependymoma and craniopharyngioma •There is need for population based registries to assess true burden of these neoplasms, particularly in Africa

Intracranial Tumours: adults vs. children •ADULTS: 2/3 supratentorial –meningioma –pituitary adenoma –malignant astrocytoma/glioblastoma •CHILDREN: 2/3 posterior fossa –pilocytic astrocytoma –medulloblastoma –ependymoma

Outline • Introduction • Historical background • Classification of intracranial neoplasms • Neuroepithelial neoplasms • Meningeal neoplasms • Sellar region neoplasms • Germ cell neoplasms • LymphoidHaematopoietic • • • • neoplasms Cranial/spinal nerve neoplasms Metastatic neoplasms Paediatric CNS neoplasms Clinical effects

Clinical effects •Clinical effects of intracranial neoplasms are those of raised ICP (headache, projectile/effortless vomiting and papilloedema) and are modified by anatomical location of the neoplasm •There may be seizures, localizing signs, behavioural abnormalities, cognitive deficits and endocrinopathies

HERNIATIONS A- cingulate B- uncal C- tonsillar

With bilateral uncal herniation there is stretching and then rupture of penetrating branches of the basilar artery supplying the midbrain and upper pons

Outline • Introduction • Historical background • Classification of intracranial neoplasms • Neuroepithelial neoplasms • Meningeal neoplasms • Sellar region neoplasms • Germ cell neoplasms • LymphoidHaematopoietic • • • • • neoplasms Cranial/spinal nerve neoplasms Metastatic neoplasms Paediatric CNS neoplasms Clinical effects Intraoperative consultation

Intraoperative consultation A primary role of pathologist in patient management -Gross examination -Frozen section -Cytology Awelimobor 2011100% concordance rate between cytological and frozen section diagnosis, as well as between frozen section and routine paraffin embedded tissue diagnosis

Glioblastoma- cytology, Frozen section and permanent section Awelimobor, 2011

Pituitary adenoma- cytology and frozen section Awelimobor, 2011

Meningioma- cytology, Frozen section and permanent section Awelimobor, 2011

Outline • Introduction • Historical background • Classification of intracranial neoplasms • Neuroepithelial neoplasms • Meningeal neoplasms • Sellar region neoplasms • Germ cell neoplasms • LymphoidHaematopoietic • • • • • • neoplasms Cranial/spinal nerve neoplasms Metastatic neoplasms Paediatric CNS neoplasms Clinical effects Intraoperative consultation Concluding remarks

Concluding remarks •Biological characteristics of intracranial neoplasms have been discussed •These neoplasms may originate from a. Brain structure and its coverings b. Neighbouring tissues by direct invasion c. Distant sites by haematogenous spread • Clinical effects are those of raised ICP and are modified by the anatomical location of the neoplasm

Further reading (1) J Neurol Sci (Turk) 2007;24(3):212 -8. (2) CA Cancer J Clin 2011;61(2):69-90. (3) West Afr Med J Niger Pract 1967;16(1):31 42. (4) Asian Pac J Cancer Prev 2008;9(2):267 -70. (5) Neurosurgery in Africa. Ibadan: Ibadan University Press; 1989. (6) West Afr Med J 1963;12:251-63. (7) Cancer 1980;46(10):2322-4. (8) East Afr Med J 2000;77(1):4-8. (9) National Postgraduate Medical College of Nigeria; 2008. (10) Arch Pathol Lab Med 2009;133(1):78-82. (11) Pediatr Pathol Lab Med 1996;16(5):791 800. (12) Ann Trop Paediatr 2002;22(2):159-63. (13) J Neurol Neurosurg Psychiatry 2004;75 Suppl 2:ii2-11. (14) WHO Classification of Tumours of the Central Nervous System. Lyon: IARC; 2007. (15) WHO Classification of Tumours of the Central Nervous System. Lyon: IARC; 2013. (16) Afr J Med Sci 1973;4(2):143-59. (17) Afr J Med Sci 1973;4(2):99-106. (18) Afr J Med Sci 1973;4(2):178-86. (19) J Natl Cancer Inst 1975;55(2):281-4. (20) J Clin Neurosci 2006;13(6):649-54. (21) West Afr Med J 1963;12:251-63. (22) Afr J Med Med Sci 1976;5(3):181-4. (23) Cancer in Africa: epidemiology and prevention. Lyon: IARC Press; 2003. (24) Childs Nerv Syst 1985;1(1):39-44. (25) Childs Nerv Syst 2010;26(8):1021-7. (26) Trop Doct 2004;34(4):223-5. (27) Robbins and Cotran pathologic basis of disease. Philadelphia: Saunders Elsevier; 2010. (28) Robbins basic pathology. Philadelphia: Saunders Elsevier; 2013. (29) Greenfield’s neuropathology. London: Edward Arnold; 2008. (30) Escourolle and Poirier manual of basic neuropathology. Philadelphia: Butterworth Heinemann, 2004. (31) Modern surgical neuropathology. Cambridge: Cambridge University Press, 2009. (32) Neuropathology. Pathology Articles. Medscape. http://emedicine.medscape.com/pathology

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