CNS Tumors

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If we are able to step back calmly from
situations and simply observe without judgment or
criticism, we will begin to know the truth.
BK.
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Pathology of CNS Tumors

• Dr. Venkatesh M. Shashidhar, Associate Professor
  of Pathology
• Fiji School of Medicine

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General Considerations

• Common childhood tumor.
• Comprise 10 of all tumors
• Peak incidence at 5th decade
• Supratentorial tumors in adults
• Infratentorial tumors in childhood

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General Considerations

• Primary children - 50 infiltrative
• Metastatic adults - well-demarcated.
• Limited space, Vital structures
• Location determines prognosis.
• Rare extra neural metastasis.

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Classification

• Primary neural tumors - Rare (Neuroblastoma,
  ganglioneuroma)
• Common - Tumors of supporting structures.
• Glia, Meninges, Blood vessels, fibrous tissue.
• Secondary Metastasis (50)

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Types of Brain Tumors

• Meninges meningioma, hemangiopericytoma
• Glia astrocytoma, oligodendroglioma, ependymoma,
  choroid plexus papilloma..
• Vascular hemangioblastoma.
• Primitive cells neuroblastoma, germinoma,
  medulloblastoma, pineoblastoma, retinoblastoma
• Neuronal ganglioglioma, gangliocytoma
• Pituitary adenoma, craniopharyngioma
• Nerves schwannoma, neurofibroma, MPNST

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Meningioma

• Arise from meningothelial cells of arachnoid
  granulations.
• Adjacent to venous sinuses.
• Nodular, capsulated, slow growing-Benign.
• Form whorls of cells, Psammoma bodies in the
  center.
• Effect by pressure.
• No infiltration or metastasis (Benign).

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Meningioma
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Meningioma
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Meningioma
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Meningioma
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Menoingioma
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Meningioma
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Meningothelial whorls
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Meningothelial whorls
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Psammoma bodies in meningioma
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Glioma

• Gliomas are neoplasms of glial cells.
• Astrocytoma Commonest benign tumor with
  malignant behavior.
• Ependymoma Rare, 4th ventricle.
• Oligodendroglioma Benign, adults, rare.

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Astrocytoma

• Fibrillary or diffuse astrocytoma - 80
• Anaplastic/high grade astrocytoma
• Glioblastoma multiforme
• Gemistocytic astrocytoma
• Pilocytic astrocytoma(Juvenile)
• Pleomorphic astrocytoma
• Xanthastrocytoma
• Gliomatosis cerebri. Others..

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Astrocytoma

• Cerebrum, 4th to 6th decade.
• Headache, seizures neurological deficits.
• 3 tier or 4 tier grading system.
• Anaplasia,
• Mitotic activity,
• Necrosis
• Endothelial proliferation.
• Well differentiated, anaplastic Glioblastoma
  multiforme.

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Astrocytomas
Adults Childhood
SupratentorialSolidMalignantFibrillary Infrat
entorialCysticBenignPilocytic
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Fibrillary astrocytoma microscopic

• Low grade- hypercellularity, pleomorphism
• Anaplastic- high grade plus will have more
  mitosis vascular endothelial proliferation
• Glioblastoma multiforme- plus necrosis and
  pseudopalisades. Grossly variegated appearance
  (multiforme)

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Glioma Brain Stem
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Glioma Cerebrum
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Glioma Rosenthal fibres.
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GBM
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Glioma Cerebrum
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Glioblastoma Multiforme
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Glioblastoma Multiforme
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GBM- IPx Positive for GFAP
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Glioblastoma Multiforme
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Glioma
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Glioma
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Glioma
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Astrocytoma
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Astrocytoma
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Glioblastoma - pseudopalisade
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Glioblastoma Multiforme
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Glioblastoma Multiforme
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Glioblastoma Multiforme
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"The gem cannot be polished without friction, nor
man perfected without trials or problems or
exams!." --Chinese proverb
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Pilocytic astrocytoma

• Common in childhood
• Most slow growing of the gliomas
• Sites cerebellum, around III V., optic nerve
• Grossly cystic with mural nodule
• Microscopic
• elongated hair-like (pilo) elongated cells
• Rosenthal fibers

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Rosenthal fiber definition

• Dense, eosinophilic fibers within cytoplasmic
  processes of astrocytes.
• Correspond to aggregate accumulation of
  intermediate filaments in these processes.

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Pilocytic Astrocytoma
"juvenile astrocytomas, cystic Most common in
children. below the tentorium, posterior fossa.
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Pilocytic astrocytoma Mural nodule
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Oligodendroglioma

• Cells of origin Oligodendrocytes
• Common in cerebral hemispheres
• Calcifications common among all gliomas
• Grades Low grade Anaplastic

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Oligodendroglioma
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Oligodendroglioma
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Pilocytic astrocytoma
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Ependymoma-hemorrhage
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Ependymoma Cerebellum
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Spinal Ependymoma
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Ependymoma
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Ependymoma - Myxopapillary
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Neuroectodermal Tumors

• Origin from primitive blast cells.
• Rosettes - attempted nerve formation.
• Medulloblastoma Cerebellum
• Retinoblastoma - Retina
• Neuroblastoma Adrenal glands
• Ganglioneuroma - Mediastinum

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Medulloblastoma

• Origin primitive neuroectodermal cells
• Age 1st decade of life. Most common brain tumor
  at this age.
• Site vermis of cerebellum
• May cause hydrocephalus
• Subarachnoid dissemination

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Medulloblastoma
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Medulloblastoma
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Homer-Wright rosettes
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Colloid cyst III Ventricle
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Medulloblastoma
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Ganglioneuroma
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The ability to listen, understand and empathise
creates a feeling of trust and friendship in
others. If we are always willing to listen, we
can help them discover their own solutions to the
problems they have to face. BK.
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Peripheral Nerve(Sheath) Tumors

• Neurofibroma
• Schwannoma

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Nerve Sheath Tumors

• Neurofibroma
• Epi endoneurial fibroblasts.
• Form whorls of fibroblasts
• Well differentiated, benign,
• Two types
• Classic form - Cutaneous / nerve - Solitary
  collagen matrix, spindle cells,
• Plexiform - Multiple, infiltrative, myxoid.

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Nerve Sheath Tumors

• Schwannoma
• Schwann cells, form whorls
• Nuclear palisading
• Antoni A B pattern.
• Verocay bodies.

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Schwannoma
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Neurilemmoma
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Schwannoma/Acoustic neuroma
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Schwannoma
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Antony A - Antony B
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Antony B
Antony A
Palisades
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Schwannoma Cerebellum
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Neurofibromatosis

• Type I (common)(AD, 17q, 13000)
• Plexiform solitary neurofibromas
• Optic nerve gliomas, Lisch nodules, Café au lait
  spots.
• Type II (rare)(22q, 140,000)
• Bilateral acoustic schwannoma/osis
• Multiple meningioma/osis, ependymoma of spinal
  cord

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Phakomatosis (Neurocut. dysplasia)

• Neurologic abnormalities defects of skin or
  retina (ectodermal).
• Neurofibromatosis (von Recklinghausen)
• Tuberous Sclerosis
• Sturge-Weber Sy (Encephalofacial Angiomatosis)
• von Hippel-Lindau Disease
• Neurocutaneous Melanosis

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Neurofibromatosis - Von Recklinghausen

• Dominant inheritance
• Multiple neurofibromas
• Central - CNS
• peripheral nerves
• Increased incidence of
• meningioma
• glioma
• schwannoma - bilateral VIII N.
• Cafe-au-lait (melanosis) in skin
• Elephantiasis increased connective tissue

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Von Recklinghausens Disease
Café-au-lait spots
Multiple neurofibromas
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Tuberous Sclerosis
1. Dominant inheritance 2. Clinical
triad seizures mental retardation adenoma
sebaceum 3. Retinal hamartoma (phakoma) 4.
Tubers in cerebral cortex 5. Subependymal giant
cell astrocytoma 6. Hamartomas in other organs
heart, kidney
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Tubers
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Adenoma sebaceum
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Von Recklinghausen Disease
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Neurofibromatosis Type II

• Bil Schwannomas
• Meningiomas
• Gliomas
• Pheochromocytomas

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Von Recklinghausen Disease
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Plexiform Neurofibroma
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Café-au-lait spots
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Plexiform Neurofibroma
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Malignant Neurofibroma
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Medulloblastoma
"small round blue cell" tumors and it most often
occurs in children.
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Brain Metastasis - (lung)
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Metastatic tumors
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Brain Metastasis
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Peripheral nerve tumors

• Neurofibroma
• Schwann cells, neurites, fibroblasts
• Fusiform and involves nerve trunk
• Not encapsulated
• Not resectable without sacrificing nerve
• Micro- Intermingled cells with wavy nuclei

• Schwannoma
• Schwann cells
• Compress the nerve trunk
• Encapsulated
• Easily resectable without nerve damage
• Microscopic
• Antony A and B fibers
• Verocay bodies

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AcousticSchwannoma
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Traumatic Neuroma
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Brain Tumors Microscopic
Tumor
Microscopic Meningioma Whorls and
psammoma bodies Glioblastoma
Pseudopalisades Oligodendroglioma
Mosaic/poached-egg Ependymoma
Perivascular pseudorosettes Medulloblastoma
Rosettes (Homer-Wright)
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Thank You

• Dr. Venkatesh M. Shashidhar
• Senior Lecturer in Pathology
• Fiji School of Medicine

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Embryonal tumors(Primitive neuroectodermal
tumors)

• Neuroblastoma - cerebral hemispheresNeuroblastic
  (neuronal) differentiation
• Medulloblastoma - cerebellumNeuronal and glial
  differentiation
• Ependymoblastoma ventriclesEpendymal
  differentiation
• Pineoblastoma- pineal regionRetinoblastic
  differentiation
• All commonly known as Primitive neuroectodermal
  tumor (PNET)

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Histologic patterns definitions

• Whorls onion-skinning pattern of tumor cells
• Psammoma bodies laminated calcium
• Pseudopalisading lining up of the tumor cells
  around a central necrotic area
• Palisade lining up of tumor cells around their
  own cytoplasmic processes. No necrosis.
• Pseudorosette tumor cells around blood vessels,
  cells equidistant from vessel walls.
• Rosettes tumor cells around central lumen or
  fibrillary area of cellular processes

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Tumors of Nerve Rootsand Peripheral Nerves
1. Schwannoma viii Cranial nerve (Acoustic
sch.) Spinal roots, posterior Peripheral
nerves 2. Neurofibroma Spinal Roots,
rare Peripheral nerves 3. Malignant
variants Malignant peripheral nerve sheath
tumor (MPNT) Rare
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Metastatic brain tumors

• Most common brain tumor in adults.
• Common primary sites melanoma, lung, breast, GI
  tract, kidney.
• Most are in cerebrum (MCA territory).
• In gray-white junctions due to rich capillarity
• Discrete, globoid, sharply demarcated tumors.
  Amenable to surgical resection.
• Single or multiple.
• Brain edema frequent.