Brain Neoplasms: General Considerations

1. Comprise: 10% of all tumors 2. Most common childhood neoplasms 3. Peak incidence at 5th decade 4. Supratentorial tumors in adults 5. Infratentorial tumors in childhood

Brain Neoplasms: General Considerations

6. Different tumors in different ages
7. Primary tumors infiltrative, metastatic well-demarcated 8. Intraneural seeding occur, but no extraneural metastasis 9. Produce neurologic symptoms by size, location, invasiveness, and secondary effects

Varieties of brain tumors

Meninges: meningioma, hemangiopericytoma Astrocytes: astrocytoma (various types) Oligodendrocytes: oligodendroglioma Ventricles: ependymoma, choroid plexus papilloma, colloid cyst Vascular: hemangioblastoma Primitive cells: germinoma, medulloblastoma, neuroblastoma, pineoblastoma, retinoblastoma Neuronal: ganglioglioma, gangliocytoma Pituitary: adenoma, craniopharyngioma Nerves: schwannoma, neuroblastoma

Incidence of Intracranial Gliomas (All ages)

Glioblastomas
Astrocytomas

55.0%
20.5%

Ependymomas
Medulloblastomas

6.0%
6.0%

Oligodendrogliomas
Choroid plexus papillomas

5.0%
2.0%

Colloid cysts

2.0%

Incidence of Primary Intraspinal Intramedullary Gliomas

Ependymomas
Astrocytomas (grades 1 and 2)

63.0% 24.5% 7.5%

Glioblastomas (Astrocytomas grades 3 and 4)

Oligodendrogliomas Other tumors

3.0%
2.0%

Frequent brain tumors

Meningioma Astrocytoma/glioblastoma Oligodendroglioma Ependymoma Medulloblastoma Schwannoma/neurofibroma Phakomatosis

Meningioma

Arachnoid cells origin Attached to dura, subdural Common sites Changes in cranium Hyperostosis Invasion

Microscopic: whorls and psammoma bodies

Gliomas

Astrocytes- astrocytomas Fibrillary Pilocytic Oligodendrocytes- oligodendrogliomas Ependyma- ependymomas

Astrocytomas
Adults: Supratentorial Solid Malignant Infratentorial Cystic Benign

Childhood:

Adult vs childhood astrocytomas

Adult: fibrillary. Grading varies from low grade malignancy to one of most malignant brain tumor. Childhood: pilocytic. Very low grade tumor (benign).

Fibrillary astrocytomas

Grossly solid Common in cerebral hemispheres Low grade in young, higher grade in older Grading astrocytoma (low grade) Anaplastic astocytoma glioblastoma multiforme

Fibrillary astrocytoma: microscopic

Low grade- hypercellularity, pleomorphism Anaplastic- as above plus mitosis, vascular endothelial proliferation Glioblastoma multiforme- as above plus necrosis and pseudopalisades. Grossly variegated appearance (multiforme)

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

Rosenthal fiber definition

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

Oligodendroglioma

Slow growing tumor

Potentially malignant
Calcifications

Tumors in Ventricles

1. Ependyma: Ependymoma 2. Choroid Plexus: Papilloma

Ependymomas

Arise from ependymal lining- ventricles and central canal of spinal cord Common in childhood 4th V. common in cerebrum Most common tumor of spinal cord parenchyma in adult Microscopic perivascular pseudorosettes ependymal rosettes

Primitive neuroectodermal tumors

Neuroblastoma- cerebral hemispheres Medulloblastoma- cerebellum Ependymoblastoma- ventricles Pineoblastoma- pineal region

Medulloblastoma

Origin: primitive neuroectodermal cells Age: 1st decade of life Site: vermis of cerebellum May cause hydrocephalus Subarachnoid dissemination

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

Brain Tumors: Microscopic

Meningioma Whorls and psammoma bodies Glioblastoma Pseudopalisades Oligodendroglioma Mosaic/poached-egg Ependymoma Perivascular pseudorosettes Medulloblastoma Rosettes

Tumors of Nerve Roots and Peripheral Nerves

1. Schwannoma viii Cranial nerve (Acoustic sch.) Spinal roots, posterior Peripheral nerves 2. Neurofibroma Spinal Roots, rare Peripheral nerves 3. Malignant variants Rare

Peripheral nerve tumors

Schwannoma Schwann cells Compress the nerve trunk Encapsulated Easily resectable without nerve damage Microscopic: Antony A and B fibers Verocay bodies Neurofibroma Schwann cells, neurites, fibroblasts Fusiform and involves nerve trunk Not encapsulated Not resectable without sacrificing nerve Micro- Intermingled cells with wavy nuclei

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.

Phakomatosis: definition

Phakos (Greek): lentil mole or freckle. Neurologic abnormalities combined with defects of skin or retina, explained by their common ectodermal origin. Involvement of visceral organs

Phakomatosis (Neurocutaneous dysplasia)

1. Neurofibromatosis (von Recklinghausen's dis.) 2. Tuberous Sclerosis 3. Sturge-Weber disease (Encephalofacial Angiomatosis) 4. von Hippel-Lindau Disease 5. Neurocutaneous Melanosis

Neurofibromatosis
1. Dominant inheritance 2. Multiple neurofibromas Central - CNS peripheral nerves 3. Increased incidence of: meningioma glioma schwannoma - bilateral VIII N.

4. Cafe-au-lait (melanosis) in skin

5. Elephantiasis: increased connective tissue

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

Venice