PRACTICAL 19

NEOPLASMS OF
NERVOUS SYSTEM
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Neoplasms of nervous system include all intracranial tumours, tumours of spinal cord and of peripheral nerves.

CLASSIFICATION OF NEOPLASMS OF NERVOUS SYSTEM

(WHO HISTOLOGICAL CLASSIFICATION)
• Tumors of neuroepithelial tissue
• Astrocytic Tumors :
• Astrocytoma
• Anaplastic Astrocytomas
• Glioblastoma multiforme
• Pilocytic Astrocytoma
• Pleomorphic xanthoastrocytoma
• Subependymal giant cell astrocytoma.
– Oligodendroglial Tumors
– Ependymal Tumors :
• Ependymomas
• Anaplastic Ependymomas
• Myxopapillary Ependymomas
• Subependymomas.
– Mixed Gliomas
– Choroid Plexus Tumors
– Neuroepithelial tumors of uncertain origin.
– Neuronal and mixed neuronal-glial tumors
– Pineal parenchymal tumors
– Embryonal tumors
• Primitive neuroectodermal tumors (PNET)
• Medulloblastomas
• Others
• Tumors of cranial and spinal nerves
• Tumors of meninges
– Meningiomas
– Atypical meningioma
– Papillary meningioma
– Anaplastic meningioma
– Mesenchymal, nonmeningothelial tumors
• Malignant lymphomas
• Germ cell tumors
• Cysts and tumor like lesions
• Tumors of the stellar region
• Pituitary tumours
• Craniopharyngiomas
• Local extensions from regional tumors
• Metastatic tumors
• Unclassified tumors
A. NEOPLASMS OF PERIPHERAL NERVES
• Neurilemmoma (Schwannoma) and
neurofibroma are the most common tumours
of peripheral nerves derived from Schwann
cells.
• Despite their common cell of origin they show
distinct clinical presentation and histologic
features. Malignant counter part is known as
“Malignant Peripheral nerve Sheath Tumour”
(MPNST).
 Comparison of Neurilemmoma and Neurofibroma :
Neurilemmoma Neurofibroma
Peak Age 20 to 50 years 20 to 40 years,Younger
age in neurofibroma
Cutaneous nerves of head, Cutaneous nerves; deep
neck, flexor surfaces of nerves & viscera
Common extremities, less often
Location mediastinum and retro-
peritoneum

Encapsulated tumor Localized diffuse or

Histological composed of Antoni A plexiform tumor that is
Appearance and B areas, rarely usually not encapsulated
plexiform growth pattern
Degenerative
Common Occasional
Changes
Intense and relatively Variable staining of cells
S-100 uniform staining in a in a given lesion
Immunostaining given lesion
Occurrence in Uncommon Plexiform neurofibroma
NeurofibromatosisI or multiple
(VonRecklinghansen’s neurofibromascharacteristic of
disease) the disease
Malignant More common in
Extremely rare
Transformation Neurofibromatosis
1. SCHWANNOMA (NEURILEMOMA)
• It is truly encapsulated neoplasm of the human body and is almost
solitary.
• Common locations are in relation to peripheral, cranial and
sympathetic nerves. These include flexor surfaces of the extremities,
neck, mediastinum (posterior mainly) retroperitoneum, posterior
spinal roots, cerebellopontine angle.
• Macroscopy: Encapsulated, white to gray colour and firm texture
often containing cystic spaces.
• Microscopy : It shows regions of high and low cellularity called
antoni A & B areas respectively.
• - Antoni type A area is composed of proliferating Schwann cells
arranged in twisted, solid cords showing palisading of the nuclei,
( the resemblance to an exaggerated tactile corpuscles, Verocay
bodies are marked).
• - Antoni type B area is composed of loosely arranged Schwann cells
in which serous degeneration leads to formation of microcysts. The
vascularity may be a conspicuous feature and vessels may show
hyaline thickening.
2. NEUROFIBROMA
• -As a rule, the tumours are not encapsulated and have a softer
consistency than Schwannoma.
• -The more superficial tumours appear as small, soft,
pedunculated nodules protruding from the skin “molluscum
pendulum”.
• -Deeper tumours may become larger and result in diffuse
tortuous enlargement of peripheral nerves, hence designated as
“Plexiform Neurofibroma”.
• -Multiple neurofibromas represent the most important
component of the genetically determined disorder known as
Neurofibromatosis or Von Recklinghausen disease. (Autosomal
dominant disease with NF-1 gene, mutation on chromosome 17).
• Microscopy :It usually shows loose pattern of interlacing bands of
delicate spindle cells with elongated, slender and sometimes
wavy nuclei. In contrast to schwannoma, verocay bodies,
palisading of nuclei and hyaline thickening of the vessel wall are
almost always absent in neurofibroma.
 B. NEOPLASMS OF MENINGES :
• MENINGIOMA
• They are the commonest intracranial and intraspinal
tumours after gliomas.
• They arise from specialized arachnoid cap cells. They are
commonly found near superior longitudinal, transverse
and cavernous sinuses, in the falx cerebri and in the spinal
canal. Most of them are attached to dura but are usually
free from brain or spinal cord.They occur during middle
and later ages of the life and are more common in women
(M: F 2: 3).
• Macroscopy : They may take various forms like,well
circumscribed nodular growths, plaque like sessile growth
or diffuse meningiomatous growth. When it invades the
skull, it produces localized or widespread thickening of
bone (Hyperostosis).
• Microscopy : The structure is variable. The
basic cellular constituents resembling
arachnoid cells are,
(i) meningocytes, which are plump, elongated
or polyhedral cells with illdefined cell margins
(producing syncytial appearance) and round to
oval nuclei,
(ii) fibroblasts, elongated cells with scanty
cytoplasm and slender nuclei. These two types
of cells are arranged in whorl pattern.
• Depending upon the predominant type of the cells meningiomas are
further subclassified as :
‒ Meningotheliomatous (Syncytial), Fibroblastic, Psammomatous,
Microcystic, Secretory, Lymphoplasmocytic
• Metaplastic
• Rhabdoid
• Chordoid
• Transitional or
• Angiomatous.
Rhabdoid, atypical, clear cell, anaplastic, papillary, and hemangiopericytic
subtypes of meningioma are considered as aggressive or
malignantmeningiomas.
• Most meningiomas are slow growing benign and well localized tumours.
However others may invade the skull bones, the brain or dural venous
sinuses. Occurrence of secondary deposits in such tumours is extremely
rare.
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