Due to technological advancements and better understanding of brain tumors, there had been a significant improvement in the clinical outcome of brain tumors. The majority of the brain tumors are now diagnosed at a very early stage. CT scan or MRI of the brain is a very common diagnostic tool and help to diagnose any lesion inside the brain.
All the brain tumors need not be operated. Non invasive single dose radiation therapy like Gamma knife or Cyberknife is a noninvasive way of treating small lesions of the brain.
Stereotactic biopsy helps in minimal invasive way of diagnosing brain tumor.
Stereotactic Radiotherapy ( SRT) is used to treat malignant lesions of the brain. Even multiple metastasis of systemic cancers of the body to the brain can be treated with surgery and SRT.
Meningioma which constitute about 15% of all the primary brain tumors can be excised completely without any recurrence.
So, if somebody is diagnosed as a case of brain tumor, he or she should not lose hope. Unlike other tumors of the body, the brain tumors are not labeled as cancer and non cancer. Rather, there are differerent grades of the tumor ( WHO Grade 1 to IV). Grade 1 tumors are usually benign and do not require chemotherapy or radiotherapy.
Neuro-oncology constitutes the major part of clinical practice of neurosurgeons. Almost every neurosurgeon operates and manages cases of CNS tumours. Many advances have occurred in last few decades and now many options are available for investigating and treating such lesions. Translational research has provided hope for the better clinical outcome in future. MRI with contrast , MR spectroscopy, PET scan are the new investigative modalities to detect neoplasis and metastasis . Immunotherapy, vaccine therapy, molecular based targeted therapy, Bevacizumab, Temozolomide, Brachytherapy, stereotactic radiotherapy, stereotactic biopsy of the lesion, gamma knife are the new treatment options for treating CNS tumors.
Classification of CNS tumors by WHO (World Health Organization) in 2007 identifies 7 broad categories of CNS tumors:
1. tumors of neuroepithelial tissue
2.tumors of cranial and paraspinal nerves
3. tumors of the meninges
4. lymphomas & hematopoietic neoplasms
5.germ cell tumors
6. tumors of the sellar region
7. metastatic tumors
This framework is necessary to have an idea about all CNS tumors.
It is very important to use authentic terms for the cns tumors and have an overall view about all the tumors of the central nervous system. Various nomenclatures and classifications are used for description. Therefore, it is imperative to know what is most authentic and easiest way to remember.
The pathological classification of cns tumors is the most common and most practical way of nomenclature and classification of such tumors.
CNS tumors, especially gliomas, contrary to the other cancers of the body , cannot be classified like Benign or Malignant or Cancer and Non cancer or like Benign lesion of the Breast ( Fibroadenoma) and Cancer of the breast ( Ca Breast) , or Benign Prostrate Hyperplasia (BPH) and Cancer of the Prostrate.
On the contrary, the Gliomas or Astrocytomas are classified on the basis of features of neoplasia- like cellular atypia, nuclear proliferation, vascular proliferation & necrosis. So, there is a grading of neoplasia.
Cushing and Bailey classification was an earlier effort to classify brain tumors.
World Health Organization ( WHO) classification of tumors of central nervous system ( 2007) is a comprehensive, and authentic source of reference.
1. Tumours of neuroepithelial tissue
1.1. Astrocytic tumours
Pilocytic astrocytoma & Subependymal giant cell astrocytoma (WHO grade I)
Pilomyxoid astrocytoma & Pleomorphic xanthoastrocytoma (WHO grade II)
Diffuse astrocytoma (WHO grade II)
Variants: protoplasmic, gemistocytic, fibrillary, mixed
Anaplastic astrocytoma (WHO grade III)
Glioblastoma (WHO grade IV)
a. Giant cell glioblastoma
b. Gliosarcoma
Gliomatosis cerebri (WHO grade III)
1.2. Oligodendroglial tumours
Oligodendroglioma (WHO grade II)
Anaplastic oligodendroglioma (WHO grade III)
1.3. Oligoastrocytic tumours
Oligoastrocytoma (WHO grade II)
Anaplastic oligoastrocytoma (WHO grade III)
1.4. Ependymal tumours
Subependymoma & Myxopapillary ependymoma (WHO grade I)
Ependymoma (WHO grade II)
Anaplastic ependymoma (WHO grade III)
1.5. Choroid plexus tumours
Choroid plexus papilloma (WHO grade I)
Atypical choroid plexus papilloma ( WHO grade II)
Choroid plexus carcinoma (WHO grade III)
1.6. Other neuroepithelial tumours
Astroblastoma (WHO grade I)
Chordoid glioma of the third ventricle (WHO grade II)
Angiocentric glioma (WHO grade I)
1.7. Neuronal and mixed neuronal-glial tumours
Dysplastic gangliocytoma of cerebellum (Lhermitte-Duclos),
Desmoplastic infantile astrocytoma/ganglioglioma,
Dysembryoplastic neuroepithelial tumour,
Gangliocytoma ,
Ganglioglioma
Papillary glioneuronal tumour
Rosette-forming glioneuronal tumour of the fourth ventricle, &
Paraganglioma are WHO grade I tumors.
Central neurocytoma & Extraventricular neurocytoma , Cerebellar liponeurocytoma (WHO grade II)
Anaplastic ganglioglioma (WHO grade III)
1.8. Tumours of the pineal region
Pineocytoma (WHO grade I)
Pineal parenchymal tumour of intermediate differentiation (WHO grade II, III)
Pineoblastoma (WHO grade IV)
Papillary tumors of the pineal region (WHO grade II, III)
1.9. Embryonal tumours
Medulloblastoma (WHO grade IV)
Medulloblastoma with extensive nodularity (WHO grade IV)
Anaplastic medulloblastoma (WHO grade IV)
CNS Primitive neuroectodermal tumour (WHO grade IV)
CNS Neuroblastoma (WHO grade IV)
Atypical teratoid/rhabdoid tumour (WHO grade IV)
2. Tumours of cranial and paraspinal nerves
Schwannoma (WHO grade I)
Neurofibroma (WHO grade I)
Perineurioma (WHO grade I, II, III)
Malignant peripheral nerve sheath tumour (MPNST) (WHO grade II, III, IV)
3. Tumours of the meninges
3.1 Tumours of meningothelial cells
Meningioma
Variants ( Subtypes ): meningothelial, fibrous (fibroblastic), transitional (mixed), psammomatous, angiomatous, microcystic, secretory, clear cell, chordoid, lymphoplasmacyte-rich, and metaplastic
Atypical meningioma (WHO grade II)
Anaplastic meningioma (WHO grade III)
3.2 Mesenchymal tumours
Lipoma , Angiolipoma ,Liposarcoma, Leiomyoma, Leiomyosarcoma, Solitary fibrous tumour , Fibrosarcoma , Rhabdomyoma, Rhabdomyosarcoma,Chondroma , Chondrosarcoma, Osteoma,Osteosarcoma, Osteochondroma, Angiosarcoma, Kaposi Sarcoma, Ewing Sarcoma - PNET
Malignant fibrous histiocytoma
Hibernoma
Haemangioma
Epithelioid hemangioendothelioma
Haemangiopericytoma & Anaplastic haemangiopericytoma (WHO grade III)
3.3 Primary melanocytic lesions
Diffuse melanocytosis
Melanocytoma
Malignant melanoma
Meningeal melanomatosis
3.4 Other neoplasms related to the meninges
Haemangioblastoma (WHO grade I)
4. Tumors of the haematopoietic system
Malignant Lymphomas
Plasmocytoma
Granulocytic sarcoma
5. Germ cell tumours
Germinoma
Embryonal carcinoma
Yolk sac tumour
Choriocarcinoma
Teratoma
Mixed germ cell tumours
6. Tumours of the sellar region
Craniopharyngioma (WHO grade I)
Granular cell tumour (WHO grade I)
Pituicytoma (WHO grade I)
Spindle cell oncocytoma of the adenohypophysis (WHO grade I)
7. Metastatic Tumours
I have made an effort to create an outline and curtail the details so that any doctor can have an overview of all the tumors of CNS. The details of all such tumors should be read in conjunction with the radiological findings which is more interesting and easy.
The most recent classification of CNS is proposed by WHO in 2016.
WHO (2016) Classification
of Tumors of Central Nervous System
In year 2016, for the first time, the World Health Organization
( WHO) classification of CNS tumors uses molecular parameters in addition to
histology to define many tumor entities. Major restructuring has been done in
diffuse gliomas, medulloblastomas and other embryonal tumors. It has added
newly recognized neoplasms, and has deleted some entities, variants and
patterns that are no longer have diagnostic and/or biological relevance.
Summary
of changes in new classification
Major
restructuring of diffuse gliomas, Medulloblastomas, other embryonal tumors
& incorporation of genetically defined entities
The
term “primitive neuroectodermal tumor” is removed
Incorporation
of a genetically defined ependymoma variant - RELA fusion positive
Addition of
Newly
recognized entities, variants and patterns have been added:
1. IDH-wildtype and IDH-mutant
glioblastoma
2. Diffuse midline glioma, H3 K27M–mutant
3. Embryonal tumour with multilayered
rosettes, C19MC-altered
4. Ependymoma, RELA fusion–positive
5. Diffuse leptomeningeal
glioneuronal tumor
6. Anaplastic PXA
7. Epithelioid glioblastoma
8. Glioblastoma with primitive neuronal
component
9. Multinodular and vacuolated pattern of ganglion cell tumor
Deletion of
Gliomatosis
cerebri
Protoplasmic and fibrillary
astrocytoma variants
cellular ependymoma variant
Primitive Neuroectodermal tumor
Addition
of Brain invasion as a criterion for atypical meningioma
Restructuring
of solitary fibrous tumor and hemangiopericytoma ( SFT/HPC) as one entity
Expansion
& clarification of entities included in of Nerve sheath tumors, with
addition of hybrid nerve sheath tumors & separation of melanotic schwannoma
from other schwanomas
Expansion
of entities included in hematopoietic/lymphoid tumors of the CNS ( lymphomas
& histiocytic tumors)
NEW
CLASSIFICATION
Diffuse astrocyic and oligodendroglial tumors
Diffuse astrocytoma, IDH-mutant
Gemistocytic
astrocytoma, IDH-mutant
Diffuse astrocytoma, IDH-wildtype
Diffuse astrocytoma, NOS
Anaplastic astrocytoma, IDH-mutant
Anaplasticastrocytoma, IDH-wildtype
Anaplastic astrocytoma, NOS
Glioblastoma, IDH-mutant
Glioblastoma, NOS
Diffuse midline glioma, H3 K27M-mutant
Oligodendroglioma, IDH-mutant and
1p/19q-codeleted
Oligodendroglioma, NOS
Anaplastic Oligodendroglioma, IDH-mutant and
1p/19q-codeleted
Anaplastic oligodendroglioma, NOS
Pilocytic astrocytoma
Pilomyxoid astrocytoma
Subependymal giant cell astrocytoma
Pleomorphic xanthoastrocytoma
Anaplastic pleomorphic xanthastrocytoma
Ependymal tumors
Subependymoma
Myxopapillary ependymoma
Ependymoma
Papillary ependymoma
Clear cell ependymoma
Tanycytic ependymoma
Dysembryoplastic neuroepithelial tumor
Gangliocytoma
Ganglioglioma
Anaplastic ganglioglioma
Dysplastic cerebellar gangliocytoma (
Lhermitte-Duclos disease)
Desmoplastic infantile astrocytoma and
ganglioglioma
Papillary glioneuronal tumor
Rosette forming glioneuronal tumor
Diffuse leptomeningeal glioneuronal tumor
Central neurocytoma
Extraventricular neurocytoma
Cerebellar liponeurocytoma
Paraganglioma
Tumor of the Pineal region
Pineocytoma
Pineal parenchymal tumor of intermediate
differentiation
Pineoblastoma
Papillary tumor of pineal region
Embryonal tumors
Medulloblastoma, genetically defined
Medulloblastoma, WNT
activated
Medulloblastoma,
SHH-activated and T53-mutant
Medulloblastoma,
SHH-activated and T53-wildtype
Medulloblastoma, non-WNT/non SHH
Medulloblastoma, group 3
Medulloblastoma, group 4
Medulloblastoma, histologically defined
Medulloblastoma, classic
Medulloblastoma,
desmoplastic/nodular
Medulloblastoma with
extensive nodularity
Medulloblastoma, large
cell/anaplastic
Medulloblastoma NOS
Embryonal tumor with multilayered rosettes,
C19MC-altered
Embryonal tumor with multilayered rosettes,
NOS
Medulloepithelioma
CNS Neuroblastoma
CNS ganglioneuroblastoma
CNS embryonal tumor, NOS
Atypical teratoid/ rhabdoid tumor
CNS embryonal tumor with rhabdoid features
Tumors of cranial and paraspinal nerves
Schwannoma
Cellular Schwannoma
Plexiform Schwannoma
Melanotic Schwannoma
Neurfibroma
Atypical neurofibroma
Plexiform neurofibroma
Perineurioma
Hybrid nerve sheath
tumors
Malignat peripheral
nerve sheath tumor
Epitheloid MPNST
MPNST with perineurial
differentiation
Meningioma
Meningioma
Meningothelial meningioma
Fibrous meningioma
Transitional meningioma
Psammomatous meningioma
Angiomatous meningioma
Microcystic meningioma
Secretory meningioma
Lymphoplasmacyte-rich meningioma
Metaplastic meningioma
Chordoid meningioma
Clear cell meningioma
Atypical meningioma
Papillary meningioma
Rhabdoid meningioma
Anaplastic ( malignant) meningioma
Mesenchymal, Non-meningothelial tumors
Solitary fibrous tumor/hemangiopericytoma
Hemangioblastoma
Hemangioma
Epitheloid hemangioendothelioma
Angiosarcoma,
Kaposi Sarcoma,
Ewing sarcoma/ PNET
Lipoma
Angiolipoma
Hibernoma
Liposarcoma
Desmoid type fibromatosis
Myofibriblastoma
Meningeal melaonocytosis
Meningeal melanocytoma
Meningeal melanoma
Meningeal melanomatosis
Lymphoma
Diffuse large B-cell lymphoma of the
CNS
Immunodeficiency-associated CNS lymphoma
AIDS-related diffuse large B-cell lymphoma
EBV-positive diffuse large B-cell lymphoma, NOS
Lymphomatoid granulomatosis
Intravascular large B-cell lymphoma
Low-grade B-cell lymphoma of the CNS
T-cell & NK/T cell-lymphoma of the
CNS
Anaplastic large cell lymphoma ,
ALK-positive
Anaplastic large cell lymphoma ,
ALK-negative
MALT lymphoma of the dura
Histiocytic tumors
Germinoma
Embryonal carcinoma
Yolk sac tumor
Choriocarcinoma
Teratoma
Mature, Immature
Teratoma with malignant transformation
Mixed germ cell tumor
Tumors of the sellar region
Craniopharyngioma
Admantinomatous
craniopharyngioma
Papillary
craniopharyngioma
Granular cell tumor of sellar region
Pituicytoma
Spindle cell oncocytoma
Metastatic tumors
Neurosurgery is the cornerstone of the management of tumors of the brain and spinal cord. Because glioma annd metastasis constitute majority of tumors, very judicious approach is required for the diagnosis and treatment of such lesion. Many options are available but they may be costly, unnecessary and harmful. A very small low grade glioma be an incidental finding and surgery may not be required. A small neoplastic lesion of less than 3 cm may be treated with gamma knife which is a non surgical treatment and utilizes precise and focussed single dose radiation over a deep seated lesion in the eloquent area of the brain.
About 4 decades ago, whole brain radiotherapy and steroids were the main treatment modality for treating brain metastasis. But now few brain metastatic lesions may be excised safely and stereotactic radiotherapy can be given postoperatively, reducing the morbidity in a patient.
Advances in the skull base approaches have made the neurosurgery relatively safer, and few tumors can be excised completely. This may be true in cases of intracranial meningiomas.