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Best Cancer Hospital in India


neuro Oncology

Surgical Indications


  1. Tumor
  » Glioma
» Meningioma
» Schwannoma
» Neurofibroma
» Ependymoma
» Peipheral nerve sheath Tumor
  2. Trauma
  » Subdural Hematoma
» Extradural Hematoma
» Intracerebral Hematoma
» Depressed fracture -Spinal trauma and fracture
  3. Vascular
  » Aneurysm
» AV malformation
» Cavernoma
  4. Congenital
  » Meningocele
» Meningomyelocele
» Tethered cord
» Spinal Dysraphism
  5. Degenerative disorder Spine
  » Disc protrusion
» Spinal Canal
- Stenosis
- Spondylolisthesis
  6. Gamma Knife
  7. Stereotaxy
  » Biopsy
» Functional Neuro- Surgery
   


A.  Tumor

Glioma , Tumour originating from the glial cells are called glioma. Glial cells are the non neuronal cells of the brain and the spinal cord. They can be differentiated in many ways

1. By Origin -  Ependymoma — ependymal cells  Astrocytomas, Glioblastoma multiforme is the most common astrocytoma. Oligodendrogliomas —Oligodendrocytes, Mixed gliomas, such as Oligoastrocytomas - contain cells from different types of glia.



2. By Grade of the tumour  -

Low-grade gliomas are well-differentiated these are benign, WHO Grade I, and carries a better prognosis for the patient. Examples – Pilocytic Astrocytomas,  Myxopapillary Ependymoma
High-grade gliomas are undifferentiated or anaplastic; these are malignant tumour and carry a worse prognosis. Examples:   Anaplastic, Astrocytoma, Glioblastoma Multiforme


By location

  • Supratentorial: Above the tentorium
  • Infratentorial : Below the tentorium, in the cerebellum, mostly in children (70%)
  • Pontine: Located in the pons of the brainstem. The Pons controls critical functions such as breathing making surgery extremely dangerous.
High-grade gliomas are highly-vascular and have a tendency to infiltrate in the surrounding tissues. They have extensive areas of necrosis and hypoxia. Often tumor growth causes a breakdown of the blood brain barrier in the vicinity of the tumor. As a rule, high-grade gliomas almost always grow back even after complete surgical excision. On the other hand, low-grade gliomas grow slowly, often over many years, and can be followed without surgical treatment unless they grow and cause symptoms.

Symptoms

Symptoms of gliomas depend on which part of the central nervous system is affected. A brain glioma can cause headache, nausea, vomiting, seizures, and cranial nerve disorders  as a result of increased intracranial pressure.  Spinal cord gliomas can cause pain, weakness or numbness in the extremities. Gliomas do not metastasize by the bloodstream, but they can spread via the cerebrospinal fluid and cause "drop metastases" to the spinal cord.

Treatment Standard therapy

Treatment for brain glioma depends on the location, the cell type and the grade of malignancy. Often, treatment is a combined approach, using surgery, radiation therapy, and chemotherapy. Spinal cord tumors can be treated by surgery and radiation.


Prognosis  

Gliomas cannot be cured. The prognosis for patients with high-grade gliomas is generally poor, and is especially so for older patients. Survival of the  anaplastic astrocytoma is about three years. Glioblastoma multiforme has a worse prognosis with less than 12 month survival after diagnosis.



B.Meningioma 

Meningiomas are the second most common primary tumour  of the central nervous system, arising from the arachnoid "cap" cells of the arachnoid villi in the meninges . These tumors are usually benign in nature; however, they can be malignant
Meningioma can be familial (Neurofibromatosis) or can be sporadic. Other causes trauma, radiation

Signs and symptoms

 Small tumors  2.0 cm or less  are usually incidental findings and usually does not cause any signs and symptoms. Larger tumors can cause symptoms depending on the size and location.

  • Seizure may be caused by meningiomas that overlie the cerebrum
  • Weakness of the limbs may be caused by tumors growing in the motor cortex.
  • Raised intracranial pressure due to a tumour of large size but is less frequent than in gliomas.
Diagnosis

Meningiomas are readily visualized with contrast CT, MRI , and arteriography, all attributed to the fact that meningiomas are extra-axial and vascularized. CSF protein is usually elevated if lumbar puncture is attempted.


Treatment

Observation

For a small tumour   a regular close follow up with the patient with periodic imaging can be done. This is only possible when the tumor is small and patient does not have any neurological deficit. Observation is not recommended in tumors that are already causing symptoms. Furthermore, close follow-up with imaging is required with an observation strategy to rule out an enlarging tumor.


Surgical resection

Meningiomas can usually be surgically resected with permanent cure if the tumor is superficial on the dural surface and easily accessible. Transarterial embolization has become a standard preoperative procedure in the preoperative management. If tumor is not accessible or adherent to a vital structure total removal is nearly impossible. Malignant transformation is rare.

The probability of tumor recurrence or growth after surgical resection can be estimated by the tumor's WHO Grade and by the extent of surgery by the Simpson Criteria.

Radiation therapy

Radiation therapy may include Gamma Knife, proton beam treatment, or fractionated external beam therapy. Radiation therapy is often considered for WHO Grade I meningiomas after subtotal (incomplete) tumor resections. In the case of a malignant meningioma, the current standard of care involves post-operative radiation treatment regardless of the degree of surgical resection.]This is due to the proportionally higher rate of local recurrence for these higher grade tumors.

Conventional chemotherapy

Current chemotherapies are likely not effective. Antiprogestin agents have been used, but with variable results. Recent evidence that hydroxyura has the capacity to shrink unresectable or recurrent meningiomas is being further evaluated.


 C. Schwannoma
Schwannoma tumors are slow-growing  benign tumors  that grow from inside the nerve sheath. Unlike other neural tumors that arise from the nerve sheath, schwannomas arises from the schwann cells of the individual nerve fibers. Schwannomas   may show   random growth, or may grow as a part of some specific conditions such as Von Recklinghausen's disease, sometimes called neurofibromatosis (NF).Physical examination, laboratory investigations and imaging help to diagnose Neurofibromatosis. Treatment for schwannoma tumors varies depending on the size, number and location and clinical features of the patient.

Diagnosis

Schwannomas are predominantly benign tumors. In rare cases, the tumors can become malignant. The tumors can grow anywhere throughout the nervous system. The most common places for schwannoma tumors are in the brain, throughout the trunk, and the limbs  

Surgery
Surgical intervention is the most common treatment for schwannomas.The aim of the surgery is total removal of the tumour   with preservation of neural function to reduce the post operative complications. It requires sophisticated gadgets like operating microscope and intraoperative electrophysiological monitoring. Neurosurgeon-oncologists deal with malignant neural tumors, so patients with malignant schwannomas benefit greatly from multidisciplinary approach

Gamma Knife Surgery

It is a highly focused stereotactic Gamma Radiation to a specific area of the brain. The success rate is high and complications are very few. It can be used where surgery is difficult due to a difficult location of a tumor or potential risk of complications are very high .It can be used when  there are contraindications for surgery . Also Gamma Knife can be used when there is a significant residual tumor left after a surgery.

Close follow up
If the patient does not have any or minimal signs or symptoms due to this tumor, a close observation is the best choice. It comprises of periodic clinical check up and imaging

D. Neurofibroma A neurofibroma is a benign nerve sheath tumor in the peripheral nervous system. Usually found in individuals with neurofibromatosis type 1 (NF1), a genetically-inherited disease, they can result in a range of symptoms from physical disfiguration and pain to cognitive disability. Neurofibromas arise from Schwann cells in the NF1 gene that codes for the protein neurofibromin. This protein is responsible for regulating the RAS-mediated cell growth pathway. In contrast to schwannomas, another type of tumor arising from Schwann cells, neurofibromas incorporate many additional types of cells and structural elements in addition to Schwann cells, making it difficult to identify and understand all the mechanisms through which they originate and develop.

Neurofibromas have been subdivided into two broad categories: dermal and plexiform. Dermal neurofibromas are associated with a single peripheral nerve, while plexiform neurofibromas are associated with multiple nerve bundles. According to the World Health Organization classification system, dermal and plexiform neurofibromas are grade I tumors.

Types of Neurofibroma:-

Dermal Neurofibromas

Dermal Neurofibroma, sometimes referred to as cutaneous neurofibroma, typically arise in the teenage years and are often associated with the onset of puberty. They continue to increase in number and size throughout adulthood, although there are limits to how big they get. They look like lumps on or under the skin. While dermal neurofibromas can lead to stinging, itching, pain and disfiguration. They do not undergo malignant transformation.

 Plexiform Neurofibromas Plexiform neurofibromas are often congenital defects, and are the more troublesome type. They can be very large and can cause pain, disfigurement, neurological and other clinical deficits. While dermal neurofibromas originate in nerves in the skin, plexiform neurofibromas can grow from nerves in the skin or from more internal nerve bundles. Internal plexiform neurofibromas are very difficult to remove completely because they extend through multiple layers of tissue and the attempt would damage healthy tissue or organs. Plexiform neurofibromas also have the potential to cause severe clinical complications if they occur in certain areas. In addition, about 10% of plexiform neurofibromas undergo malignant transformation. This transformation turns the plexiform neurofibroma into a malignant peripheral nerve sheath tumor (MPNST).

Treatment
Surgery is the most preferred choice of treatment . Dermal neurofibromas are not removed unless they are painful or causing disfigurement.

E. Ependymoma

Ependymoma is a tumor that arises from the ependyma, a tissue of the central nervous system. Usually, in children the location is intracranial, while in adults it is spinal. The common location of intracranial ependymoma is the fourth ventricle. Ependymomas are also seen with Neurofibromatosis Type II. Ependymomas make up about 5% of adult intracranial gliomas and up to 10% of childhood tumors of the central nervous system (CNS). Their occurrence seems to peak at age 5 years and then again at age 35.

About 85% of ependymomas are benign myxopapillary ependymoma . It is a localized and slow growing, low-grade tumor. Although some ependymomas are of a more anaplastic and malignant type, most of them are not anaplastic.

Treatment:  Surgical removal. Incomplete removal or malignant tumor requires radiotherapy.

Gamma Knife Surgery  - A   surgery  without  incision

 
Gamma Knife Surgery represents one of the most revolutionary means available to treat the brain diseases.It is painless, bloodless and precise therapy with a very short duration of therapy and  hospitalisation. In this technique there is no need to open the brain. Although it is termed as surgery but in true sense it is not the conventional  surgery.Gamma knife is a highly precise stereotactic focussed  Gamma Radiation directed at the diseased  part of the brain . It can be applied in cases where conventional surgery is difficult. It is a  non invasive technique.

History
In 1950 Swedish  Neurosurgeon Professor  Lars Leksell at Karolinska  Institute in Stockholm  and radiation biologist Borge Larsson of the Gustaf  Wermer Institute,Uppsala University began to investigate combining radiation beams with stereotactic (guiding ) devices capable of pinpointing targets within the brain.In 1967 the first Gamma Knife device was constructed and  Professor Leksell termed as `Stereotactic Radiosurgery `Professor Leksell and his colleague built his second Gamma Knife machine in 1975.It was for the Neurological service.The subsequent devices are installed in different countries.

The Gamma Knife contains 201 Cobalt -60 sources approximately placed in circular array in a heavily shielded assembly.The unit directs the Gamma radiation to a target point ie. abnormal brain tissue .Patient wears a specialized helmet that is fixed  to their skull so that brain tumour remains stationary at target point of Gamma rays.The powerful radiation treats the diseased brain tissue while the surrounding brain tissue is intact. It is as precise as 0.3 mm.So a relatively inoperable area can be treated safely in Gamma Knife.A single dose radiation therapy is capable of treating the brain disease.



The procedure
A patient who comes for Gamma Knife therapy must be assesed first  by the  treating neurosurgeon . On the day of surgery under local anaesthesia and if necessary sedation a frame is fixed in the head. An iamging is done thereafter.Now while the patient can relax now the Gamma Knife team works on 3 dimensional computurised technology to plan the treatment.Once the planning is complete patient is palced in the Gamma Knife unit for the radiation therapy.Gamma Knife is a metal helmet like device that emits radiation.After the radiation the frame is removed. Patient is discharged in the next day.

Indications
» Gamma Knife therapy can be directed for both benign and malignant tumours
» Benign tumours like Meningioma,Acoustic Scahwannoma,Pituitary adenoma,Craniopharyngioma  and Pineal region tumour
» Malignant tumours like  Metastases,Chordoma, Medulloblastoma,  Medulloblastoma,Astrocytoma.Ependymoma
» Arterio vascular malformation (AVM )
»  Painful condition like Trigeminal Neuralgia
» Movement disorder like Parkinson`s disease
» Gamma Knife therapy is restricted to a mean spherical diameter of the tumour 35 mm.Larger volume of tumour may be treated     but it  may lead to ineffective total dose from radiological stand point.This may cause suboptimal outcome.


Results

Gamma Knife stops tumour growth in 90-95 % of cases. It also causes shrinkage of the tumour growth in the majority.It takes from a week to  a year to exhibit  its effect depending on how rapidly the tumour cells are dividing.

Mechanism
Radiation is invisible energy that penetrates in to the tumour cells and vessels.Radiation therapy uses high energy light beams  ( X- rays or Gamma rays ) or charged particles ( electron or proton beams ) to damage  DNA  of the tumour cells or lesions.The aim is to damage the chromosome of a cell so that it dies spontaneously or it dies  during proliferation.

GK works in AVM by damaging abnormal tangle of blood vessels and forming scar tissue.The lumen of these scarred blood vessels clogged up  and sealed off.So hemorrhage cannot occur.

Side effects

» It is relatively safe procedure
» Some immediate side effects like nausea, headache, vomiting