Management overview of Meningioma,

Pituitary gland and Spinal cord tumours

By – Dr Satyajeet Rath

Guided by – Prof Kamal Sahni

Dt – 20/04/2017

Meningioma

Meningioma

Meningiomas: MC primary intracranial tumours.

majority are benign, with about 1%-3% classified as malignant

98% are intracranial but may arise anywhere in central nervous system

Classified into grades : WHO grade I,II and III : On the basis of local invasiveness

and cellular features of atypia.

Histological verification is not mandatory in all cases

Originates from arachnoid cap cells

Incidence increases with age, More common in females

40% and 80% of meningiomas contain an abnormal chromosome 22 (22q12.2)

NF type 2 :Multiple meningiomas

Express ER (40%)/PR(70%) +ve , may grow faster in pregnancy

Supratentorial – 85-90%

Infratnetorial – 5-10%

Extradural <1%

MRI

• MRI is preferred , as it is superior in demonstrating dural origin, as well as

vascularity, edema, sinus and bone invasion.

• Present as solitary round tumours, with close contact to the duramater and strong

enhancement with contrast.

• Thickening of the duramater at the perimeter of the tumour (so-called dural tail) is

displayed by T1 with gadolinium.

• Isointense or hypointense to gray

matter on T1.

• Isointense or hyperintense on T2

weighted images.

• Areas of necrosis and calcification

do not enhance

Meningioma of the

right convexity

with typical

radiological signs

(A) Bone window of

the cerebral CT scan

in coronal view

showing hyperostosis

facing the tumour

(arrow). (B) Cerebral

MRI (fluid-attenuated

inversion recovery)

showing the tumour

slightly hyperintense.

The oedema of the

parenchyma adjacent

to the tumour is shown

by the arrow. (C) T2

sequences in coronal

view showing

cerebrospinal fluid

(arrows) interposed

between tumour and

parenchyma, showing

the extra-axial nature

of the tumour. (D) T1-

weighted MRI after

gadolinium injection

depicting intense

enhancement of the

tumour. The arrow

shows the large

contact of the tumour

with the dura mater

and the thickening of

the adjacent dura

mater

Treatment Options

• MC intracranial tumours BUT the level of evidence to provide recommendations for the diagnosis and t/t is low compared with other tumours such as HGG

• Surgery

– Objective: total removal of the meningioma, dural attachment and bone involved with the tumor

– Priority: preserve and improve neurological function

• Radiotherapy

– Indications: • Residual tumor left at operation

• Recurrence

• Tumors could not be treated surgically

• Malignant histology

The available treatment modalities are-

Observation Surgery Radiotherapy Chemotherapy

Management outline

EANO 2016 Recommendations

Simpson Grade of Resection

Observation

• Asymptomatic patients with little or no edema in the adjacent brain

areas

• Patients with mild or minimal symptoms

• Patients in whom treatment carries a significant risk

Meningioma Grade 1 – Role of Sx

• Asymptomatic: Observation (6 monthly to annually: Clinical examination

and MRI )

• Imaging strongly S/O Meningioma => Histological verification is not

mandatory.

• However, exclusion of rare differential diagnoses such as metastasis is

recommended

• Radiologically growth / clinical symptoms: Surgery is the First Choice

• Extent of resection should be confirmed by a postoperative MRI (within 48 h

after surgery / after 3 months to avoid artefacts)

Meningioma Grade 1 – Role of Radiotherapy

• Indications:

Elderly patients (older than 65 years)

Not safely accessible by surgery.

Incomplete surgical resection

SRS-for small tumours Evidence**: 35 retrospective studies showed a 5-year progression-free survival of 86−100% after primary stereotactic radiosurgery.

Fractionated EBRT: 50−55 Gy given in doses of 1·8−2·0 Gy per fraction can be applied

(Evidence level III, Recommendation level B).

• **Rogers L, Barani I, Chamberlain M, et al. Meningiomas: knowledge base, treatment outcomes, and uncertainties.

• A RANO; review. J Neurosurg 2015; 122: 4–23.

• Radiotherapy with subtotal resection: is associated with disease control and

survival rates similar to those reported for gross total resection

• IMRT/ fSRT: to spare critical neurovascular structures surrounding the tumour and

to reduce the risk of long-term cognitive deterioration => similar disease control to

conventional radiotherapy.

Surgery is the first choice of treatment.

Should aim to achieve Simpson Grade I resection

• Role of adjuvant radiotherapy after partial resection??

Adjuvant radiotherapy (54–60 Gy given in 1·8−2·0 Gy per fraction) should

be considered (evidence level III, recommendation level C)

Fractionated RT is preferred over SRS techniques

although SRS offers similar results for small tumours/ tumour residual

Meningioma Grade 2 – Role of Sx

• Role of adjuvant radiotherapy after gross complete resection?????

– ROAM/EORTC 1308 trial (ISRCTN71502099) : whether radiotherapy reduces the risk of or delays tumour recurrence.

Newly diagnosed Atypical Meningioma (WHO grade II)

Gross total resection (Simpson grade I–III)

early adjuvant radiotherapy (60 Gy in 30 fractions) vs. observation

• Role of adjuvant radiotherapy after partial resection??

Adjuvant radiotherapy (54–60 Gy given in 1·8−2·0 Gy per fraction) should be considered (evidence level III, recommendation level C)

Fractionated RT is preferred over SRS techniquesalthough SRS offers similar results for small tumours/ tumour residual

Meningioma Grade 2 – Role of Radiotherapy

Meningioma Grade 2 – Role of Chemotherapy

and targeted therapy

Retrospective studies and small prospective studies: (WHO grade II and III meningiomas)

o Hydroxycarbamide

o Cyclophosphamide/Doxorubicin/Vincristine chemotherapy,

o interferon-alfa,

o Megestrol acetate, Medroxyprogesterone acetate, Octreotide, Pasireotide (long-acting

release),

o Imatinib, Erlotinib, Gefitinib, Vatalanib, Sunitinib,

o Bevacizumab.

Most promising results for Bevacizumab, Vatalanib, and Sunitinib

EORTC phase 2 trial (NCT02234050): efficacy of TRABECTEDIN shown promising activity in

recurrent WHO grade II and grade III meningiomas.

Meningioma Grade 3

• Surgical resection:

– should be as radical as possible

• Adjuvant Radiotherapy: Fractionated

– Dose: at least 54 Gy 1·8−2·0 Gy fractions

– Dose:

– Complete resection: 54 Gy

– Incomplete resection: 6o Gy

Ongoing trials

RTOG 0539 trial (NCT00895622):

• WHO Grade II: RT with 54 Gy in 30 fractions after gross total resection

• High-risk meningioma (ie, WHO Grade II recurrent disease, WHO Grade II after

subtotal resection, and all WHO grade III) are receiving up to 60 Gy.

EORTC 22042-26042 trial (NCT00626730):

WHO Grade II and III after Gross Total resection: 60 Gy in 30 fractions

After subtotal resection: 60 Gy in 30 fractions followed by Boost 10 Gy in 5 fractions to

residual

- Results awaited

Spinal Meningioma

• Surgical resection: 1st Choice

• To remove the tumour and decompress the spinal cord is the therapy of choice

• If surgical resection not possible: SRS or hypofractionated RT (recommendation level: good practice point)

• Adjuvant therapy is done according to WHO grade and resection.

Follow-up

WHO grade I:

First 5 years: Annually

After 5 years: every 2 years.

WHO grade II :

First 5 years: Every 6 months

After annually after 5 years

WHO grade III :

Every 3–6 months indefinitely

Radiotherapy

• Conventional radiation therapy

• Stereotactic radiosurgery

Gamma Knife

Cyber Knife

• Fractionated stereotactic radiosurgery

• Intensity-modulated radiation therapy (IMRT)

SRSKondziolka et al. (1999):

– 99 patients from U. Pittsburgh

– 43% SRS alone, 57% surgery + SRS

– median tumor margin dose 16 Gy, max dose 32 Gy, median tumor volume 4.7 cc.

• LC 93-95% ,PFS 90- 93% in both arms at 5–10-year.

Stafford et al. (2001): – 190 patients from Mayo Clinic,

– 59% had prior surgery, 12% with atypical or malignant histology.

• Median tumor margin dose was 16 Gy. Median prescription isodosevolume was 8.2 cm3.

• Five-year LC for patients with benign, atypical, and malignant tumors were 93, 68, and 0%, respectively.

• Five-year CSS for patients with benign, atypical, and malignant tumors were 100, 76, and 0%, respectively.

• 628 patients (130 men and 498 women) - gamma knife radiosurgery for intracranial meningioma,

• with single lesion meningioma, followed up after 6 months with imaging, and then at 24 months with a clinical examination.

• The median patient's age was 56.8 years. Maximal dosage was 27.8 Gy and marginal dosage was 13.9 Gy.

Results

• The overall tumor control rate was 95%.

• Twenty-eight patients (4.4%) showed evidence of tumor recurrence.

• Ninety-eight patients (15%) developed peritumoral edema (PTE) after gamma-knife surgery; two of them (2%) underwent surgical resections due to PTE.

• Nine patients had craniotomy and tumor removal after gamma knife surgery.

Conclusion

• Gamma knife surgery for intracranial meningioma has proven to be a safe and effective treatment tool with successful long-term outcomes.

Pituitary Tumours

Anatomy• It is called the Master of endocrine

orchestra

• 15 mm in ant-post and 12 mm in supero inferior axis

• It weighs about 0.5 gm.

• The pituitary gland occupies a cavity of the sphenoid bone called sella turcica

• Roof is formed by diaphragm sellae

• Floor – hypophyseal fossa

• On each side :The cavernous sinus with its content

• The stalk of pituitary is attached above to the floor of third ventricle

• Anterior and intermediate lobe

– Rathke’s pouch

• The posterior lobe or

neurohypophysis

– Downward pocketing of third ventricle.

• Posterior lobe

– Oxytocin and Vasopressin which are

synthesised in the supraoptic and

paraventricular nuclei in

hypothalamus

• Anterior lobe

– ACTH,TSH,GH,FSH,LH,Prolactin

Epidemiology

• Pituitary neoplasm account for 10% to 15% of diagnosed primary intracranial neoplasm

• 10% of healthy population has pituitary abnormality detected by MRI

• Approximately 70% are endocrinologically active

• 70% of adenomas present between the ages 30 -50 yrs

• Women have high incidence of pituitary adenomas(15-44 yrs)

• Incidence of macroadenomas is similar between males and females

• Annual incidence ranges from 0.5 to 0.7/100,000

• A genetic predisposition to develop adenomas has been described in– MEN I syndrome

– Carney complex

– Isolated familial somatotropinomas(IFS)

Natural History

• Usually has a long natural history with an insidious onset of symptoms

• Symptoms are usually present for years prior to diagnosis

• When small pituitary tumour tends to be smooth round tumours

• Macroadenomas are known for their local invasive properties

• Malignant behaviour with distant metastases is rare

Hyperpituitarism• HYPERPROLACTINEMIA Most common cause of pituitary hormone hypersecretion

Amennorhoea

Galactorrhoea

Infertility

• INCREASED GH

Acromegaly in adults

Frontal bossing

Increased hand foot size

Mandibular enlargement,Prognathism

Large fleshy nose

Proximal muscle wasting,carpal tunnel syndrome,macroglossia

Gigantism in children

• INCREASED ACTH Causes cushing syndrome

Central obesity

Plethoric moon facies

Purple striae,increased bruisability

Glucose intolerence

Acne,hirsuitism

Proximal muscle weakness

Hypertension

Amennorhoea,infertility

Hypopituitarism

• Growth hormone deficiency: Short stature(Dwarfism)

• Gonadotrophins deficiency: Infertility, decreased sexual functions ,loss

of secondary sexual characters, menstrual

irregularities

• TSH deficiency : Hypothyroidism

• ACTH deficiency :Hypocortisolism

• Prolactin deficiency :Lactation failure

• Vasopressin deficiency : Diabetes insipidus

FEATURES OF SELLAR MASS LESION

• PITUITARY Hypopituitarism

• OPTIC CHIASMA Bitemporal Hemianopia

Superior temporal defect

• CAVERNOUS SINUS Ophthalmoplegia

Ptosis

Diplopia

• OTHERS Head ache

Hydrocephalus

Dementia

Diagnostic Work-up

Detailed History and complete physical examination

Confirmation of diagnosis

• Radiological Examination

MRI-preferred modality better visualisation of soft tissue and

vascular structure than CT Scan

• Biopsy –In a case of non secreting lesion

HORMONAL ANALYSIS

• Serum Prolactin level

• Growth hormone:basal growth hormone level

IGF-I

Glucose suppression,insulin tolerence

• ACTH Hypersecretion:

Serum ACTH,Dexamethasone supression test

24 hrs urine for 17-hydroxy corticosteroids

and free cortisol

• Gonadal function: FSH, LH, Esradiol,Testosterone

• Thyroid function test

• Adrenal function: basal plasma,urinary steroids

cortisol response to insulin induced hypoglycaemia

Classification of Pituitary tumours

• ANATOMICAL SIZE

Microadenoma(<10 mm)

Macroadenoma(>10 mm)

• PHYSIOLOGICAL

Ant pituitary

1. Prolactin

2. Growth hormone

3. Adrenocorticotrophic hormone

4. Leutinizing hormone

5. Follicle stimulating hormone

6. Thyroid stimulating hormone

Post pituitary

1. Oxytocin

2. Vasopressin

Classification(Cont…)

• ACCORDING TO CLINICAL SYMPTOMS Functional

Non functionaL

• ACCORDING TO EXTENT OF EXPANSION OR EROSION OF SELLA

Grade 0: Intrapituitary microadenoma with normal sellar appearance

Grade I: Normal-sized sella with asymmetric floor

Grade II: Enlarged sella with an intact floor

Grade III: Localized erosion of sellar floor

Grade IV: Diffuse destruction of floor

• ACCORDING TO SUPRASELLAR EXTENSION

Type A: Tumor bulges into the chiasmatic cistern

Type B: Tumor reaches the floor of the 3rd ventricle

Type C: Tumor is more voluminous with extension into the 3rd ventricle up to the foramen of Monro

Type D: Tumor extends into temporal or frontal fossa

Pathological classification

Ant Pituitary has 5 specific cell types

• Somatotrophs:– produces growth hormone,acidophilic

• Lactotrophs: – produces prolactin,acidophilic

• Corticotrophs:– produces ACTH,MSH,basophilic

• Thyrotrophs:– produces TSH,basophilic

• Gonadotrophs:– FSH,LH,basophilic

• Post pituitary: pituicytes and non myelinated fibres

Management

• Observation

• Surgery

• Radiotherapy

• Medical Management

Observation

• In asymptomatic non secreting microadenomas

• Small asymptomatic prolactinomas

2-4 mm no testing required

5-9 mm MRI can be done once yearly

• Indications for intervention

Tumour growth on imaging

symptoms of hypersecretion

development of visual field defects

Surgery

INDICATIONS

• It is the first line treatment for

most symptomatic pituitary

tumours

• Useful when medical therapy

fails

• When prompt relief from mass

effect and hormone secretion is

required

• Pituitary apoplexy

Transfrontal

Trans-sphenoidal

Types MICROSCOPIC TRANSSEPTAL TRANSSPHENOIDAL

• Current standard surgical procedure

• Safe procedure with mortality rate 0.5%

• Contraindications are sphenoid sinusitis,

ectatic midline carotid arteries

lateral surpasellar extent

ENDOSCOPIC TRANSNASAL TRANSSPHENOIDAL

• Allows better visualisation of pituitary gland, hyophyseal stalk,

cavernous sinuses,optic nerve and suprasallar areas

TRANSCRANIAL

• Requires craniotomy and retraction of frontal lobes

• Used for large invasive tumours with significant suprasellarextension

• When transsphenoidal approach is contraindicated

COMPLICATIONS OF SURGERY

• CSF rhinorrhoea

• Meningitis

• Haemorrhage

• Stroke

• Damage to pituitary

• Visual loss

Management algorithm for a nonfunctioning Pituitary Microadenoma

Management algorithm for a nonfunctioning Pituitary Macroadenoma

Management algorithm for a Prolactinoma

Management algorithm for a GH-secreting adenoma

Management algorithm for a ACTH-secreting adenoma

Radiotherapy

• INDICATIONS

1. Hypersecretion and mass effect due to large tumours

2. Incomplete resection of tumour

3. Progressive disease after surgery

4. Recurrent tumours

Radiotherapy

• TECHNIQUES

Conventional External Beam Radiotherapy Manual planning

2D Planning

3D CRT

Fractionated Stereotactic Radiation Therapy

Gamma Knife Radiosurgery

MANUAL AND 2D PLANNING

Positioning

Supine with neck flexed and head at

45 degrees

Pituitary board can be used to achieve

this

Immobilisation done with

thermoplastic mask

VOLUME

The entire pituitary gland with

extensions and a margin of 1-1.5

cm

PORTALS

Two parallel and opposite lateral fields and one anterior or vertex beam that

enters above the eyes

The centre of the pituitary is located at a point 2-2.5 anteriorly to tragus and

2-2.5 cm superiorly to that point

Taking this point as centre a field of( 4*4)cm-(6*6) cm is marked

ENERGY

4-10 Mev or Co 60

DOSE

Nonfunctioning tumours 45-50.4 Gy@1.8 Gy/#

Functional tumours 50.4-54 Gy

3D PLANNING

• Image based treatment planning using a 3D technique is the standard of care

• Defining the tumour volume

MRI,CT as well as clinical and surgical findings should be used to define the tumourvolume

CT simulation assists in defining treatment volume

GTV is the pituitary adenomas including any extention into adjacent anatomic regions

CTV :GTV+5 mm in a clear defined tumour or entire sella and cavernous sinus with invasive tumours

PTV:CTV+5mm

FRACTIONATED STEREOTACTIC RADIOTHERAPY(FSRT)

FSRT is characterised by improved patient localisation,tighter volume

definition more conformal isodose distributions

It has better safety profile and efficacy

• IMMOBILISATION

Aim is to achieve a patient positioning error of less than 3mm by

various means like

Invasive halo ring

Radiocamera bite block

Non invasive Head frames

Stereotactic(cont…)

• TARGET VOLUME DELINEATION

• GTV is designed with help of MRI and extent of cavernous sinus invasion should be included

• No additional margins is required for CTV

• PTV:CTV +2-3 mm margin

• TREATMENT PLANNING

• Depends on the delivery systems available

• Options include Multiple spherical shotsDynamic conformal archesNonisocentric robotic delivery

• DOSE 50.4 Gy in 28#@1.8Gy/#

STEREOTACTIC

RADIOSURGERY

Accepted treatment for smaller,radiologically well defined tumours located at a distance (3-5 mm) from optic apparatus

Contraindicated if optic chiasma is closer than (3 -5)mmto the tumour

Delivery systems include linear accelerator and gamma knife

Head is fixed with an appropriate stereotactic head frame and a high resolution imaging study is obtained

Gamma knife uses smallest collimators and maximum number of isocentres .

The dose to optic chiasma is limited to <8-9 Gy

DOSE

• Non functioning (12-20Gy)

• Functioning (15-30 Gy)

RT Dosing Guidelines

EBRT

(1.8Gy/fx)

Radio-surgery

(optic chiasm

dose < 9 Gy)

Local

Tumor

Control

Biochemical

Control

Nonfunctioning

tumors

45-50.4

Gy

12-24 Gy to

margin

95% NA

Functioning

tumors

45-54 Gy 25-30 Gy to

margin

90-95% 33-95%

Results

MODALITY SURGERY

VS

SURG+POSTOP RT

SURG+POST OP

RT

VS

RT ALONE

GAMMA KNIEF

RADIOSURGERY

RESULTS Park et.al

10 yrs recurrence rate

2.3%with rt,50.5%only

surgery

Grigsby et al

Proggression free

survival at 5 yrs 96%

and 20 yrs 88%

Maschiro.et al

Tumour control at 5 yrs is

93.6% and

endocrinological

improvement is 80.3%

CONCLUSION Post op RT should be

preferred

Surg+rt had a greater

control of local disease

Results are similar to

#EBRT but gamma knief

seems to be safer in terms

of complications

• In acromegaly, the rate of reduction of GH after conventional therapy is a 50% drop in 27 (± 5) months (Biermasz et al., 2000).

• It has also been expressed as halving of mean GH level in a population of acromegalic patients in about 2 years (Ciccarelli et al., 1989).

• The rate of reduction of IGF-I is slower with normalization in 60% of patients 5–10 years after treatment (Biermasz et al., 2000).

• In Cushing's disease, the normalization of plasma and urinary cortisol has been reported in 50–100% of patients. A detailed prospective study of 30 adults demonstrated remission in all patients 60 months after radiotherapy, with the majority normalizing in the first 2 years after treatment (Estrada et al., 1997).

COMPLICATIONS OF RADIOTHERAPY

• ACUTE REACTIONS

Fatigue, Focal alopecia, Otitis

• CHRONIC REACTIONS

1. Hypopituitarism primarily due to hypothalamic injury. In patients with normal pituitary function around the time of radiotherapy, hormone

replacement therapy is required in 20–40% of patients at 10 years

2. Damage to optic apparatus

1·5% risk of radiation optic neuropathy (Brada et al., 1993; Tsang et al., 1994)

3. Secondary brain tumours

The reported frequency is in the region of 2% at 10–20 years (Brada et al., 1992; Tsang et al., 1993; Erfurth et al., 2001).

4. Brain necrosis

0·2% risk of necrosis of normal brain structures (Becker et al., 2002).

5. Although radiation is blamed for potential cognitive impairment, there is no clear evidence that small-volume fractionated irradiation affects cognitive function beyond the deleterious effect of surgery and the pituitary adenoma (Grattan-Smith et al., 1992; Peace et al., 1997).

Spinal Cord

Tumours

Introduction

• Rare heterogeneous group of tumors.

• 15% of all primary cns neoplasms arise in the sc.

• Incidence higher in males than females

• Age – 10 to 40 yrs

• Extramedullary tumors comprises around 2/3 & intramedullary ~ 10%.

• Cervical spine involvement is seen in 15-20%, thoracic in 50-55%, lumbar in 25-30% cases.

BIOLOGIC CHARACTERISTICS & MOLECULLAR BIOLOGY

1. Diversity of spinal axis tumors due to large spectrum of

phenotypically distinct cells in the axis.

2. Most are benign.

3. Significant morbidity due to direct compression of important neural

structures.

4. Histology is an important prognostic factor

– Ependymoma better prognosis than > astrocytoma

– Low grade & pilocytic astrocytoma better prognosis.

Radiotherapy

• Controversial since SC tumors are indolent (SC toxicity)

• Not indicated in – Completely excised intra medullary

– Ependymoma and astrocytoma low grade

• Incomplete / piece meal excision :- adjuvant radation

• Follow up after surgery and second surgery/adjuvant RT if recurrence particularly in children

• RT induced spinal deformity due to damage of epiphyseal plate, soft tissue fibrosis and contracture.

• In high grade astrocytomas & ependymomas adjuvant RT is recomendedirrespecive of extent of resection ( Mayo data –IJROBP:27-- 1986,2000)

Radiation TechniqueConventional technique

– Cervical:-parallel opposed portal

– Thoracic:-direct posterior or posterior wedge

– Lumbar & cauda equina:-opposed AP/PA fields

– In female :-lateral techniques are used to avoid the dose to ovary

• Depth of spinal cord:-determined from CT/MRI

Beam energy:-

– Cervical:-4 to 6 mv

– Thoracic & lumbar:-combination of low energy (4-6 mv) & high energy(8 to 25 mv)

• Dose:-

– Low grade astrocytoma and ependymoma gtr/50.4 Gy after str

– High grade astrocytoma:-54 Gy

– High grade ependymoma csi = 36Gy + boost 50.4 Gy – 54 Gy

– Meningiomas : 50.4 GY or 16 GY/fx 80% IDL by SBRT

• Margins:-

– 3-5cms suproinferior, thecal sac at S 2-3 needs to be covered in caudal

ependymomas

– 0.5-1 cm margin for low grade astrocytoma

• Imrt

• SRT & Proton beam therapy

Radiation toxicity

• Reversible myelopathy (2-6 mths) – Lhermitte’s sign is seen

characterized by shock like sensation in hands and feet when

neck is flexed.

– Lasts for weeks .

– Requires no treatment.

• Progressive myelopathy (13-29 mths) - characterized

parasthesia,progressive motor weakness pain/temp

lossbowel/bladder dysfunction

• Spinal cord tolerance– Quantec guidelines show for

conventional RT a dose of 50Gy, 60Gy& 69Gy is associated

with 0.2%, 6% & 50% rates of myelopathy.

Chemotherapy

• Limited role

• Used after all modalities are exhausted or in <3 yr age group to borrow time for RT

• Platinums & etoposides are most active agents for ependymoma

• Temozolamide may be used in spinal glioma. Used as concurrent with RT and then maintainance therapy

• In <3year age groups– intensive treatment with carboplatin, procarbazine, vincristine, cyclophosphamide, etoposide, cisplatin agents

Astrocytoma

• Age– paediatric and adolescent

• Site – mostly cervical & thoracic

• Mostly focal but whole cord involvement may occur.

• Majority are low grade (who gr I & II )

• Accompanying syrinx in 40 % cases.

• Complete surgical resection is often impossible.

• Juvenile pilocytic astrocytoma (who gr I) due to its non infiltrative nature can be

treated with radical resection

• Fibrillary(gr II), Anaplastic (gr III), GBM(gr IV)– due to local infiltration resection

enblock not possible onlybiopsy/subtotal resection done

• Adjuvant radiotherapy indicated

Nerve sheath tumours

• Arise from schwann cells

• Types – schwannoma, neurofibroma,ganglioneuroma

• Majority are intradural arising from dorsal sensory nerve root

• When it has both intra and extra dural component it is called dumbbell lesion.

Schwannoma –

– Most frequently seen in cervical and lumbar region

– Present with radicular sensory change with weakness being a less common sign

– Mostly solitary but when associated with nf2 & schwannomatosis these are multiple

– Pathological types – conventional (mc),cellular,melanotic ,plexiform(do not undergo malignant change)

– Grow eccentricaly without nerve infiltration

• Treatment – radical surgical resection(hemilaminectomy)

• Recurrence is rare and they usually donot undergo malignant transformation.

Neurofibroma

• Commonly seen in NF-1

• Cervical spine most commonly affected

• Often multiple, benign

• Encases the nerve root hence excision without sacrificing the nerve is difficult

Ganglioneuroma

• Mostly benign and paraspinal

• Arises from sympathetic nervous system

• Pathological types– a. Ganglioneuroma (Extradural, Dumbell)

– b. Gangliocytoma

– c. Ganglioganglioma (Intradural, Intramedullary)

Hemangioblastoma

• Highly vascular tumors

• May be associated with Von- Whippel - Lindau

• Usually dorsal

• Sometimes multiple

• Renal cell carcinoma must be searched for

• Treatment – surgical resection

Thank You