A concise presentation about spinal cord tumors, including different treatment options

1. SPINAL CORD TUMOR
DR DODUL MONDAL
MD, DNB
All India Institute of Medical Sciences, New Delhi
Dodul Mondal

2. • INTRODUCTION
• ANATOMY
• EPIDEMILOGY
• NATURAL HISTORY
• CLASSIFICATION
• ROUTES OF SPREAD
• CLINICAL PESENTATION
• DIAGNOSTIC WORKUP
• PROGNOSTIC FACTOR
• DISEASE SPECIFIC MANAGEMENT
• CONCLUSION
Dodul Mondal

3. INTRODUCTION
• Rare tumor
• Constitutes 3-4% of all CNS and 6% of pediatric CNS tumors
• Oversimplified anatomical classification
• Young children more affected than adults
• Lack of RCT
• Mainly retrospective series
• Surgery and radiation mainstay of treatment
• Judicious use of radiation important
Dodul Mondal

4. ANATOMY
CSF
DURA
INTERVERTEBRAL DISC
SPINAL CORD
VERTEBRAL BODY
EPIDURAL SPACE
Dodul Mondal

5. Normal transverse spine Transverse spine with extradural mass
Intradural extramedullary mass Intramedullary massDodul Mondal

6. EPIDEMIOLOGY
Primary Spinal Cord Tumors – Uncommon
Primary Brain Tumor : Primary Spinal Cord Tumor = 20:1 (children) **
5:1 (adult)
Younger individuals affected more than adults
Constitutes 3-4 % of all primary CNS tumors & 6% of CNS tumors of children**
**CBTRUS-Statistical Report 2011
Dodul Mondal

7. EPIDEMIOLOGY cont…
 Primary tumor of SC are more frequent in children & more than half occur
below 10 years of age*
 36% intramedullary
27% extramedullary
24% extradural
75% of intramedullary tumor were astrocytomas*
**John K, Howard L. Weiner Pediatric Intramedullary Spinal Cord Tumors: Special Considerations . Journal of Neuro-
Oncology Volume 47, Number 3 / May, 2000
Dodul Mondal

8. NATURAL HISTORY
Mostly benign
IM tumors produce damage by local invasion & cystic compression
EM tumors compress, stretch or distort the cord & nerves
May involve focally or entire length of spinal cord
Dodul Mondal

9. CLASSIFICATION
SPINAL CORD TUMOR
Intra Dural
EXTRAMEDULLARY
Meningioma
Nerve sheath tumor
Vascular tumor
Ependymoma
INTRAMEDULLARY
Ependymoma
Astrocytoma
Oligodendroglioma
Extra Dural
Metastatic
Osteoid osteoma
Osteoblastoma
ABC
Plasmacytoma / Myeloma
Chordoma Chondrosarcoma
Osteosarcoma
Ewing’s sarcoma
Epidural hemangioma
Lipoma
Extradural meningioma
Nerve sheath tumor
Lymphoma
Dodul Mondal

10. ROUTES OF SPREAD
Low Grade-Generally localized
High Grade-Spread via CSF
Occasionally hematogenous route to Lungs
Lymph Node spread is generally not seen as CNS has no lymphatics
Dodul Mondal

11. CLINICAL PRESENTATION
Depends on the anatomical location & site of the lesion
Local
Focal Pain(75%):
• In EM tumors pain is more in the night due to venous congestion
Distal Neurological:
Paresis & Numbness
Autonomic Dysfunction
Sensory Deficits
Muscle Wasting
Loss of Reflexes
Failure to achieve milestones
Gait disturbance
Dodul Mondal

12. DIAGNOSTIC WORK UP
GENERAL
History
Physical examination
Complete neurological evaluation
IMAGING
Plain radiography
MRI whole spine with contrast
MRI brain
CT myelography
Intraoperative ultrasound
LABORATORY TESTS
CSF chemistry
CSF cytology
Dodul Mondal

13. RADIOGRAPHY/MYELOGRAPHY
• Overall 50% lesions of primary spinal tumors detected with plain X-ray
• Features are due to raised ICP
• Erosion of pedicles
• Increased AP diameter
• Scalloping of posterior vertebral body
• Scoliosis or kyphosis in children
• Calcification in meningioma
• Myelography in patients unsuitable for MRI
• CT myelography better
Dodul Mondal

14. COMPUTED TOMOGRAPHY
• Better for bony lesions and bony extent detection
• Extradural pathologic processes
• Paraspinal soft tissue masses
• Erosion of bone
• Calcification of meningioma
Dodul Mondal

15. MRI
• Gd-DTPA enhanced MRI imaging modality of choice
• CSF, white and gray matter, bone and bone marrow, fat, and flowing blood can
be distinguished
• MRI brain should also be done
• Maximum impact in the field of spinal tumors amongst all spinal pathologies
• Preoperative study of choice so as to narrow the differential diagnosis and
guide surgical resection.
• All spinal cord gliomas enhance
• LP should be done after MRI
Dodul Mondal

16. • Cord expansion-Essential imaging criterion for intramedullary spinal
neoplasm*
• MR sensitive to hemorrhage- can identify intratumoral bleeding
• Cysts are common associated finding
• Majority of intramedullary neoplasms show at least some enhancement
• Contrast study essential:
Enhancement useful in focal masses, especially hemangioblastoma and mets
Contrast helpful in differentiating suspected neoplasms from other etiologies. e.g.
infection, syrinx.
Actual lesion smaller than area of cord enlargement-Useful to pinpoint sites for
biopsy.
• *Epstein et al: IM tumors of spinal cord , Journal of Neurosurgery1993;79:204-209.
Dodul Mondal

17. PROGNOSTIC FACTORS
Major factors:
Tumor type and grade
Tumor extent and location
Patient age, presenting neurologic function
Treatment-related factors:
Tumor resectability
Use of radiation therapy for certain tumor types
Lower grade, younger age, and surgical resection were associated with
significantly better overall survival and cause-specific survival for both
astrocytomas and ependymomas*
• Milano MT, et al. Primary spinal cord glioma: a Surveillance, Epidemiology, and end results database study. J
Neurooncol2010;98(1):83–92
Dodul Mondal

18. PROGNOSTIC FACTORS…cont
 Patients with ependymoma survive longer without recurrence than patients with
astrocytoma
 Rostral tumors have a worse survival and neurologic outcome
 Cervical lesions had a higher surgical risk and complication rate
 Tumors affecting the rostral or cervical spinal cord were more likely to be
astrocytoma
 Tumors in the caudal spinal cord, filum terminale , or cauda equina were more likely to
be ependymomas
 High histopathologic tumor grade is associated with a high rate of disability and death
Extensive involvement of the spinal cord with an ependymoma is associated with a worse
outcome
Younger age is associated with a better than 5-year recurrence-free survival rate
Dodul Mondal

19. MANAGEMENT OF INTRAMEDULLARY TUMORS
• Most common: Astrocytoma, Ependymoma (95%)
• Complete surgical excision without compromising neurologic function
• Introduction of bipolar forceps
• Microsurgical techniques, use of CUSA (Cavitron Ultrasonic Surgical Aspirator )
• CUSA allows aspiration of tissue fragments from within 1 mm of the vibrating tip
• Intraoperative ultrasonography:
 Localize the lesion
 Define its extent
 Characterize the tumor as cystic or solid
 Facilitates placement of a myelotomy incision and initiation of tumor resection
 Assess the progress of tumor resection and adjacent cyst drainage with internal
spinal cord decompression
• Risk of paralysis after surgery less
Dodul Mondal

20. MANAGEMENT OF INTRAMEDULLARY TUMORS .. cont
• Resection of astrocytic tumors begins from within the tumor at the initial midline
myelotomy
• Removal until interface between tumor and normal spinal cord evident
• Complete excision achieved no postoperative therapy
• Delaying adjuvant radiation important in children
• Complete resection not achieved PORT
• PORT can be delayed even after STE till time of recurrence
• Recurrence assess for reresection and judicious use of PORT
Dodul Mondal

21. ASTROCYTOMA
 Low backache
 Painful scoliosis
 Thoraciccord (67%)*
 Cervical cord (49%)
 Entire spinal cord (holocord presentation)common in children (up to 60%)
quite rarein adults
 Isolated conus medullaris (3%)& rare infilum terminale
 Less than 10% of pediatric and 25% of spinal cord astrocytomas are
malignant .**
*Epstein et al Adult IM Astrocytoma of SC,J of Neurosurgery 77:355-359;2002
**Stein et al.IM tumors in adults.Neurosurgery Clin of N America 1998:1:609-630
Dodul Mondal

22. ASTROCYTOMA
Arise from the cord parenchyma not from the central canal, are
usuallyeccentric within the cord
Usually have poorly defined margins
Multisegment involvement of the cord is rule
Associated polar and intratumoral cysts.
Usually low grade fibrillary Astro is MC type followed by anaplastic
Astro
GBM is rare.
Dodul Mondal

23. ASTROCYTOMA cont..
 Iso- to hypo intense relative to the cord on T1W
 Hyper intense on T2W images
 Enhancement is seen in all cord astrocytomas, Inhomogenous enhancement
 Tumor, syrinx and cysts can be delineated on MR
 Rare SC GBM has propensity of leptomeningeal spread in 60% of patients*
 Completely resected tumor do not require PORT
 Incompletely removed tumor PORT should be considered
 Ciappetta et al, Spinal GBM: Report of 7 patients with spinal GBM and Review of
literature.Neurosurgery 28:302-306,1991.
Dodul Mondal

24. Treatment Recommendations for Pediatric Spinal Cord
Astrocytoma
Nadkarni TD,Childs Nerv Syst 1999;15:17-28Dodul Mondal

25. EPENDYMOMA
 Most common intramedullary spinal neoplasm in adults*
 Common in young adults
 Common in male patients
 Location:
2/3rd arise in lumbosacral region
40% arise from filum terminale
*Torres et al: Intramedullary ependymoma of spinal cord , J Neurosurgery 62;523-532;1992
Dodul Mondal

26.  Arise from ependymal cells of the central canal, and symmetric
cord expansion is the rule.
 Slow growth and tends to compress adjacent spinal cord tissue rather
than infiltrate it.
 Usually histologically benign and have long and indolent course.
EPENDYMOMA
Dodul Mondal

27. HISTOLOGICAL TYPES
 Cellular Ependymoma - mostly in cervical cord
 Myxopapillary Ependymoma - exclusively in conus medullaris and
cauda equina
 Primary Anaplastic Ependymoma of spinal cord are rare
 Other histological types:
 Subependymal
 Epithelial
 Mixed
Dodul Mondal

28. MR Imaging characteristics:
 Iso or hypointense relative to spinal cord on T1.
 Occasional hyperintense mass secondary to hemorrhage.
 Hyperintense relative to spinal cord on T2.
 Cap sign, a rim of extreme hypointensity (hemosiderin) seen at the poles of
the tumor on T2W (20%—30%)
…. thought to be secondary to hemorrhage.
 Cord edema around the masses (60%).
Dodul Mondal

29. Cysts In Ependymoma
Cysts are a common feature
Mostly non tumoral (polar) (70-80%)
NON TUMORAL CYSTS
Located at the poles of the solid
portion of the tumor .
Reactive dilatation of the central
canal (syringomyelia).
Not part of the tumor itself .
No enhancement.
TUMORAL CYSTS
Contained within the tumor
itself.
Peripheral enhancement
More in astrocytomas than
ependymomas.
Dodul Mondal

30. Filum Terminale Ependymoma
(Myxopapillary)
Dodul Mondal

31. EPENDYMOMA
T 1W-HYPO to ISO T 2W-HYPERINTENSE T 1W- CONTRAST
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32. EPENDYMOMA WITH BLEED
Plain T1W sagittal
 Iso to Hypo on T1
 Hyper on T2
 Bleeding-Hyper on T1
 Enhancement
 Homogeneous
 Cap sign seen
BLEED
Dodul Mondal

33. Nadkarni TD. Childs Nerv Syst 1999;15:17-28
Treatment Recommendations for Pediatric Spinal Cord
Ependymoma
Dodul Mondal

34. Difference Between Astrocytoma and Ependymoma
ASTROCYTOMA EPENDYMOMA
Cervical & Thoracic cord
LS spine, Cauda equina & conus
medullaris
Centrally located in cord Eccentric
Hemorrhage uncommon Hemorrhage common
Infiltrative with poorly defined
margins(multiple segment inv.)
Well defined due to pseudocapsule
Heterogenous enhancement Homogenous enhancement
Dodul Mondal

35. RADIOTHERAPY FOR INTRAMEDULLARY TUMORS
• Dose: 50.4 Gy. 1.8 Gy per fraction for low grade tumor
• 5 fractions per week
• Dose for high grade tumor is 54Gy in 1.8Gy/fx
• Limited data for dose response
• Dose below 50Gy is associated with high failure rate
• Where CSI is indicated, entire Craniospinal axis receives 36Gy to 45 Gy followed
by boost to gross tumor to a dose of 50.4-54 Gy
Dodul Mondal

36. RADIOTHERAPY TECHNIQUE
CONVENTINOAL TECHNIQUE
 Position: Supine/Prone
 Usually direct posterior field
 Superior and inferior border: Myelography defined gross tumor plus
2 vertebrae above and below
 Lateral border: To include tip of the lateral process of vertebral body
 Field placement should be judicious to avoid exit dose to vital OAR s
 Cervical vertebra can be treated with bilateral parallel opposed fields
 Lumbar region tumors can be treated with antero posterior field
arrangement
 Oblique wedge pairs can also be used for thoracolumbar region
For female patients lateral fields may be better for lumbosacral spine
 Dose distribution should be homogenous and toxicity minimal
Combination of low and high energy depending on site
Image courtesy: Unknown contributor Dodul Mondal

37. RA
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Image taken from Principles and Practice of Radiation Oncology, 6th Ed, Perez and Brady
Dodul Mondal

38. RADIOTHERAPY TECHNIQUES cont..
 For Low grade:
 CTV= GTV+ 0.5 to 1 cm margin
 CTV includes pre op tumor seen on MRI with intratumoral cysts
 Not necessary to include syrinx, unless radiographically or Sx evidence
of tumor
 For High grade:
 CTV= GTV +1.5 to 2 cm margin craniocaudally
 Should encompass inter vertebral foramina (IVF) if involved
 If cauda equina involved - CTV should include entire thecal sac to cover
meningeal sleeves in the IVF
 Myxopapillary ependymomas involving the conus, a 1.5-cm CTV margin
cephalad and caudal to the GTV is used
 Not beyond the thecal sac, which is typically at the level of S2-3
Dodul Mondal

39. Pioneering work by Merchant and Thompson et al from St.Judes on EBRT in
pediatrics Spinal cord tumors.
In a retrospective study of High grade tumors with a post op margin to GTV
less than 1.5 cm yielded increase local failure rate.(13% vs. 39%) P-signicant.*
*Merchant TE,Thompson SJ et al.High grade pediatrics spinal cord tumors. Pediatrics Neurosurgery
1999;39:1-5.
Dodul Mondal

40. STEREOTACTIC RADIOSURGERY
• Single fraction high dose radiation to the target and reduced dose
to normal tissue to reduce toxicity
• Most experiences in metastatic disease
• Intradural benign tumors
• 16-18 Gy in single fraction to 24-30Gy in 4-5 fractions
• Spinal Cord tolerance
• 13Gy/1fx, V10<10%(2-3mm above and below the target)
Dodul Mondal

41. Dodul Mondal

42. CRANIOSPINAL IRRADIATION IN SPINAL TUMOR
Multifocal dissemination
Leptomeningeal spread
CSF positive for malignant cells
Dose: 36-45 Gy to whole spine followed by boost
Radiation dose de-escalation not discussed
Dodul Mondal

43. Craniospinal Irradiation cont..
• one of the most complex radiotherapy techniques
• The CTV for CSI has an irregular shape that consists of the whole of the
brain and spinal cord and overlying meningeal coverings
Dodul Mondal

44. Patient Positioning and Immobilization
• Traditionally prone position
• Supine position also safe
• More comfortable
• If anesthesia is required, allows better control of the airway
• Immobilization is essential and involves the use of a head shell or
full-body immobilization
Dodul Mondal

45. Target Volume Definition
• Coverage of the entire target volume is critical
• CT simulation is invaluable for target volume definition - adequate coverage of the CTV
in the subfrontal region, cribriform plate, anterior and temporal lobes are more easily
ensured
• CT is also helpful in identifying the lateral aspect of CTV for the spine field that
includes the extensions of the meninges along the spinal nerve roots
• MRI is better to determine the lower limit of CTV for the spine field.
• Traditionally the lower border of the spine field was placed at the lower border of the
S2 (it is below S2 in 7% of children)
Dodul Mondal

46. Treatment Planning and Delivery
• Photons ( 6 to 10 MV range) - provide satisfactory PTV coverage
• Blocks are used in the lateral fields to shield facial structures and teeth
• The field must be wide enough to encompass the intervertebral foramina
• Block heart, lung, kidney, gonad
• Compensators may be needed to achieve dose homogeneity throughout the target
volume.
Dodul Mondal

47. The use of CT simulation with
contouring of the cord and overlying
meninges that extend laterally to the
lateral aspect of the spinal ganglia
results in a field width that is
narrower than one based on bony
anatomy.
The addition of shielding reduces
even further the volume of normal
tissues included in the treated
volume
Treatment planning by DobbsDodul Mondal

48. Field matching
• To cover the clinical target volume for CSI, lateral opposed fields upto the lower
border of C3-4, are used to treat the brain and a direct posterior field is used to cover
the spinal axis
• Precise matching of beam divergence is required – cranial fields with a collimator
rotation of 7–10° are used to match the divergence of the posterior spinal beam
• The field junction may be shifted or feathered by 1 to 2 cm once or twice to avoid any
excessive overlap of dose over the cervical spine – moving junction/ feathering
technique
Dodul Mondal

49. • The field junction, which is over the cervical cord (C3), usually is moved weekly to
avoid over- or under dosage
Treatment planning by DobbsDodul Mondal

50. Dose solutions in conventional planning
• Whole brain -
• Treatment is given isocentrically using a linear accelerator and opposing lateral
beams as defined in the simulator.
• The position of the lower cranial border is shifted by 1cm every wk to change the
level of the junction with the spinal field.
• Spinal field -
• Despite the use of an FSD extended up to 140 cm, two adjacent fields are
commonly required to cover the spinal cord in adults and older children.
• Both this and the craniospinal beam junction are moved caudally every wk
Dodul Mondal

51. Technique for calculating the gap at spinal beam junctions
• Half beam blocking using shielding or
independent collimator jaws can be
used to eliminate divergence up to the
match line, but accuracy is then
dependent on precise immobilisation
and reliability of skin marks
• It can also be done by using a gap
between beams so that the beam
edges converge at a planned depth
Dodul Mondal
Image from Principles and Practice of Radiation Oncology, 6th Ed, Perez and Brady

52. MENINGIOMA
• Usually benign
• Well-encapsulated
• Easily separated from the spinal cord
• Most can be completely excised
• Rare
• May arise anywhere within the intradural space
• Found in the thoracic region in approximately 80% of patients
• Uncommon in the lumbar region
• Rare in the sacrum
• At least 80% of meningiomas occur in women 40 years of age or older
Dodul Mondal

53. • Maximal surgical excision with preservation of neurologic function
• Posterior approach with a standard posterior laminectomy
• As much as 15% of spinal meningiomas
• Prognosis is excellent for most patients
• Rarely recur after total excision
• Subtotally resected meningiomas may recur late after surgery
• Some advocated postoperative radiation using either conventional fractionated
external-beam radiotherapy or stereotactic radiosurgery
Dodul Mondal

54. METASTASIS
• Common complication in advanced malignancy
• Not every metastasis is emergency
• Malignant epidural spinal cord compression (MESCC) is emergency
• Acute neurologic deterioration
• Prompt decision making necessary
• Pretreatment neurologic function most important determinant
• Pain is most common presentation (85-95%)
• Motor deficit 60-80%
• Sensory deficit 40-60%
• MRI gold standard investigation modality
Dodul Mondal

55. Treatment
Corticosteroids
In a randomized trial 96mg loading dexamethasone followed by 96mg/d x3d
followed by taper before RT vs. no steroid showed increased ambulatory rates at
3m , 6m*
Dose: Two different dose schedules
*Sorensen S et al. Eur J Cancer 1994:30A:22-7
100 mg loading dose followed by 96mg/d 10 mg loading followed by 16mg/d
Dodul Mondal

56. Surgical decompression
Life expectancy important determinant
Laminectomy
Reserved for posterior lesion
Anterior approach with immediate stabilization for anterior lesion
Combination of anterior and posterolateral approach
Surgery followed by RT provides higher overall ambulatory rate (85% vs 64%,
P<0.001)*
*Klimo et al. Neuro Oncol 2005:7:64-76
Dodul Mondal

57. RADIOTHERAPY
• Standard of care since 1950
• Preservation or improvement of neurologic function
• Palliation of pain
• Predictors of outcome:
• Extent of functional limitation at the beginning
• Tumor type
• Rapidity of neurologic deterioration
• Dose/fractionation:
• Different dose schedules used
• 30Gy/10fx/2wk
• 16Gy/2fx/2d
• 8Gy/1fx/1d
• PROTRACTED SCHEDULE REDUCES RISK OF NECESSITY OF REPEAT RADIATION
• SINGLE FRACTION MORE SUITABLE FOR PATIENTS WITH LIMITED LIFE
EXPECTANCY Dodul Mondal

58. OPTIMUM RT DOSE SCHEDULE??
Dodul Mondal

59. NEWER TECHNIQUES
Dodul Mondal

60. PROTON THERAPY
• Conventional photon/X-ray therapy produces tissue damage in the entry and exit
path
• More dose deposited in the entry track
• Radiation induced normal tissue damage
• Proton therapy gives high dose to the tumor tissue with reduced dose to entry
and exit path
• Positively charged hydrogen atoms
• High energy produced by acceleration in cyclotron/synchrotron
• Bragg peak effect
Dodul Mondal

61. Dodul Mondal

62. PROTON THERAPY cont..
• Lack of RCT of proton
vs. photon in spinal
cord tumor
• Dosimetric advantage
in reducing exit dose to
vital anterior
structures
• Particularly promising
in radiation of entire
craniospinal axis
• Exit dose to the thyroid,
heart, lung, gut, and
gonads can be reduced
with proton therapy
Dodul Mondal
Treatment planning by Dobbs

63. SRS (STEREOTACTIC RADIO SURGERY)
• Accurate delivery of high dose of radiation to the target
• Use of multiple highly targeted radiation
• Reduced dose to surrounding normal tissue
• Most spine SRS used in metastatic disease
• Limited data in intradural tumor
METASTATIC DISEASE:
 1-2 Session
8-18 Gy
Improved pain control
Better quality of life due to reduced toxicity
Less side effects
Dodul Mondal

64. SRS IN INTRADURAL TUMOR
• Only few reports on intradural tumor
• Largest series treated 73 benign tumor
• Used CyberknifeTM
• Dose 16-30 Gy
• 1-5 fx
• Median FU of 37
• Local control rate 98%
• Pain improved in:
• 70% of meningiomas
• 50% of schwannomas
• 0% of neurofibromas.
• Three patients developed radiotherapy-related spinal cord toxicity 5 to 13 months
after treatment
• Overall radiographic response rates for benign intradural tumors in the series
reported to date range from 28% to 39
Neurosurgery. 2008 Apr;62(4):887-95Dodul Mondal

65. SRS IN INTRADURAL TUMOR cont…
• 7 patients with intramedullary hemangioblastoma and ependymoma treated
at Stanford University showed good result*
• Dose used 16-30Gy in 1-5 fx or 18-25 Gy in 1-3 fx
• Early results promising
*Ryu SI, Kim DH, Chang SD. Stereotactic radiosurgery for hemangiomas and ependymomas of the spinal cord. Neurosurg
Focus 2003;15(5):E10
Dodul Mondal

66. Stereotactic radiosurgery for an L1 hemangioblastoma. A single dose of 20
Gy was delivered with CyberKnife using 87 noncoplanar beams
Perez, Sixth Ed
Dodul Mondal

67. CHEMOTHERAPY IN INTRAMEDULLARY LOW GRADE
ASTROCYTOMA AND EPENDYMOMA
To delay radiation in young children
Of no proven value till now
 Lack of large RCT
 Mostly extrapolated from intracranial tumor
 Platinum and Etoposide mostly considered
Temozolomide showed response in a retrospective series of 22 patients
with recurrent GrII gliomas*
*Chamberlain MC. Cancer 2008;113(5):1019–1024
Dodul Mondal

68. • 16-month polychemotherapy regimen
• Seven cycles of carboplatin 15 mg/kg or 450 mg m2 on day D1
• Procarbazine 4 mg/kg or 200 mg m2 on D 1–7
• Etoposide 5 mg/kg or 150 mg m2 on D2 and 23
• Cisplatin 1 mg/kg or 30 mg m2 on D22 and 23
• Vincristine 0.05 mg/kg or 1.5 mg m2 on D 43C
• Cyclophosphamide 50 mg/kg or 1500 mg m2 on D 43
• For children over 3, doses were calculated in mg/m2 /day
• Doireau V. Br J Cancer 1999;81(5):835–840
Dodul Mondal

69. Chemotherapy should be considered for children with a poor prognosis due to
relapse or metastasis of an intramedullary tumour
Chemotherapy could even be the first line of treatment for metastatic and
unresectable tumours (e.g. holocord tumours) and replace extensive irradiation
Dodul Mondal

70. Children’s Cancer Group 945
• N=13
• High-grade astrocytic spinal cord neoplasms
• Two cycles of “8-drugs-in-1-day” chemotherapy before radiation therapy
• Eight additional cycles thereafter
• 5 years PFS=46%
• 5 year OS=54%
• Conclusion: More intensive therapy necessary
Dodul Mondal

71. SUMMARY
• Rare tumors
• Children are more affected
• Intramedullary tumor mainly astrocytoma and ependymoma
• Contrast enhanced MRI investigation of choice
• Biopsy for histopathology confirmation
• Surgery mainstay of treatment
• Completely resected low grade intramedullary astrocytoma/ependymoma do not require
adjuvant treatment
• High grade tumors require adjuvant radiation
• Radiation to be delayed if possible
• Chemotherapy role still not defined
• Judicious treatment policy and technique of paramount importance
Dodul Mondal

72. THANK YOU
Dodul Mondal