Cord Tumors Tumors M.ZERAH Université PARIS V. Hopital Necker Enfants Malades
Jan 16, 2016
Paediatric Spinal Cord Tumors
Tumors
M.ZERAHUniversité PARIS V.
Hopital Necker Enfants Malades
Paediatric Spine Tumours40% Intramedullary
20% intradural / extramedullary
40% extradural
1 : 1 million of children / y 15 / y in France
Bicêtre-Necker 1984 - 2004 : 148 children (<16y)
Literature 1924 - 2005 : 1923 children
New-York (Fred Epstein) : 164 “children” (<21 y)
Intramedullary TumoursPersonal Series &
Literature
Spinal Cord Tumors
<5 y 5-10 y 10-15 y 15-30 y 30-45 y 45-70 y > 70 y0%
5%
10%
15%
20%
25%
30%
35%
Age at Surgery
26%
Pain : 72%
Spinal deformity : 68%
Motor Signs : 79%
Gait : 63%
Upper Limbs : 16%
Bowel/Bladder dysfunction 18%
Hydrocephalus : 8%
Miscellaneous : 12%
Initial Symptoms
Solid part of the tumor
2 to 20 levels (average : 7)2 to 20 levels (average : 7)
Cystic Part
Present in 86% of the cases
Rostral and caudal in 62% of the cases
Average of 11 levels
20% of brain stem extension
Diagnosis
One exam : MRI
One goal : Cystic / Solid
No histological diagnosis on MR ...
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AstrocytomaEpendymoma
Neurinoma
Mélanoma
Germinoma
PathologyFrench Series (1998)
- Pilocytic55%
- Grade II 23%- Grade III
17%- Grade IV
5%
Surgery
Surgical Procedure
Laminotomy
Ultrasonography
Evoked potential ?
Gross total removal of the solid part of the tumor (except in malignant astrocytomas)
No cyst removal
Laminoplasty
Brace (3 to 6months)
LaminotomyQuickTime™ et un
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“open book”
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Unilateral
Laminotomy
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Monobloc
Gross total removal1984 - 2004
Astrocytoma (90%)
I & II (90%)* 87%
III & IV (10%) 15%
Ependymoma (10%) 100%
* 2/3 pilocytic
Spinal DeformityGroup I : Laminectomy
1984 - 1988 : 20 Children
post operative appearence or worsening of a spinal deformity
Group II : Laminotomy
1988 -1993 : 21 Children
post operative appearance or worsening of a spinal deformity
16
4
p < 0,0001
M.ZERAH 1995
Grade Definition
INeurologically normal; mild focal deficit not significantly affecting function of involved limb; mild spasticity or reflex abnormality; normal gait
IIPresence of sensitivomotor deficit affecting function of involved limb; mild to moderate gait difficulty; severe painor dysesthetic syndrome impairing patient quality of life; still functions and ambulates independently
Spinal Cord Tumors
Clinical / Functionnal classification scheme
Clinical / Functionnal classification scheme
Grade Definition
IIIMore severe neurological deficit; requires cane/brace forambulation or significant bilateral upper extremity impairement;may or may not function independantly
IV Severe deficit; requires wheelchair or cane/brace with bilateralupper-extremity impairement; usually not dependent
from Mc Cormick J NeuroSurg (1990) 72 : 523-532
Spinal Cord Tumors
Post-Operative Status
Spinal Cord Tumors
Preoperative Postoperative
I (15%) II (34%) III (40%) IV (15%) Total
I (9%) 9% 0% 0% 0%
II (26%) 4% 17% 5% 0% 5%
III (18%) 0% 13% 5% 0%
IV (47%) 0% 4% 30% 13%
Total 51% 44%
Preoperative Postoperative
I (15%) II (34%) III (40%) IV (15%) Total
I (9%) 9% 0% 0% 0%
II (26%) 4% 17% 5% 0% 5%
III (18%) 0% 13% 5% 0%
IV (47%) 0% 4% 30% 13%
Total 51% 44%
Late Follow-up
Spinal Cord Tumors
Preoperative Postoperative
I (13%) II (34%) III (40%) IV (15%) Total
I (23%) 11% 8% 4% 0%
II (30%) 2% 22% 6% 0% 16%
III (29%) 0% 4% 25% 0%
IV (20%) 0% 0% 5% 15%
Total 16% ( 5% Mortality) 68%
Preoperative Postoperative
I (13%) II (34%) III (40%) IV (15%) Total
I (23%) 11% 8% 4% 0%
II (30%) 2% 22% 6% 0% 16%
III (29%) 0% 4% 25% 0%
IV (20%) 0% 0% 5% 15%
Total 16% ( 5% Mortality) 68%
92% ambulatory23% clinically normal85% normal schooling
Surgical removal must be the goal in the pediatric spinal cord
tumours
In most of the cases the prognosis is good
Remaining problems
(Astrocytomas)Is there a difference between partial and total removal ?
Followup and treatment in case of partial removal
Treatment of recurrences ?
treatment of “non surgical” tumours (panmedullary , multipolar, metastatic Tumours)
Grade IV
Is there any difference between Total and Subtotal Removal ?
Przybylski, CNS 1997 N = 18. 1976 - 1992
“No patients relapse after total resection, whereas more than two-thirds treated with other than complete resection relapsed within 3 years (p = 0.029)……prolonged relapse-free survival was observed in all patients treated with complete resection, including 3 with anaplastic tumors”
Is there any difference between Total and Subtotal Removal ?
…Sandler Neurosurg. 1992 (n = 21)Lunardi Acta Neurochir 1993 (n = 25)Hulshof Radioth & Oncology 1993 (n = 50)Huddart J Neurosurg 1993 (n = 27)Cristante Neurosurg. 1994 (n = 69) O’Sullivan J. Neurosurg 1994 (n = 31)Minehan J. Neurosurg 1995 (n = 79)Innocenzi CNS 1996 (n = 45)Jyothirmayi J Neuro Oncology 1997 (n = 23)Goh Pediatr Neurosurg 1997 (n = 44)...
No difference
N = 73. 13 Departments. 1971-1994
Is there a difference between Total removal and Subtotal removal +
Radiotherapy ?“ we would recommend postoperative radiation therapy in all the intraspinal gliomas where total resection was not possible”
Hulshof, 1996
“ Conservative surgery followed by radiotherapy appears to have a role in achieving tumor control and neurologic recovery in patients with low grade astrocytomaa of the spinal cord”
Jyothirmayi, 1997
Is there a difference between Total removal and Subtotal removal +
Radiotherapy ?
YesYes NoNo
Lunardi, 1993Cristante, 1994Innocenzi,1996Zileli, 1996Przybylski, 1997Goh, 1997Jallo, 2001
Huddart, 1993O’Sullivan, 1994Minehan, 1995Shirato, 1995
Sandler, 1992
What is the real efficacy
of Radiotherapy ?
O’Sullivan, 1994 1959 - 1990 n = 31 (Toronto)
“These statistic suggest that irradiation treatment without resection may achieve long-term control in children with astrocytoma or ependymoma of the spinal cord”
10-year survival rate = 80%20-year survival rate = 53%
Biopsy + RxTherapy
Is there any advantage of adjuvant radiotherapy in case of total removal ?
YesYes NoNo
Rossitch, 1990Sandler, 1992Lunardi, 1993 Huddart, 1993Cristante, 1994Epstein, 1994
O’Sullivan, 1994Minehan, 1995Shirato, 1995 Innocenzi,1996Zileli, 1996Przybylski, 1997Goh, 1997Jallo, 2001
What are the true complications of Surgery, Radiotherapy and Chemotherapy ?
Radical Surgery : Clinical deterioration 10 - 40%
Radiotherapy : Second cancer 10% (O’Sullivan)
Malignant recurrence
ChemotherapySecond Cancer?
Toxicity
Chemotherapy and Glial tumors in
childrenAllen, JNS : 1998(CCG)13 childrenHigh-grade astrocytomas8-in-1 5-year survival : 54% (median : 76 Mo)But Surgery + RxTherapy
- Bouffet, Med & Ped Oncol : 1997- Biopsy - Vincristine + Carboplatin- FU : 14 Mo- Full neurologic recovery- Complete radiologically remission
Chemotherapy and Glial tumors in
children Doireau, Grill, Zerah (IGR,
Necker) : 1998
8 children Astrocytomas 5 progressive diseases,
3 relapses 3 metastases BB SFOP (CPVCEC) No Radiotherapy
Median FU : 3 years 7 alive 5 progression free 4 radiologically complete remission
To conclude
PSCT. Removable. McC ≤ III. Gr < IV
Total Removal (Frozen section ?)
Clinical Surveillance whatever the quality of the surgery
Gr I or II Astro
MR at 3 months
MR at 3 months
Nl
70%
Nl
100%
Remaining Tumor
30%
“Stable”
50%
Progression
50%
Astro I, II
T Progression
Second SurgeryChemotherapy??
Surveillance
Failure FailureRadiotherapy
Astro I, IIAstro I, II
CR, PR, Stab.
Holocord Tumor. McC = 4. Gr III or IV
Biopsy. Debulking
Gr I, II, III
Chemotherapy
Gr IV
RxT ± ChT
Second look Surgery ?
Mik. 2000. 6m-ountreatable Pain. Complete Paralysis of
the left superior limb
Astro Gr II“70% removal”(US)
Mik. 2000. 3 m post-opNo more Pain. Persistance of the paralysis
Chemotherapy (BBSFOP)
2008
Mik. 2008. 8 y F-UTotal Recovery
Stabilisation of the MR
El. 2 year-old girl
- Neck Pain-Torticollis- “Mild Traction”-Tetraplegia
El. Apr 1998
El. Oct 98 (3months)“< 90%” Removal. Mild tetraparesis
Astro Gr I
Wait and See
El. Apr 1999 (1y F-U)
No ClinicalSigns
El. Apr 2000 (2 y F-U)
El. April 2002 (4 y F-U)
El. Apr 2008 (10 y F-U)