Paediatric Spinal Cord Tumors Tumors M.ZERAH Université PARIS V. Hopital Necker Enfants Malades

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)