Spinal cord tumors in children under the age of 3 years: a retrospective Canadian review.
BACKGROUND
Tumors of the spinal cord are exceedingly rare in infancy and only a paucity of literature exists describing the spectrum of this disease and its management. The objectives of our study were to describe the demographic characteristics of spinal cord tumors (SCT) in children less than 3 years of age at diagnosis and to review their treatment and outcome.


METHODS
A national retrospective chart review was conducted on patients under the age of 3 years diagnosed with a primary tumor of the central nervous system (CNS) between 1990 and 2005 across Canada. Inclusion criteria were: age ≤ 3 years, histologic confirmation of the diagnosis, and residency in Canada. A centralized database was created and information regarding SCT was extracted.


RESULTS
Twenty-five of five hundred seventy-nine patients (4.3%) in the data bank had a SCT. The majority of tumors were low-grade astrocytomas (14/25). Leptomeningeal dissemination based on neuroradiologic imaging and/or cerebrospinal fluid cytology was present in five (20%) patients. The majority of patients underwent an incomplete surgical resection (52%). Most patients (64%) did not receive postoperative radiotherapy or chemotherapy. Seventy-two percent (18/25) developed recurrent/progression of disease. Overall 2- and 5-year survival for low- and high-grade malignancies was 93 ± 6.4% and 37.5 ± 17.1% respectively. Significant predictors of survival included mean duration of symptoms prior to initial diagnosis and recurrence/progression of disease.


CONCLUSIONS
Relapse/progression of disease in infant SCT is frequent. Prolonged survival of low-grade tumors is possible with further therapy; however, the prognosis of high-grade malignancies remains poor.
Division of Oncology, Children's Hospital, London Health Sciences Center, London, Ontario, N6C 2V5, Canada. Shayna.zelcer@Lhsc.on.ca
Shayna
S
Zelcer
Daniel
D
Keene
Ute
U
Bartels
Anne-Sophie
AS
Carret
Bruce
B
Crooks
David D
DD
Eisenstat
Chris
C
Fryer
Lucie
L
Lafay-Cousin
Donna L
DL
Johnston
Valerie
V
Larouche
Albert
A
Moghrabi
Beverly
B
Wilson
Mariana
M
Silva
Josee
J
Brossard
Eric
E
Bouffet
D002170:Canada / Q000453:epidemiology; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D008297:Male; D011379:Prognosis; D012008:Recurrence; D012189:Retrospective Studies; D013120:Spinal Cord Neoplasms / Q000401:mortality* / Q000473:pathology* / Q000628:therapy
J Neurosurg. 1998 Feb;88(2):215-20
9452226
Childs Nerv Syst. 2003 Sep;19(9):641-9
12908118
Med Pediatr Oncol. 1997 Dec;29(6):560-2
9324344
Int J Epidemiol. 1994 Jun;23(3):458-64
7960369
J Neurosurg. 1994 Oct;81(4):507-12
7931582
J Neurosurg. 2000 Oct;93(2 Suppl):183-93
11012047
J Neurosurg. 1997 May;86(5):747-54
9126887
J Neurosurg. 1985 Nov;63(5):669-75
4056869
Pediatr Blood Cancer. 2004 Nov;43(6):629-32
15390309
Br J Cancer. 1999 Nov;81(5):835-40
10555754
J Neurosurg. 1996 Dec;85(6):1036-43
8929492
Acta Neurochir (Wien). 1993;120(1-2):59-65
7679540
Cancer. 1998 Dec 1;83(11):2391-9
9840540
Childs Nerv Syst. 1999 Jan;15(1):17-28
10066016
J Neurooncol. 1996 May-Jun;28(2-3):245-56
8832466
Cancer. 2003 Sep 15;98(6):1243-52
12973849
J Neurosurg. 1982 Nov;57(5):685-9
7131070
Med Pediatr Oncol. 1998 May;30(5):311-2
9544231
Childs Nerv Syst. 1998 Jul;14(7):317-21
9726582