Choroid plexus tumors in children less than 36 months: the Canadian Pediatric Brain Tumor Consortium (CPBTC) experience.
BACKGROUND
Choroid plexus tumors (CPT) are rare pediatric tumors. A population-based study on choroid plexus carcinoma (CPC) and choroid plexus papilloma (CPP) was carried out to describe the incidence, demographic, and outcome data and to identify potential prognostic factors.


METHODS
The CPT population from the Canadian databank of CNS tumor in children ≤ 36 months diagnosed between 1990 and 2005 was reviewed


RESULTS
Out of the 579 reported cases of CNS tumors, 37 were CPT. The annual age-adjusted incidence rate was 0.22 + 0.12 (95% CI 0.16-0.28)/100,000 children < 3 years. There were 21 (56.7%) CPP and 16 (43.3.5%) CPC. Twenty patients (54%) were males. Median age at diagnosis was 7 months(range 0-30). Ten patients(62.5%) with CPC and one with CPP were metastatic at diagnosis. Twenty patients with CPP (95%) had a complete resection, whereas 6/16 CPC (37.5%) achieved a resection >90%. Fourteen CPC patients received adjuvant chemotherapy. None of the 37 patients received adjuvant radiation. At completion of survey, all CPP and five CPC were alive. Median survival time for CPC patients was 15 months (0-120). One death was related to intraoperative hemorrhage, another to chemotherapy-induced toxicity, and one to secondary AML. Age at diagnosis, degree of resection and metastatic status were not significant prognostic factors for CPC.


CONCLUSIONS
By contrast to CPC, CPP have an excellent prognosis following surgery alone. Survival of CPC remains poor. However, these data may suggest adjuvant chemotherapy can alter the aggressive natural history of CPC. As with other rare CNS tumors, international collaboration is required to identify optimal therapy.
Lafay-Cousin
Lucie
L
Pediatric Hematology Oncology and Bone marrow transplantation program, Alberta Children's Hospital, 2888 Shaganappi Trail NW, Calgary, AB, Canada. lucie.lafay-cousin@albertahealthservices.ca
Keene
Daniel
D
Carret
Anne-Sophie
AS
Fryer
Chris
C
Brossard
Josee
J
Crooks
Bruce
B
Eisenstat
David
D
Johnston
Donna
D
Larouche
Valerie
V
Silva
Marianna
M
Wilson
Beverly
B
Zelcer
Shayna
S
Bartels
Ute
U
Bouffet
Eric
E
D017668:Age of Onset; D000970:Antineoplastic Agents / Q000627:therapeutic use; D002170:Canada / Q000453:epidemiology; D002277:Carcinoma / Q000453:epidemiology* / Q000628:therapy*; D017024:Chemotherapy, Adjuvant; D002675:Child, Preschool; D016545:Choroid Plexus Neoplasms / Q000453:epidemiology* / Q000473:pathology / Q000628:therapy*; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D008297:Male; D019635:Neurosurgical Procedures; D020288:Papilloma, Choroid Plexus / Q000453:epidemiology* / Q000473:pathology / Q000628:therapy*; D011379:Prognosis
D000970:Antineoplastic Agents
Neoplasma. 2004;51(4):306-12
15254663
Pediatr Blood Cancer. 2007 Dec;49(7 Suppl):1074-82
17943961
J Neurosurg. 1987 Jun;66(6):800-5
3572508
Zentralbl Allg Pathol. 1989;135(3):235-40
2773602
J Neurooncol. 2002 Apr;57(2):123-6
12125972
Pediatr Neurosurg. 1995;22(4):189-96
7619719
Anticancer Res. 2005 Nov-Dec;25(6C):4429-33
16334121
J Neurosurg Pediatr. 2010 Jun;5(6):615-21
20515336
J Neurooncol. 2007 Dec;85(3):345-51
17576522
Int J Radiat Oncol Biol Phys. 2010 Sep 1;78(1):79-84
20004534
J Neurosurg. 1998 Mar;88(3):521-8
9488307
J Neurooncol. 2009 Dec;95(3):383-392
19543851
Cancer. 1992 Jan 15;69(2):580-5
1728390
J Neurooncol. 1992 Jan;12(1):69-74
1541980
Childs Nerv Syst. 1999 Oct;15(10):571-7
10550588
Br J Cancer. 2002 Nov 4;87(10):1086-91
12402146
Pediatr Neurosurg. 2000 Apr;32(4):192-9
10940770
N Engl J Med. 1993 Jun 17;328(24):1725-31
8388548
Int J Radiat Oncol Biol Phys. 1999 May 1;44(2):249-54
10760416