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Neuroblastoma with symptomatic epidural compression in the infant: the AIEOP experience.

Abstract：

BACKGROUND:Symptoms of epidural compression (SEC) in children with neuroblastoma (particularly infants) may be misinterpreted, leading to delay in diagnosis. PATIENTS AND METHODS:Clinical, imaging and follow-up data of 34 infants with neuroblastoma and SEC diagnosed between 2000 and 2011 at Italian AIEOP centers were retrieved and reviewed. RESULTS:Median age at initial SEC was 104 days (IQR 47-234). Main symptoms included motor deficit (85.3%), pain (38.2%), bladder and bowel dysfunctions (20.6% each). In the symptom-diagnosis interval (S-DI) (median, 12 days; IQR 7-34), the frequency of grade 3 motor deficit increased from 11.8% to 44.1% and that of bladder dysfunction from 20.6% to 32.4%. S-DI was significantly longer (P = 0.011) for patients developing grade 3 motor deficit. First treatment of SEC was neurosurgery in 14 patients, and chemotherapy in 20. SEC regressed in 11 patients (32.3%), improved in 9 (26.5%), and remained stable in 14 (41.2%), without treatment-related differences. Median follow-up was 82 months. At last visit, 11 patients (32.3%) were sequelae-free while 23 (67.7%) had sequelae, including motor deficit (55.9%), bladder (50.0%) and bowel dysfunctions (28.4%), and spinal abnormalities (38.2%). Sequelae were rated severe in 50% of patients. Severe sequelae scores were more frequent in patients presenting with spinal canal invasion >66% (P = 0.039) and grade 3 motor deficit (P = 0.084). CONCLUSIONS:Both neurosurgery and chemotherapy provide unsatisfactory results once paraplegia has been established. Sequelae developed in the majority of study patients and were severe in a half of them. Greater awareness by parents and physicians regarding SEC is warranted.

journal_name

Pediatr Blood Cancer

journal_title

Pediatric blood & cancer

authors

De Bernardi B,Quaglietta L,Haupt R,Castellano A,Tirtei E,Luksch R,Mastrangelo S,Viscardi E,Indolfi P,Cellini M,Tamburini A,Erminio G,Gandolfo C,Sorrentino S,Vetrella S,Gigliotti AR

doi

10.1002/pbc.25028

subject

Has Abstract

pub_date

2014-08-01 00:00:00

pages

1369-75

issue

eissn

1545-5009

issn

1545-5017

journal_volume

pub_type

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Neuroblastoma with symptomatic epidural compression in the infant: the AIEOP experience.