Neuroblastoma with symptomatic epidural compression in the infant: The AIEOP experience. Issue 8 (12th March 2014)

• Record Type: Journal Article
• Authors: De Bernardi, Bruno; Quaglietta, Lucia; Haupt, Riccardo; Castellano, Aurora; Tirtei, Elisa; Luksch, Roberto; Mastrangelo, Stefano; Viscardi, Elisabetta; Indolfi, Paolo; Cellini, Monica; Tamburini, Angela; Erminio, Giovanni; Gandolfo, Carlo; Sorrentino, Stefania; Vetrella, Simona; Gigliotti, Anna Rita
• Is Part Of: Pediatric blood & cancer. Volume 61:Issue 8(2014:Aug.)
• Page Start: 1369
• Page End: 1375
• Publication Date: 2014-03-12
• Subjects: Tumors in children -- Periodicals; Blood -- Diseases -- Periodicals; Cancer in children -- Periodicals; 618.92
• DOI: 10.1002/pbc.25028
• Languages: English
• ISSNs: 1545-5009

Abstract

<abstract abstract-type="main"> <title> <x xml:space="preserve">Abstract</x> </title> <sec id="pbc25028-sec-0001" sec-type="section"> <title>Background</title> <p>Symptoms of epidural compression (SEC) in children with neuroblastoma (particularly infants) may be misinterpreted, leading to delay in diagnosis.</p> </sec> <sec id="pbc25028-sec-0002" sec-type="section"> <title>Patients and Methods</title> <p>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.</p> </sec> <sec id="pbc25028-sec-0003" sec-type="section"> <title>Results</title> <p>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 (<italic>P</italic> = 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% (<italic>P</italic> = 0.039) and grade 3 motor deficit (<italic>P</italic> = 0.084).</p> </sec> <sec id="pbc25028-sec-0004" sec-type="section"> <title>Conclusions</title> <p>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. Pediatr Blood Cancer 2014; 61:1369–1375. © 2014 Wiley Periodicals, Inc.</p> </sec> </abstract>