Background. Spinal canal invasion (SCI) occurs in 10-15% of children with newly diagnosed peripheral neuroblastic tumors (PNTs). These subjects tend to have favorable clinical and biologic characteristics at onset (low stage, thoracic primary site, “normal” biologic features) and, consequently, have a better survival probability than the general PNT population. However, the question regarding the optimal treatment of this condition (in particular, if and when neurosurgery should be applied) has not been solved yet. This is a relevant issue, since a high rate of late neurologic and orthopedic sequelae has been reported in these patients, and the treatment modalities could influence their frequency and severity.
Methods. A prospective registry to collect clinical, therapeutic and follow-up data on symptomatic and asymptomatic patients with PNT and imaging evidence of SCI was set up by the SIOPEN NB-SCI Study Group. The type and severity of symptoms were to be reported according to CTCAE (version 4.0) and ASIA scale, except for pain (FLACC scale) for children younger than 36 months. A minimum of 150 patients is expected to be recruited.
Results. Patient enrolment started on July 2014. Up to January 2016, data regarding the clinical presentation, imaging studies, biology, treatment and related response, as well as neurological and orthopedic outcome, have been collected on 24 patients from 8 countries. In the 19/24 presently evaluable, 10 are infant and 9 are older children, the thoracic primary site prevails (10/19), 11 are symptomatic and 8 are asymptomatic, 12 have localized and 7 disseminated disease (six stage 4 and one stage 4s). Patient recruitment is expected to increase after clearance from Ethics Committees of other national groups.
Conclusion. We encourage all physicians who diagnose/treat patients with PNT and SCI to participate in this prospective international cooperative study.