Neuroblastoma with intracranial involvement: An ENSG study

Abstract

We report the experience of the European Neuroblastoma Study Group (ENSG) with central nervous system (CNS) involvement of neuroblastoma. Among this series of intensively treated patients, CNS neuroblastoma was diagnosed by computerised tomography (CT) scanning, rather than by autopsy. Cranial disease occurred in 5% of ENSG patients. Of 11 patients with intracranial disease, 4 had disease in the posterior fossa, a site rarely reported previously. Furthermore, 5 cases had CNS metastases at a time when there was no detectable disease elsewhere, rather than as part of extensive relapse. The pattern of disease we observed, at least for those with parenchymal disease, is in keeping with arterial spread. Although CT scanning is the optimal modality for identifying CNS disease, 2 cases had normal head CT scans prior to the onset of CNS disease. As most patients had symptoms of raised intracranial pressure (RICP) at the time the CNS disease was diagnosed, there does not seem to be any indication for routine CT scanning of the head at diagnosis, but this should be performed as soon as any symptoms or signs appear. With patients living longer with their disease, vigilance must be maintained during follow-up.

Central nervous system metastases in neuroblastoma

“…Headache, nausea, and emesis were associated with parenchymal disease; whereas patients with meningeal disease presented more often with pain, fever, or motor signs. We found a slightly greater proportion of patients with meningeal involvement (65%) compared with what is reported in the literature (47%), [1][2][3][4][12][13][14][15] and this value might have been even greater if routine spinal MRI studies had been performed. The time to CNS recurrence in the literature ranged from 2 months to 34 months, with median similar to the 14 months reported here.…”

“…4 The crude overall incidence in previous series of patients with CNS metastasis was 50 of 1315 patients (3.8%), slightly lower compared with our study. [1][2][3][4][12][13][14][15] However, because the rate of CNS recurrence is dependent on the length of follow-up, and none of the previous reports provided KaplanMeier estimates of recurrence over time, the crude percentage may underestimate the actual risk, which is reported here as 8% at 3 years. Almost all reported CNS metastases have occurred at the time of recurrence rather than at diagnosis.…”

Neuroblastoma metastatic to the central nervous system

“…2 Although surgery, radiotherapy, and chemotherapy often controlled tumor progression from localized CNS metastases, remission periods were temporary. The median survivals from the time of CNS recurrence were 1 month, 26 4.5 months, 27 5 months, 2 and, at best, 14 months. 24 …”

Recurrent Neuroblastoma

“…Depending on the frequency of follow-up and the type of investigations used to monitor relapse, 30 to 73% of patients are asymptomatic at relapse (Kushner et al, 2009;Lau et al, 2004 Table 1. Characteristics of 31 relapsed NB patients from a single institution (Lau et al, 2004) While central nervous system (CNS) disease is very rare at diagnosis, CNS recurrence has been reported in 1 to 5% of all patients with Stage 4 NB (Kellie et al, 1991;Kramer et al, 2001;Matthay et al, 2003a;Shaw & Eden, 1992). CNS metastases represent 6 to 10% of all disease recurrences and 50 to 70% of these CNS recurrences are isolated CNS relapse.…”