gms | German Medical Science

Objective: Neurosurgical experience with neuroblastoma (NB) is limited. Especially cranial metastases (met.) are reported on anecdotal basis only and occur in advanced stage disease. Due to a high prevalence of stage 4 NB patients (P) at our department of pediatric oncology, we report our experience in P requiring neurosurgical interventions.

Methods: We report a single center experience with regard to neurosurgical procedures related to NB.

Results: Over 150 pediatric P with stage 4 NB were treated at the pediatric oncology unit since 2009. 14 of them (8f, 6m, mean age 5.2 years, ranging from 0 to 14 years, n=13 stage 4, n=1 stage 1) needed neurosurgical therapy. 8/14 P had intracranial and 6/14 intraspinal met. In the group of 8 P with intracranial NB met. there were 3/8 P with tumor growth originating from the skull with infiltration into the adjacent soft tissue, but without penetrating the dura (occipital, frontal, presellar region in one patient, respectively). Gross total resection (GTR) was done. Carcinomatous meningiosis did occur one year later in one of them. Of the 4/8 P with intradural, intraaxial met. there were 2 located in the occipital and temporal lobe and GTR was done, respectively. In one P, tumor growth was combined intradural and extradural. GTR of the intradural and subtotal resection of the extradural tumor part was done in a staged resection, respectively. This P suffered from a central nervous lymphoma during follow-up. In one P, the tumor location was around the trigeminal nerve and subtotal resection was done. One P presented with multiple supratentorial and spinal met. and Rickham reservoir has been inserted for chemotherapy only. 6/8 P (75%) of this group died after a mean follow-up of 13 mths (range from 2 to 28 months) after their neurosurgical procedure from progressive disease.

In the group of 6 P with intraspinal, extradural tumor occurred from continuous growth through the neuroforamen. Tumor growth did respect the dura mater, but was always infiltrative to the adjacent soft tissue and bones. Tumor related spinal cord compression occurred between Th5 and S2, but was usually restricted to 1 to 3 levels. GTR of the intraspinal tumor part was done in all 6/6 P. In one patient, a relapse with intradural, intramedullary tumor growth did occur. 2 P died 5 and 10 mths after the neurosurgical procedure and 2 P were lost to follow-up, respectively. Two P present a stable disease 24 and 60 mths after the neurosurgical disease, respectively (the latter with stage 1 NB). Local radiation therapy after the neurosurgical procedure was done in some patients.

Conclusion: Neuroblastoma is a rare entity in neurosurgery and seems to be a characteristic manifestation in advanced stage disease. Cranial met. predominantly occur in the skull and grow infiltrative to the soft tissue or intraaxial and have a dismal prognosis. Therefore, surgical goals need to be clearly defined with respect to palliation.