Surgical pathway and management of pineal region tumours in children

Pineal region tumours are rare. Surgery is associated with high morbidity, and consensus on ideal management pathways remains variable.

Patients with pineal region tumours were identified from the neuro-oncology database, and their data were retrospectively reviewed. Data collection included presentation, germ cell markers, tumour size and location, imaging, histology, treatment and control/relapse rates.

Twenty-four patients were treated at Alder Hey Children's Hospital between 1998 and 2010. Median age at diagnosis was 12.7 years. Sixteen patients presented with hydrocephalus. Twelve were successfully treated with endoscopic third ventriculostomy (EVT), and four required shunt insertion during follow-up. Fifteen patients had endoscopic biopsies. Eleven biopsies were performed at the same time as third ventriculostomy, and four patients without significant hydrocephalus had endoscopic image-guided biopsies. Eight patients had stereotactic biopsies (either because of slit ventricles at diagnosis or because of a ventriculoperitoneal shunt already in place), and only one patient had an open biopsy. No morbidity or mortality was associated with the biopsies. Histology showed four pineal parenchymal tumours, nine gliomas and eleven germ cell tumours. Thirteen patients underwent surgical resection. Significant tumour debulking was achieved in all of these patients, with gross total resection in 70 % of cases. There was one intraventricular haemorrhage (full recovery) and one mild hemiparesis. There was no surgical mortality.

The mainstay of hydrocephalus management is EVT which provides a safe and reliable route for biopsy. In our experience, craniotomy and tumour resection are effective and safe.