Artikel
Pineal region tumours in children: a single center experience
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Veröffentlicht: | 2. Juni 2015 |
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Objective: Tumours of the pineal region are rare. Surgery is considered to be associated with high morbidity and the ideal surgical management especially in children is quite variable. Our aim was to evaluate the presentation, entity and operative outcome of children with tumours of the pineal region in a single institution.
Method: Children (<18 years) with tumours of the pineal region admitted to our department between 1980 until 2012 were identified. Their data were retrospectively reviewed with respect to presentation, histology, treatment, complications, hydrocephalus and route of operation.
Results: 56 children were treated at our institution between 1980 and 2012. Median age at diagnosis was 9.8 years (range 0-17 years). There were 36 male and 20 female patients. Major symptoms were headache (66.1%), symptoms of increased intracranial pressure (53.6%), and gaze palsies (19.6%). 31 (55.4%) patients presented with hydrocephalus requiring urgent treatment. In 37 (66.1%) children conservative diagnostics/management was performed; 6 (10.7%) children received a biopsy.
13 (23.2%) children were operated by the infratentorial, supracerebellar approach, facilitating gross total resection in 11 (84.6%) patients. Nevertheless, 9 (69.2%) of them needed permanent ventricular shunting. Within this group, there was no fatal operative outcome. Perioperative morbidity was minimal with 1 (7.7%) pneumocephalus, and 1 (7.7%) CSF fistula. Additional postoperative ataxia and gaze palsy was present in 1 (7.7%) patient, respectively. Both symptoms resolved until 6-months follow-up.
Histology in the operated group (biopsy and infratentorial, supracerebellar approach) revealed 2 (10.5%) pineocytomas (WHO °I), 1 (5.2%) pineocytoma of intermediate differentiation (WHO °II/III), 3 (15.8%) pineoblastomas, 8 (42.1%) germinomas, and 5 (26.3%) teratomas.
Conclusions: Increased intracranial pressure (53.6%) and gaze palsies (19.6%) are the major symptoms of pineal region tumours. The infratentorial, supracerebellar approach is safe with minimal perioperative morbidity/mortality and ensures maximal tumour resection (84.6%). Shunting remains necessary in 69.2% of these patients.