gms | German Medical Science

Objective: There is no general agreement on the term congenital brain tumours. Most authors agree on the definition of definitely congenital, probably and possibly congenital. Children younger than 12 months of age represent about 2% of all pediatric brain tumours and still have a poor prognosis. The purpose of this description was to analyze the specific clinical features, diagnosis and survival in terms of treatment.

Methods: This study analyzed retrospectively 22 infants with brain tumours treated in our institution between 1998 and 2011. Their clinical records were examined concerning age at diagnosis, clinical symptoms, MRI-studies, surgical procedures, histology and adjuvant therapy.

Results: 22 infants, including 15 girls and 7 boys, were diagnosed and treated between 1998 and 2011. The median age at diagnosis was 4 months, ranging between antenatal diagnosis at 34 weeks of gestation to 11 months. The main clinical feature in our group was macrocrania, an enlargement of the head, in up to 70% with diastasis of the cranial sutures or a tense fontanel. Focal neurological symptoms were seen in only 5 patients, three patients were diagnosed during prenatal ultrasound. Six patients had definitely congenital tumours and became symptomatic during the first week of life. All patients underwent surgery. Histology showed 33% (7) benign tumours, while 67% (14) had malignant tumours with rhabdoid tumours and anaplastic ependymoma in the posterior fossa as the most frequent malignant tumour types. The location favors the supratentorial compartment, less than half of the tumours presented in the posterior fossa, one large tumour (myofibroma) showed an extradural localization. Resection was complete in six patients, subtotal in 12 patients. One patient underwent stereotactic biopsy, due to the location of the lesion and two had an endoscopic biopsy. One patient died perioperatively. 15 patients received adjuvant chemotherapy.

Conclusions: Brain tumours still account for the majority of solid tumours in children. Tumours of the central nervous system in infants are rare and show a great spectrum of histological types. Most of the tumours have a massive size at diagnosis so that prognosis is still poor. The detection of a congenital brain tumour does not preclude normal development but warrants early intervention. Given the wide spectrum of possible histologies, an early multidisciplinary approach assures optimal outcome.