Pinealoblastomas in children.

INTRODUCTION: Series of pinealoblastomas (PB) usually comprise small number of cases as this tumor type is extremely rare and occurs mainly in childhood (especially under 9 years of age). Frequently, PB are reported together with others pineal parenchymal tumors (PPT) or pineal tumors, making characterization far from adequate.
MATERIALS AND METHODS: Our series of CNS pediatric tumors comprises 1,350 cases of whom 16 are PPT, 12 PB, two pineocytomas (PC), and two mixed or transitional tumors (PC/PB). We have only analyzed the PB considering clinical features, treatment strategy, prognosis, recurrences, and mortality.
RESULTS: PB represented 0.89%. Mean age was 7 years. Male-female ratio was 8/4. All patients complained of increased intracranial pressure, eight presented ocular symptoms, two cerebellar, and one endocrine disturbances. Patients underwent CT scans and/or MRI. All children had negative serum and CSF markers and only one case had positive tumor cells in the CSF on admission. Hydrocephalus (12/12) was treated with ventriculoperitoneal shunt in 11/12 and endoscopic third ventriculostomy (ETV) in 1/12. We performed 11 surgical procedures (seven by occipital transtentorial approach) and one endoscopic biopsy. Total removal was achieved in two, partial removal (50-90%) in seven, and biopsy in three patients or <50%. Adjuvant therapy included radiotherapy and chemotherapy. Recurrences appeared in 8/12 cases (mean time of recurrence=27.28 months). Six patients died (mean survival=29.55 months). Mean follow up for the six patients alive was 54 months and mean follow up for all 12 children was 38.7 months.
CONCLUSION: In our opinion, PB have a poor prognosis and are very aggressive, especially in small children. Survival rate at 1 and 5 years in the present series is 66.6% (8/12) and 50% (6/12), respectively. We propose an algorithm for the treatment of pediatric patients with PB.