Retrospective Analysis of the Clinical Characteristics, Therapeutic Aspects, and Prognostic Factors of 18 Cases of Childhood Pineoblastoma.

BACKGROUND

Pineoblastoma is a rare malignant tumor of the pineal gland, which is more common in children.

METHODS

We retrospectively reviewed 18 cases of pineoblastoma in children (10 girls), including general, clinical, and therapeutic information, and factors affecting prognosis.

RESULTS

The median age of the children was 51.7 months (range, 19-156 months). Presenting symptoms included vomiting (64.70%), headache (47.06%), weak or unsteady walking (35.29%), and nausea (29.41%). Rarer symptoms (1 patient each) included limb rigidity, inability to speak, double vision, fever, and Parinaud syndrome. Five and 13 children, respectively, underwent subtotal and gross total resection; 5 and 13 children received adjuvant craniospinal irradiation therapy and chemotherapy. Two children received both craniospinal irradiation and chemotherapy. The 5-year overall survival of the patients was 27.8% (5/18). The survival rate of children older than 4 years (66.7%) was significantly higher than that of younger children (8.3%). The 5-year overall survival rate of boys (50.7%) was higher than that of girls (10.0%); that of children who underwent gross total resection (30.8%) was higher than that of children who underwent subtotal resection (20.0%); and that of children treated with adjuvant craniospinal irradiation (50.7%) was higher than that of those not given craniospinal irradiation (10.0%). However, in each of these 3 comparisons the differences were not significant.

CONCLUSION

Pineoblastoma is rare but often fatal, especially in children younger than 4 years. Survival rates tend to be higher in boys, children undergoing gross total resection (rather than subtotal), and those given craniospinal irradiation.