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

Highlights

• Surgery is the first-line therapy for childhood pineoblastoma.

• Younger age was associated with poorer prognosis in childhood pineblastoma.

• Factors including gender, completeness of resection, and radiation therapy may influence the prognosis.

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.

Introduction

Pineoblastoma (PB) is a malignant tumor of the pineal gland, a type of primary supratentorial midline primitive neuroectodermal tumor of the central nervous system. On imaging and histologic examination, PB closely resembles medulloblastomas and retinoblastomas. PB is considered highly aggressive, with a World Health Organization classification of grade IV; approximately 24% to 50% are aggressive pineal parenchymal tumors.¹ In contrast to germ cell tumors, PB has a slightly female predilection, with a male-to-female ratio of 0.7:1.²

Clinically, the PB tumor is usually large and compresses the cerebral aqueduct, resulting in obstructive hydrocephalus. It is hydrocephalus that causes the common presenting symptoms of headaches, nausea and vomiting, or difficulty looking up. Compression on the tectal plate may induce Parinaud syndrome. Because of its high malignancy, PB is prone to invading the cerebrospinal fluid,³ and approximately 14% to 43% of patients with PB have tumor cells in the cerebrospinal fluid at the time of diagnosis.4, 5, 6

Magnetic resonance imaging (MRI) and computed tomography (CT) are valuable diagnostic methods that can reveal the location, size, and shape of the PB tumor; determine whether it is calcified; and evaluate hydrocephalus. Biopsy can determine the tumor type.1, 4, 6, 7 Surgical removal can cure some patients and at least reduces the tumor size, relieves hydrocephalus, and provides a definitive diagnosis. About 50% to 70% of PB are highly sensitive to radiation therapy. Radiation combined with chemotherapy may increase the rate of cure.1, 4, 6, 7, 8

PB is rare, and it is therefore difficult to establish an optimal treatment strategy based on clinical trial comparisons. In addition, most reported PB studies have focused on adult patients. To promote better understanding of PB in children and to provide factors that may affect the survival rate, we retrospectively reviewed the clinical characteristics of 18 younger patients with PB, and we compared treatment responses and prognoses with previous reports in the literature.

This project was authorized by the Neurosurgical Clinical Information and Biobanking Project of Beijing Tiantan Hospital (Brain Tumor Section). The Institutional Review Boards of Beijing Tiantan Hospital approved the study (register number KY2014-021-02).