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Clinical and neuroradiologic findings in infants with intracranial ependymomas. Pediatric Oncology Group
A M Comi1, J W Backstrom, P C Burger
1Department of Pediatrics, State University of New York at Buffalo; School of Medicine and Biomedical Sciences, USA.
Insights
Pediatric intracranial ependymomas in children under 3 years old often present with vomiting and ataxia. Imaging reveals mostly infratentorial tumors with characteristic enhancement and hydrocephalus.
Area of Science:
- Pediatric neuro-oncology
- Neuroradiology
- Pediatric oncology
Background:
- Intracranial ependymomas are a significant cause of brain tumors in young children.
- Treatment protocols for very young children (<3 years) with malignant brain tumors, including prolonged chemotherapy and delayed radiation, were investigated.
Purpose of the Study:
- To review the clinical characteristics, neuroradiologic findings, and initial imaging studies of intracranial ependymomas in patients under 3 years of age.
- To understand the typical presentation and imaging features of these tumors in a young pediatric population.
Main Methods:
- Retrospective review of 40 patients under 3 years old diagnosed with intracranial ependymomas between 1986 and 1990.
- Analysis of clinical data including presenting symptoms and duration of symptoms.
- Review of initial neuroradiologic studies, including noncontrast CT, contrast CT, T1-weighted MRI, and T2-weighted MRI findings.
Main Results:
- Common symptoms included vomiting (70%), ataxia (53%), and headache (28%).
- Eighty-eight percent of tumors were infratentorial, with an average size of approximately 4.2 cm.
- Contrast CT showed enhancement in 97% of cases, T1 MRI showed low signal intensity (67%), and T2 MRI showed high signal intensity (94%). Hydrocephalus was present in 85% of patients.
Conclusions:
- Intracranial ependymomas in very young children present with specific clinical and neuroradiologic features.
- The findings highlight the common infratentorial location, characteristic imaging findings, and high incidence of hydrocephalus in this age group.
Abstract:
Typical clinical characteristics, neuroradiologic findings, and initial neuroradiologic studies were reviewed for 40 patients <3 years of age with intracranial ependymomas, who were treated in the Pediatric Oncology Group (prolonged postoperative chemotherapy and delayed radiation for children <3 years of age with malignant brain tumors). The study included 16 females and 24 males, aged 3 to 35 months, who were diagnosed and registered in the study between 1986 and 1990. Commonly, patients presented with vomiting (70%), ataxia (53%), headache (28%), lethargy (28%), increased head circumference (23%), and irritability (23%). Duration of symptoms before diagnosis ranged from 1 day to 11 months. Thirty-five tumors (88%) were infratentorial; average tumor size was 4.3 (+/-1.4) x 4.2 (+/-1.7) x 4.1 (+/-1.8) cm at presentation. Noncontrast CT scans were performed on 23 patients; 13 (57%) were isodense to surrounding brain tissue and 13 (57%) were calcified. Contrast CT scans of 29 patients revealed that 28 (97%) were enhanced. Of the 15 T1-weighted MRI scans, 10 (67%) demonstrated low-signal intensity tumors, and 15 (94%) of the 16 T2-weighted scans revealed high-signal tumors. Forty-three percent of the tumors were cystic. Blood was observed within only 2 tumors and peritumoral edema was uncommon. Twenty-five percent of the ependymomas extended out to involve the dura, and 97% of the infratentorial tumors showed characteristic plasticity. Hydrocephalus was present in 34 (85%) children.