Congenital brain tumors in a series of seven patients
Farideh Nejat1, Syed Shuja Kazmi, Shahin Behjati Ardakani
1Department of Neurosurgery, Children's Hospital Medical Center, Medical Sciences, Tehran University, Tehran, Iran. nejat@sina.tums.ac.ir
Insights
Congenital brain tumors are rare but deadly, with high mortality despite advances in diagnosis and surgery. Choroid plexus papilloma offers the best prognosis, while teratomas and primitive neuroectodermal tumors have the worst outcomes.
Area of Science:
- Pediatric Oncology
- Neuro-oncology
- Developmental Neuroscience
Background:
- Congenital brain tumors are exceptionally rare pediatric neoplasms.
- This review focuses on congenital brain tumors diagnosed in infants under two months old.
Observation:
- Seven cases of congenital brain tumors were identified over a 5-year period.
- All patients presented with symptoms of intracranial hypertension and hydrocephalus.
- Neuroimaging revealed heterogeneous tumors with cystic and solid components, predominantly in the infratentorial region.
Findings:
- The diagnosed tumors included teratomas, primitive neuroectodermal tumors, ependymoblastoma, and choroid plexus papilloma (CPP).
- Despite surgical intervention in six patients, mortality rates were high due to operative complications, pneumonia, chemotherapy, and tumor recurrence.
- Only the patient with CPP survived long-term.
Implications:
- While advanced imaging improves diagnosis, congenital brain tumor mortality remains high.
- Early diagnosis and management are crucial, but prognosis is often poor.
- Choroid plexus papilloma (CPP) demonstrates a better prognosis compared to teratomas and primitive neuroectodermal tumors.
Background:
Congenital brain tumors are very rare. We review these tumors in patients younger than 2 months diagnosed in our Department.
Methods:
Seven congenital brain tumors were diagnosed during 5 years. Clinical and radiological findings and prognosis were analyzed.
Results:
The study included 5 female and two male infants. Two cases were diagnosed antenatally by means of ultrasonography. All patients presented with intracranial hypertension. All neuroimaging studies revealed nonhomogenous tumors with cystic and solid components, except for the case with choroid plexus papilloma (CPP). Hydrocephalus was evident in all of them. Most were infratentorial lesions. There were three teratomas, one primitive neuroectodermal tumor, one ependymoblastoma and one CPP. Six patients were operated on, with one intraoperative death. Two passed away postoperatively with aspiration pneumonia. One patient died due to complications of chemotherapy and another one due to tumor recurrence 1 year after surgery. Only the patient with CPP is alive after 2 years.
Conclusions:
Today, the availability of noninvasive imaging procedures such as computerized tomography scan and magnetic resonance imaging has improved the diagnosis of congenital brain tumors. In spite of development in prenatal diagnosis, appropriate pre- and postoperative management, the mortality associated with these tumors still remains high. The final prognosis in these patients is still discouraging despite early surgery and operative and anesthetic improvements. CPP is accompanied by the best prognosis, whereas teratoma and primitive neuroectodermal tumors have the worst prognosis.
