Intracranial meningiomas in individuals under the age of 30; Analysis of risk factors, histopathology, and recurrence rate

Abstract

Background: Intracranial meningiomas are rare in individuals under age 30 years. The purpose of this study was to analyze the demographics and clinical features of intracranial meningiomas in individuals under age 30 years. Methods: A retrospective analysis of clinical, radiographic, and pathology data of patients under age 30 years who presented with an intracranial meningioma was performed. Results: Thirty five patients met the criteria. Mean age was 25 years, 11 were male and 24 female. Five patients had previous radiation exposure. The presenting symptom was headache in 16 patients (45.7%) and seizure in 9 (25.7%). Cranial base tumors comprised 74%; 26% were supratentorial. Simpson grade 1 or 2 resection was achieved in 16 patients (48.5%); grade 3 or 4 resection was achieved in 17 patients (51.5%). Tumor pathologic grade was WHO grade I in 27 (77.1%), grade II in 6 (17.1%) and grade III in 2 patients (5.7%). Postoperative radiation therapy for tumor progression was administered in 13 patients. Radiographic follow-up was available in 26 patients at an average of 6.1 years after surgery; of these, 14 had no recurrence or progression of residual tumor while 12 had recurrence or progression. Recurrence was more common in patients with WHO grade II and III tumors and radiation-induced meningiomas. Recurrent tumors tended to have a higher WHO grade. Conclusion: In this group of intracranial meningiomas in individuals <30 years, ionizing radiation was a risk factor and tumors were more common in females. A higher percentage of WHO grade II and III tumors at presentation and recurrence was noted. Recurrent tumors were more common in patients with higher WHO grade and in radiation-induced meningiomas.

Clinicopathological and molecular characteristics of pediatric meningiomas

“…Moreover, PI3K/Akt and Hedgehog (SHH) signaling pathways which are known to be activated in adults have not been examined yet in pediatric meningiomas. Further, there are no comprehensive studies on molecular genetic alterations in cranial base meningiomas in children and young adults …”

“…A higher percentage of pediatric meningiomas are histologically atypical or anaplastic as compared to adults and display an aggressive pattern of growth and recurrence . On reviewing our database, Grades I, II and III meningiomas constituted 90.8%, 7.2% and 1.4% of cases, respectively, in adults.…”

“…The topographical distribution of pediatric meningiomas has also been demonstrated to be different from their adult counterparts. The cerebral convexity is the most frequent location for adults . Although cerebral convexity has been reported to be the most common site in several pediatric series, meningiomas in the pediatric population have a particular predilection for the lateral ventricles, posterior fossa and brain parenchyma with an absence of dural attachment .…”

“…Given their overall rarity, very few studies have attempted to analyze the clinical and molecular characteristics of pediatric meningiomas. 7,30,31 Most reports have indicated that childhood meningiomas occur in different locations and behave differently as compared to adults. 4,23 Similar to adults, chromosome 22 abnormalities are the most frequent change.…”

“…6 Higher percentages of pediatric meningiomas are histologically atypical or anaplastic (10.4% and 8.1% respectively) and display an aggressive pattern of growth and recurrence. 7 There are no clear, statistically validated guidelines for management of pediatric meningiomas. 8 Biological behavior in childhood meningiomas, on the basis of WHO grade, remains notoriously unpredictable.…”