Background: In this study, we reported seven cases of pediatric intracranial clear cell meningiomas (CCMs) in our institution and reviewed the relevant literature to investigate the clinicopathological characteristics, treatment options, and prognosis of these rare tumors.
Methods: From January 2005 to June 2016, we retrospectively reviewed seven pediatric intracranial CCMs in terms of their clinical data, preoperative MRI features, and prognosis. Moreover, a critical review of the English language literature was also conducted.
Results: The patients consisted of two males and five females with a median age of 10.5 years (range 6-15 years) at initial surgery. Petroclival and cerebellopontine angle area was the most common location site (5/7). Accordingly, the most common initial manifestation was hearing loss (3/7), and the mean interval from onset of symptoms to admission was 6.8 months (1.5-24 months). Gross total resection was achieved in five patients. Of the six tumors with immunohistochemical records, MIB-1 labeling index varied from 3 to 20 % (mean 8.1 %). During the follow-up period (mean 76.9 months, range 16-180 months), four patients had experienced tumor recurrences and three patients died due to recurrences.
Conclusions: Pediatric intracranial CCMs have a tendency to recur. There is a significant relationship between MIB-1 labeling index and recurrence. Gross total resection is recommended; if not available, adjuvant radiotherapy should be used to reduce the recurrent rate. In addition, postoperative MRI follow-up should be monitored at an interval time after resection.
Keywords: Clear cell meningioma; Clinicopathological features; Pediatric; Prognosis.