Details

Autor(en) / Beteiligte

• Adriaensen, M. E. A. P. M.
• Schaefer-Prokop, C. M.
• Stijnen, T.
• Duyndam, D. A. C.
• Zonnenberg, B. A.
• Prokop, M.

Titel

Prevalence of subependymal giant cell tumors in patients with tuberous sclerosis and a review of the literature

Ist Teil von

• European journal of neurology, 2009-06, Vol.16 (6), p.691-696

Ort / Verlag

Oxford, UK: Blackwell Publishing Ltd

Erscheinungsjahr

2009

Quelle

Wiley Online Library All Journals

Beschreibungen/Notizen

• Background and purpose:  To investigate the prevalence of subependymal giant cell ependymomas (SEGA) in patients with tuberous sclerosis complex (TSC). Methods:  We performed a retrospective cross‐sectional study in a cohort of 285 patients with known TSC. Institutional review board approval was obtained. We included all 214 TSC‐patients who had received a contrast‐enhanced computed tomography (CT) scan of the brain. The most recent scan was evaluated for SEGA and presence of hydrocephalus. Additionally, a literature search was performed, and pooled estimates of SEGA prevalence in TSC were calculated. We used descriptive statistics, two sample t‐test, chi‐squared‐test, and meta‐analysis as appropriate. Results:  Computed tomography showed radiological evidence of SEGA in 43 of the 214 TSC‐patients (20%); 23 of 105 men (22%) and 20 of 109 women (18%; P = .52). Average maximum tumor size was 11.4 mm (range, 4–29 mm). Patients with SEGA (mean, 31 years; range, 16–58 years) were on average younger than patients without SEGA (mean, 37 years; range, 10–72 years; P = 0.007). No association between tumor size and patient age was detected. Nine patients had bilateral SEGA. Hydrocephalus was present in six of the 43 patients (14%). Meta‐analysis of reported prevalence and our current study showed that studies using radiological evidence to diagnose SEGA gave a higher pooled estimate of the prevalence of SEGA in TSC (0.16; 95% CI: 0.12, 0.21) than studies using mainly histopathological evidence of SEGA (0.09; 95% CI: 0.07, 0.12). Conclusions:  In our cohort, CT demonstrated evidence of SEGA in 20% of TSC‐patients. Prevalence of SEGA in TSC is higher in studies using radiological evidence to diagnose SEGA than in studies using histopathological evidence.