Clinical, radiological, and histological features and treatment outcomes of supratentorial extraventricular ependymoma: 14 cases from a single center

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OBJECTIVE

Reports on supratentorial extraventricular ependymoma (STE) are relatively rare. The object of this study was to analyze the clinical, radiological, and histological features and treatment outcomes of 14 patients with STE.

METHODS

Overall, 227 patients with ependymoma underwent surgical treatment in the authors’ department between January 2010 and June 2015; 14 of these patients had STE. Data on clinical presentation, radiological studies, histopathological findings, surgical strategies, and treatment outcomes in these 14 cases were retrospectively analyzed.

RESULTS

The patients consisted of 6 women and 8 men (sex ratio 0.75). Mean age at diagnosis was 24.5 ± 13.5 years (range 3–48 years). Tumors were predominantly located in the frontal and temporal lobes (5 and 4 cases, respectively). Typical radiological features were mild to moderate heterogeneous tumor enhancements on contrast-enhanced MRI. Other radiological features included well-circumscribed, “popcorn” enhancement and no distinct adjoining brain edema. Gross-total resection was achieved in 12 patients, while subtotal removal was performed in 2. Radiotherapy was administered in 7 patients after surgery. Seven tumors were classified as WHO Grade II and the other 7 were verified as WHO Grade III. The mean follow-up period was 22.6 months (range 8–39 months). There were 3 patients with recurrence, and 2 of these patients died.

CONCLUSIONS

Supratentorial extraventricular ependymoma has atypical clinical presentations, various radiological features, and heterogeneous histological forms; therefore, definitive diagnosis can be difficult. Anaplastic STE shows malignant biological behavior, a higher recurrence rate, and a relatively poor prognosis. Gross-total resection with or without postoperative radiotherapy is currently the optimal treatment for STE.

ABBREVIATIONS CE = cortical ependymoma; EMA = epithelial membrane antigen; GFAP = glial fibrillary acidic protein; GTR = gross-total resection; STE = supratentorial extraventricular ependymoma; STR = subtotal resection.

Article Information

Correspondence Kai Shu, Department of Neurosurgery, Tongji Hospital, Tongji Medical College of Huazhong University of Science & Technology, 1095 Jiefang Ave., Wuhan, Hubei 430030, PR China. email: kshu@tjh.tjmu.edu.cn.

INCLUDE WHEN CITING Published online July 7, 2017; DOI: 10.3171/2017.1.JNS161422.

Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

© AANS, except where prohibited by US copyright law.

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    Case 11. A solid, cystic lesion (arrow) located in the posterior temporal lobe (A). Case 4. A solid lesion (arrow) located in the anterior temporal lobe (B). Case 3. Axial (C) and sagittal (D) enhanced MR images showed a solid, heterogeneously enhancing lesion in the saddle and suprasellar area. Magnetic resonance spectra on adjacent parenchyma showed prominent resonance from choline, small lactate doublet, small N-acetylaspartate resonance, and high myoinositol signals (E and F). Figure is available in color online only.

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    Case 2. Enhanced MR images (A–C) obtained 4 months before surgery, showing a cystic and solid tumor mass located in the right frontoparietal lobe. The tumor size was about 37.92 × 40.11 × 33.68 mm. Enhanced MR images (D–F) obtained just before surgery, demonstrating a tumor size of about 49.25 × 46.26 × 41.53 mm, which had increased nearly 1.84 times in 4 months. H & E staining (G) demonstrated typical histological features of perivascular pseudorosettes and nuclear atypia, mitotic activity, endothelial cell hyperplasia, and tumor necrosis, corresponding to anaplastic ependymoma. Epithelial membrane antigen staining (H) was focally positive. Staining with GFAP (I) was diffusely immunopositive. S100 staining (J) was positive and demonstrated a distinct papillary pattern. Synaptophysin (K) and vimentin (L) staining were positive. Original magnifications ×10 (I and J), ×20 (G), and ×40 (H, K, and L). Figure is available in color online only.

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    Case 14. Preoperative enhanced T1-weighted MR images (A and B) showing a cystic and solid lesion located in the right parietal lobe. Postoperative enhanced T1-weighted MR images (C and D) obtained 18 months after surgery, showing a solid tumor recurrence in situ.

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    The H & E–stained sections demonstrated typical histological features of primary neurocytoma (A): small round tumor cells with neuronal differentiation and clear cytoplasm embedded in neuropil. Staining for NeuN was positive (B). Staining for synaptophysin was strongly immunopositive (C). Staining for GFAP was positive (D). Staining for S100 was partially positive (E). The H & E–stained recurrent tumor tissue demonstrated increased cellularity with perivascular pseudorosettes and uniform ependymal cells (F). Typical characteristics of anaplastic ependymoma, such as nuclear atypia, mitotic activity, endothelial cell hyperplasia, and tumor necrosis were clearly observed. Original magnifications ×10 (A and F) and ×20 (B–E). Figure is available in color online only.

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