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Anqi Xiao, Jianguo Xu, Xin He and Chao You

Choroid plexus papilloma (CPP) is extremely rare in the brainstem. The authors report the case of a 10-year-old boy with a lesion in the pons that was misdiagnosed as a glioma preoperatively. The boy underwent partial resection of the lesion, which was diagnosed as a CPP based on histopathological findings. The authors review the MRI findings in this case and conclude that the presence of a well-defined boundary and no obvious cerebral edema are valuable features for distinguishing brainstem CPP from glioma. Although previous reports of parenchymal CPPs have described enhancement on contrast-enhanced T1-weighted MR images, the lesion in this case did not demonstrate significant enhancement. The authors note that the diagnosis of extraventricular CPP cannot be ruled out in a case of brainstem tumor without marked enhancement.

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Xiaolin Ai, Zengpanpan Ye, Jianguo Xu, Chao You and Yan Jiang

OBJECTIVE

Rupture of arteriovenous malformations (AVMs) would result in high mortality and prevalence of disability in pediatric patients. Decisions regarding the treatment of AVMs need to weigh the risk of rupture over the course of their natural history against the possibility of creating a lesion during treatment. Multiple factors have been proposed to predict hemorrhagic presentation of pediatric patients with AVMs. The aim of this meta-analysis was to evaluate the predictors of hemorrhagic presentation in pediatric patients with AVMs.

METHODS

The authors searched the PubMed and EMBASE databases. Studies reporting the predictors of hemorrhagic presentation in children with untreated brain AVMs were included. The predictive ability of identified predictors was assessed by odds ratios (ORs) and 95% confidence intervals (CIs).

RESULTS

A higher risk of hemorrhagic presentation was found in AVMs with smaller size (< 3 cm, OR 2.97, 95% CI 1.94–4.54, p < 0.00001), deep venous drainage (OR 2.28, 95% CI 1.55–3.36, p < 0.0001), a single draining vein (OR 2.23, 95% CI 1.27–3.92, p = 0.005), a single feeder (OR 3.72, 95% CI 1.31–10.62, p = 0.01), a deep location (OR 1.82, 95% CI 1.22–2.72, p = 0.004), an infratentorial location (OR 2.25, 95% CI 1.19–4.26, p = 0.01), and diffuse morphology (OR 8.94, 95% CI 3.01–26.55, p < 0.0001). In addition, the AVMs with draining vein ectasia (OR 0.35, 95% CI 0.13–0.97, p = 0.04) and high Spetzler-Martin (SM) grade (OR 0.53, 95% CI 0.36–0.78, p = 0.001) had a lower risk of hemorrhagic presentation in pediatric patients.

CONCLUSIONS

Smaller AVMs, deep venous drainage, a single draining vein, a single feeder, deep/infratentorial location, diffuse morphology, and high SM grade were identified as positive predictors for hemorrhagic presentation. Particularly, patients with diffuse AVMs have a higher risk of hemorrhagic presentation than other factors and may need active treatments. However, factors such as age, sex, draining vein stenosis, and associated aneurysms were not associated with hemorrhagic presentation.

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Yaxiong Li, Fengshi Fan, Jianguo Xu, Jie An and Weining Zhang

Primary malignant peripheral nerve sheath tumors (MPNSTs) are extremely rare in patients without a history of neurofibromatosis; only 18 cases have been reported in the English-language literature to this point. The authors report their experience with 1 new case of a primary MPNST. A 33-year-old woman presented with low-back pain radiating to the right calf that progressed over 1 year. Magnetic resonance imaging of the spine revealed an intradural extramedullary lesion at the T12–L1 level. The patient was diagnosed with primary MPNST, underwent two surgical excisions and radiation therapy, and developed leptomeningeal metastases as well as brain metastases. The patient revisited the emergency room with sudden loss of consciousness. A brain CT scan displayed bilateral lateral ventricle enlargement, for which a ventriculoperitoneal shunt was inserted. These symptoms have not been described in any previous report. Primary spinal MPNST is an exceedingly rare entity, and the overall prognosis is very poor. To the authors' knowledge, no standard of care for primary spinal MPNSTs has yet been established. All 19 cases of primary spinal MPNSTs are reviewed, and the authors discuss their clinical, radiological, and therapeutic features and outcomes.

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Xin-Yu Lu, Hui Sun, Jian-Guo Xu and Qiao-Yu Li

Object

Over the last two decades, stereotactic radiosurgery (SRS) has arisen as a promising approach in the management of brainstem cavernous malformations (CMs). In the present study, the authors report a systematic review and meta-analysis of the available published data regarding the radiosurgical management of brainstem CMs.

Methods

To identify eligible studies, systematic searches for brainstem CMs treated with SRS were conducted in major scientific publication databases. The search yielded 5 studies, which were included in the meta-analysis. Data from 178 patients with brainstem CMs were extracted. Hemorrhage rates before and after SRS were calculated, a meta-analysis was performed, and the risk ratio (RR) was determined.

Results

Four studies showed a statically significant reduction in the annual hemorrhage rate after SRS. The overall RR was 0.161 (95% CI 0.052–0.493; p = 0.001), and 21 patients (11.8%) had transient or permanent neurological deficits.

Conclusions

The present meta-analysis for the radiosurgical management of brainstem CMs shows that SRS can decrease the rate of repeat hemorrhage and has a low rate of adverse effects compared with surgery. The authors suggest that SRS may be considered as an alternative treatment for brainstem CMs that are inoperable or have a high operative risk.

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Jianguo Xu, Chao You, Sizhong Zhang, Siqing Huang, Bowen Cai, Zhenggui Wu and Hao Li

Object

Craniopharyngioma is one of the most common congenital tumors of the sellar and suprasellar regions and accounts for between 4 and 6% of all intracranial tumors. Its oncogenesis and biological behavior have not been well studied, and neither a cell line nor an animal model have been established. To better understand the tumor and improve its clinical management, the authors investigated the angiogenesis and cellular proliferation in subcutaneous craniopharyngioma xenografts obtained by implanting human tumor cells into athymic nude mice.

Methods

Human craniopharyngioma cells obtained from surgical specimens were subcutaneously implanted into BALB/c-nu/nu nude mice to establish a preliminary animal model of a transplanted tumor. Immunohistochemical staining with streptavidin–peroxidase complex was used to identify the cell phenotype and to evaluate the angiogenesis and proliferation in the xenografts. Expression of cytokeratin, minichromosome maintenance deficient 6 (MCM6) protein, and endothelial cell marker CD34 on the xenograft sections were assayed quantitatively by computer-assisted microscopy.

Twenty-seven surviving subcutaneous xenografts were obtained in 15 nude mice. The total implantation success rate was 28.12% (adamantine epithelioma [AE], 37.50%; squamous papillary tumor [SPT], 18.75%). Formation of capillaries and cell proliferation were observed in all of these xenografts. Microvessel density and degree of MCM6 immunostaining were positively correlated in the surviving grafts (r = 0.410, p < 0.05), but there was no significant difference in these variables between the AE and SPT groups (p > 0.05).

Conclusions

A preliminary animal model of human craniopharyngioma was established in the nude mouse by heterotopic implantation. Surviving xenografts maintained their vascularization and proliferation activities until harvesting at 12 weeks.