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Da Li, Shu-Yu Hao, Zhen Wu, Li-Wei Zhang and Jun-Ting Zhang

Medulla oblongata teratomas are rare. The authors report 2 new cases of teratomas that occurred exclusively in the medulla oblongata. The first case was in a 9-year-old boy who presented with a 6-month history of neck pain and repeated paroxysmal vomiting. Based on preoperative radiographic findings, the initial diagnosis was of an intraaxial medulla oblongata hemangioblastoma. Intraoperatively, the cystic component of the tumor was gray, gelatinous, and soft in consistency. The solid component was light pink, rubbery, and nodular in appearance, with an identifiable boundary. The lesion was completely removed. Histopathological investigation revealed a mature teratoma. Postoperatively, the patient was supported with ventilator assistance and received a tracheotomy, but died of intracranial infection. The second case was in a 10-year-old boy with intermittent headache for 1 month. Radiographs revealed an exophytic cystic and solid lesion with dorsal involvement of the medulla oblongata. The lesion was predominantly solid, pinkish gray, tenacious, and moderately vascularized, with clearly delineated surgical dissection planes. The histopathological examination confirmed a diagnosis of immature teratoma. Total resection was achieved, followed by postoperative chemotherapy. He was alive without recurrence of the lesion or symptoms at 59 months after surgery.

Resection of medulla oblongata teratoma is challenging, with inherent surgical risks that are contingent on the tumor growth pattern. Teratomas should be considered in the differential diagnosis of brainstem lesions. Chemotherapy has been suggested for immature teratomas. Long-term follow-up and larger studies of teratomas in unusual locations are required to improve practitioners' understanding of this disease's treatment and outcomes.

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Da Li, Shu-Yu Hao, Gui-Jun Jia, Zhen Wu, Li-Wei Zhang and Jun-Ting Zhang

Object

Cerebral cavernous malformations have been studied widely, but the natural history of brainstem cavernous malformations (CMs) is not well defined, and hemorrhages caused by brainstem CMs are devastating. The goal of this study was to quantify the hemorrhage risks and functional outcomes of patients with brainstem CMs.

Methods

This prospective, longitudinal, cohort study included patients with brainstem CMs diagnosed between 1985 and 2012. The clinical courses of all patients were recorded. Predictors of hemorrhage and the overall untreated outcomes were evaluated.

Results

A total of 331 patients (46.5% female) were included, with a mean follow-up duration of 6.5 years. The annual hemorrhage rates in patients initially presenting with hemorrhage with (n = 215) or without (n = 34) focal neurological deficits were 15.9% and 12.4%, respectively. However, the annual hemorrhage rate was 8.7% in patients initially presenting without hemorrhage (n = 82). The risk factors for hemorrhage were female sex (hazard ratio [HR] 1.445, p = 0.041), prior hemorrhage (HR 1.277, p = 0.029), and perilesional edema (HR 1.830, p = 0.002). Overall, neurological function at the most recent assessment was improved compared with neurological function at diagnosis. Additionally, 307 patients (92.7%) improved or stabilized, 268 (81.0%) lived independently, and 95 (28.7%) completely recovered. Predictors favoring complete recovery were no prospective hemorrhage (HR 1.958, p = 0.001), younger age (HR 1.268, p = 0.001), and small lesion size (HR 1.578, p = 0.004).

Conclusions

Patients' initial presentation predicts their prospective annual hemorrhage rate. This study suggests that several strong risk factors for hemorrhage and predictors of brainstem CM outcomes may enable clinicians to evaluate the potential hemorrhage risks of their patients and design personalized treatments.

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Xinru Xiao, Zhen Wu, Liwei Zhang, Guijun Jia, Jie Tang, Guolu Meng and Junting Zhang

Object

In this paper the authors describe a modified far-lateral transcondylar approach to remove hypoglossal neurilemmomas (HGNs).

Methods

Between September 2008 and June 2011, 11 consecutive patients with HGNs underwent tumor removal via a modified far-lateral transcondylar approach. The average age at presentation, tumor characteristics, cranial nerve (CN) deficits, and outcomes were assessed. The modified far-lateral transcondylar approach comprises several important steps. The first step is to remove the limited posterior aspect of the condylar facet to open the hypoglossal canal. The second step is to expose the posterior arch and the transverse process of C-1. A fat layer covers the venous plexus of the vertebral artery, and careful dissection along this surface of the fat layer is important to protect the vertebral artery from damage. The neck muscles are dissected caudally to expose the extracranial component of the tumor, which is located in front of the transverse process of C-1.

Results

Eleven cases of HGNs were treated during the study period. The mean patient age was 47.4 ± 8.9 years (range 31–59 years); there were 3 men and 8 women. The mean follow-up period was 14.1 ± 9 months. All patients presented with hypoglossal nerve deficits; other commonly observed deficits included glossopharyngeal and vestibular/cochlear nerve deficits. Gross-total resection of the tumor was achieved in 10 patients. A subtotal resection of the tumor was achieved in the remaining patient. Two patients had transient postoperative facial nerve palsies, 1 patient developed a new CN XI palsy postoperatively, and 5 patients experienced transient hoarseness and difficulty swallowing. Two patients required a tracheotomy because they demonstrated dysfunction of the caudal CNs and subsequently developed postoperative pneumonia. Postoperatively, 5 patients required the temporary placement of a nasogastric feeding tube. There were no surgery-related deaths in this series.

Conclusions

The modified far-lateral transcondylar approach is an effective treatment for HGNs, yielding a high total tumor removal rate with an acceptable rate of morbidity.

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Hong Wan, Liwei Zhang, Stephane Blanchard, Stephanie Bigou, Delphine Bohl, Chuncheng Wang and Song Liu

Object

Facial nerve injury results in facial palsy that has great impact on the psychosocial conditions of affected patients. Reconstruction of the facial nerve to restore facial symmetry and expression is still a significant surgical challenge. In this study, the authors assessed a hypoglossal-facial nerve anastomosis method combined with neurotrophic factor gene therapy to treat facial palsy in adult rats after facial nerve injury.

Methods

Surgery consisted of the interposition of a predegenerated nerve graft (PNG) that was anastomosed with the hypoglossal and facial nerves at each of its extremities. The hypoglossal nerve was cut approximately 50% for this anastomosis to conserve partial hypoglossal function. Before their transplantation, the PNGs were genetically engineered using lentiviral vectors to induce overexpression of the neurotrophic factor neurotrophin-3 (NT-3) to improve axonal regrowth in the reconstructed nerve pathway. Reconstruction was performed after facial nerve injury, either immediately or after a delay of 9 weeks. The rats were followed up for 4 months postoperatively, and treatment outcomes were then assessed.

Results

Compared with the functional innervation in control rats that underwent facial nerve injury without subsequent treatment, functional innervation of the paralyzed whisker pad by hypoglossal motoneurons in rats treated 4 months after nerve reconstruction was evidenced by the retrograde transport of neuronal tracers, the recording of muscle action potentials conducted by the PNG, and the recovery of facial symmetry. Although a better outcome was observed when reconstruction was performed immediately after facial nerve injury, reconstruction with NT3-treated PNGs significantly improved functional reinnervation of the paralyzed whisker pad even when implantation occurred 9 weeks posttrauma.

Conclusions

Results demonstrated that hypoglossal-facial nerve anastomosis facilitates innervation of paralyzed facial muscle via hypoglossal motoneurons without sacrificing ipsilateral hemitongue function. Neurotrophin-3 treatment through gene therapy could effectively improve such innervation, even after delayed reconstruction. These findings suggest that the combination of surgical reconstruction and NT-3 gene therapy is promising for its potential application in treating facial palsy in humans.

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Liang Wang, Zhen Wu, Kaibing Tian, Ke Wang, Da Li, Junpeng Ma, Guijun Jia, Liwei Zhang and Junting Zhang

OBJECTIVE

Skull base chordoma is relatively rare, and a limited number of reports have been published regarding its clinical features. Moreover, the factors associated with extent of resection, as well as the value of marginal resection for long-term survival, are still in question for this disease. The objective of this study was to investigate these factors by evaluating their clinical features and surgical outcomes.

METHODS

A retrospective analysis of 238 patients with skull base chordomas, who met the inclusion criteria, was performed. This study summarized the clinical features, selection of approaches, degree of resection, and postoperative complications by statistical description analyses; proposed modified classifications of tumor location and bone invasion; studied the contributions of the clinical and radiological factors to the extent of resection by Pearson χ2, ANOVA, rank test, and binary logistic regression analysis; and estimated the differences in overall survival and progression-free survival rates with respect to therapeutic history, classification of tumor location, extent of bone invasion, and extent of tumor resection by the Kaplan-Meier method. A p value < 0.05 was considered statistically significant.

RESULTS

The study included 140 male and 98 female patients with a mean age of 38.1 years. Headache and neck pain (33.2%) and diplopia (29%) were the most common initial symptoms. Sphenoclival type accounted for the largest proportion of tumor location (59.2%); endophytic chordoma was the more common type of bone invasion (81.5%). Lateral open approaches were performed in two-thirds of the study population (78.6%). The rate of marginal resection was 66%, composed of gross-total resection (11.8%) and near-total resection (54.2%). Meningitis (8%) and CSF leakage (3.8%) were the most frequent complications. The mean follow-up period was 43.7 months. The overall survival and progression-free survival rates at 5 years were 76% and 45%, respectively. Recurrent tumor and larger tumor volume (≥ 40 cm3) were identified as risk factors of marginal resection. Patients who presented with recurrent tumor and underwent intralesional resection had a worse long-term outcome.

CONCLUSIONS

The classifications of both tumor location and bone invasion demonstrated clinical value. Marginal resection was more likely to be achieved for primary lesions with smaller volumes (< 40 cm3). The rate of CSF leakage declined due to improved dura mater repair with free fat grafts. Marginal resection, or gross-total resection when possible, should be performed in patients with primary chordomas to achieve better long-term survival.

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Hong Wan, Liwei Zhang, Dezhi Li, Shuyu Hao, Jie Feng, Jean Paul Oudinet, Michael Schumacher and Song Liu

Object

Hypoglossal-facial nerve neurorrhaphy is a widely used method for treating complete facial palsy. However, the classic surgical procedure using a “side”-to-end neurorrhaphy is not suitable for incomplete facial palsy (IFP), because sectioning of the facial nerve for neurorrhaphy compromises remnant axons and potential spontaneous reinnervation. For the treatment of persistent IFP, the authors investigated in rats a modified method using hypoglossal-facial nerve “side”-to-side neurorrhaphy.

Methods

An IFP model was created by crushing the facial nerve and then ligating the injury site to limit axonal regeneration. After 9 weeks, rats with IFP were submitted to hypoglossal-facial nerve “side”-to-side neurorrhaphy: The gap between the 2 nerves was bridged with a predegenerated peroneal nerve graft, which was sutured to only one-half of the hypoglossal nerve and to the remnant facial nerve through a small window created by removing the epineurium, thus preserving regenerating facial axons.

Results

Four months after repair surgery, double innervation of the target whisker pad by hypoglossal and facial motor neurons was supported by the recording of muscle action potentials and their retrograde labeling. Regenerated hypoglossal and facial motor neurons effectively participated in the reinnervation of the whisker pad, significantly improving facial symmetry without evident synkinesis, compared with rats that underwent IFP without hypoglossal-facial nerve neurorrhaphy.

Conclusions

This study demonstrates that hypoglossal-facial nerve “side”-to-side neurorrhaphy with a predegenerated nerve graft can lead to rapid functional benefits for persistent IFP without compromising the remnants of facial axons, thus providing a proof-of-feasibility for further studies in humans.

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Jian-Cong Weng, Da Li, Liang Wang, Zhen Wu, Jun-Mei Wang, Gui-Lin Li, Wang Jia, Li-Wei Zhang and Jun-Ting Zhang

OBJECTIVE

Intracranial giant cell tumors (GCTs) are extremely rare neoplasms with dismal survival and recurrence rates. The authors aimed to confirm independent adverse factors for progression-free survival (PFS) and to propose an optimal treatment algorithm.

METHODS

The authors reviewed the clinical data of 43 cases of intracranial GCTs in their series. They also reviewed 90 cases of previously reported GCTs in the English language between 1982 and 2017 using Ovid MEDLINE, Embase, PubMed, and Cochrane databases with keywords of “giant cell tumor” or “osteoclastoma” and “skull,” “skull base,” “temporal,” “frontal,” “sphenoid,” or “occipital.” These prior publication data were processed and used according to PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines. Aforementioned risk factors for the authors’ series and the pooled cases were evaluated in patients not lost to follow-up (m = 38 and n = 128, respectively).

RESULTS

The authors’ cohort included 28 males and 15 females with a mean age of 30.5 years. Gross-total resection (GTR) was achieved in 15 (34.9%) patients. Fifteen patients (39.5%) who did not undergo GTR received postoperative radiotherapy with a mean total dose of 54.7 ± 4.1 Gy. After a mean follow-up of 71.3 months, 12 (31.6%) patients experienced recurrence, and 4 (10.5%) died of disease. The actuarial 5-year PFS and overall survival (OS) were 68.6% and 90.0% in the authors’ cohort, respectively. A multivariate Cox regression analysis verified that partial resection (HR 7.909, 95% CI 2.296–27.247, p = 0.001), no radiotherapy (HR 0.114, 95% CI 0.023–0.568, p = 0.008), and Ki-67 ≥ 10% (HR 7.816, 95% CI 1.584–38.575, p = 0.012) were independent adverse factors for PFS. Among the 90 cases in the literature, GTR was achieved in 49 (54.4%) cases. Radiotherapy was administered to 33 (36.7%) patients with a mean total dose of 47.1 ± 5.6 Gy. After a mean follow-up of 31.5 months, recurrence and death occurred in 17 (18.9%) and 5 (5.6%) cases, respectively. Among the pooled cases, the 5-year PFS and OS were 69.6% and 89.2%, respectively. A multivariate model demonstrated that partial resection (HR 4.792, 95% CI 2.909–7.893, p < 0.001) and no radiotherapy (HR 0.165, 95% CI 0.065–0.423, p < 0.001) were independent adverse factors for poor PFS.

CONCLUSIONS

GTR and radiotherapy were independent favorable factors for PFS of intracranial GCTs. Based on these findings, GTR alone or GTR plus radiotherapy was advocated as an optimal treatment; otherwise, partial resection plus radiotherapy with a dose ≥ 45 Gy, if tolerable, was a secondary alternative. Lack of randomized data of the study was stressed, and future studies with larger cohorts are necessary to verify these findings.

Systematic review no.: CRD42018090878 (crd.york.ac.uk/PROSPERO/)

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Da Li, Shu-Yu Hao, Liang Wang, Gui-Lin Li, Jun-Mei Wang, Zhen Wu, Li-Wei Zhang, Jun-Ting Zhang and Wang Jia

OBJECTIVE

Medulloepithelioma (MEPL) is a rare, malignant primitive neuroectodermal tumor with dismal survival rates. The authors aimed to define independent risk factors for progression-free survival (PFS) and overall survival (OS) and to propose an optimal treatment protocol for MEPL.

METHODS

The authors reviewed the clinicoradiological data obtained in 12 patients with MEPL who underwent surgical treatment at their institution between January 2008 and June 2016. In addition, they reviewed 55 cases of MEPL published in the literature from January 1957 to July 2017. A pooled analysis of individual patient data of these 67 patients was performed to evaluate risk factors.

RESULTS

The authors’ cohort included 5 males and 7 females with a mean age of 15.7 years. Gross-total resection (GTR) was achieved in 10 (83.3%) patients. Radiotherapy (mean total dose 42.8 Gy) and chemotherapy were administered to 7 and 4 patients, respectively. After a median follow-up of 21.7 months, 6 (50%) patients suffered recurrence and subsequently died, with median PFS and OS times of 5.5 and 13.9 months, respectively. Among the 55 patients in the literature, 13 (23.6%) patients received GTR, and 25 (49.0%) and 15 (29.4%) received radiotherapy (median total dose 53.2 Gy) and chemotherapy, respectively. After a median follow-up of 10.0 months, the recurrence and mortality rates were 69.7% (23/33) and 70.8% (34/48), respectively, and the median PFS was 6.0 months. Of the pooled cohort, the actuarial 5-year PFS and OS were 36.3% and 29.2%, respectively, and the estimated median survival time for PFS and OS were 12.8 and 15.2 months, respectively. A multivariate Cox model verified non-GTR (HR 5.537, p < 0.001) and no radiotherapy (HR 3.553, p = 0.008) as independent adverse factors for PFS. The 5-year PFS in patients with or without GTR was 63.8% and 6.3%, respectively, and in patients with or without radiotherapy was 42.7% and 23.1%, respectively. A multivariate model demonstrated non-GTR (HR 9.089, p < 0.001), no radiotherapy (HR 3.126, p = 0.004), and no chemotherapy (HR 3.621, p = 0.004) as independent adverse factors for poor OS. The 5-year OS in patients with GTR, radiotherapy, or chemotherapy was 72.1%, 44.0%, and 58.0%, respectively. In contrast, in patients without GTR, radiotherapy, or chemotherapy, the 5-year OS was 5.8%, 14.3%, and 15.8%, respectively. Overall, in patients receiving GTR plus chemoradiotherapy, the actuarial 5-year PFS and OS were both 87.5%.

CONCLUSIONS

MEPL is a rare neoplastic entity with a poor prognosis. There are no distinguishing radiological features apart from cystic degeneration. Via the pooled analysis, the authors identified independent adjustable factors associated with PFS and OS, from which they advocate for GTR plus chemoradiotherapy with a sufficient dose if tolerable as an optimal treatment to improve outcomes. Future studies with large cohorts will be necessary to verify our findings.

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Kai-Bing Tian, Jing-Jie Zheng, Jun-Peng Ma, Shu-Yu Hao, Liang Wang, Li-Wei Zhang, Zhen Wu, Jun-Ting Zhang and Da Li

OBJECTIVE

The natural history of cerebral cavernous malformations (CMs) has been widely studied, but the clinical course of untreated thalamic CMs is largely unknown. Hemorrhage of these lesions can be devastating. The authors undertook this study to obtain a prospective hemorrhage rate and provide a better understanding of the prognosis of untreated thalamic CMs.

METHODS

This longitudinal cohort study included patients with thalamic CMs who were diagnosed between 2000 and 2015. Clinical data were recorded, radiological studies were extensively reviewed, and follow-up evaluations were performed.

RESULTS

A total of 121 patients were included in the study (56.2% female), with a mean follow-up duration of 3.6 years. The overall annual hemorrhage rate (subsequent to the initial presentation) was calculated to be 9.7% based on the occurrence of 42 hemorrhages over 433.1 patient-years. This rate was highest in patients (n = 87) who initially presented with hemorrhage and focal neurological deficits (FNDs) (14.1%) (χ2 = 15.358, p < 0.001), followed by patients (n = 19) with hemorrhage but without FND (4.5%) and patients (n = 15) without hemorrhage regardless of symptoms (1.2%). The initial patient presentations of hemorrhage with FND (hazard ratio [HR] 2.767, 95% CI 1.336–5.731, p = 0.006) and associated developmental venous anomaly (DVA) (HR 2.510, 95% CI 1.275–4.942, p = 0.008) were identified as independent hemorrhage risk factors. The annual hemorrhage rate was significantly higher in patients with hemorrhagic pres entation at diagnosis (11.7%, p = 0.004) or DVA (15.7%, p = 0.002). Compared with the modified Rankin Scale (mRS) score at diagnosis (mean 2.2), the final mRS score (mean 2.0) was improved in 37 patients (30.6%), stable in 59 patients (48.8%), and worse in 25 patients (20.7%). Lesion size (odds ratio [OR] per 0.1 cm increase 3.410, 95% CI 1.272–9.146, p = 0.015) and mRS score at diagnosis (OR per 1 point increase 3.548, 95% CI 1.815–6.937, p < 0.001) were independent adverse risk factors for poor neurological outcome (mRS score ≥ 2). Patients experiencing hemorrhage after the initial ictus (OR per 1 ictus increase 6.923, 95% CI 3.023–15.855, p < 0.001) had a greater chance of worsened neurological status.

CONCLUSIONS

This study verified the adverse predictors for hemorrhage and functional outcomes of thalamic CMs and demonstrated an overall annual symptomatic hemorrhage rate of 9.7% after the initial presentation. These findings and the mode of initial presentation are useful for clinicians and patients when selecting an appropriate treatment, although the tertiary referral bias of the series should be taken into account.

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Da Li, Shu-Yu Hao, Jie Tang, Xin-Ru Xiao, Gui-Jun Jia, Zhen Wu, Li-Wei Zhang and Jun-Ting Zhang

Object

The aim of this study was to investigate the clinical appearance of untreated pediatric brainstem cavernous malformations (CMs) and to identify the hemorrhage risks and functional outcomes.

Methods

All pediatric patients with a diagnosis of brainstem CM between 1985 and 2012 were registered. The clinical chart and radiographs were recorded, and follow-up evaluations were obtained prospectively.

Results

A total of 85 patients (69.4% male) were included with a mean age of 12.7 years. Sixty-seven patients (78.8%) had prior hemorrhage, and 6 patients (7.1%) were asymptomatic. There were 15 midbrain lesions, 53 pons lesions, and 17 medulla lesions. The mean lesion size was 1.9 cm. During a total of 401.6 patient-years of follow-up, 47 hemorrhages occurred in 37 patients, and the annual hemorrhage rate was 11.7% per patient-year. The mean hemorrhage interval was 47.8 months. The hemorrhage risk declined over time, especially after the first 2 years. Both a lesion size ≥ 2 cm (hazard ratio [HR] 2.122, p = 0.037) and the presence of perilesional edema (HR 2.192, p = 0.039) predicted future hemorrhage and were associated with a high annual hemorrhage rate. The hemorrhage-free survival at 6 months was 85.7%, and at 1, 5, 10, and 15 years was 71.5%, 49.4%, 27.5%, and 13.7%, respectively. At the most recent functional evaluation, 33 patients (38.8%) had improved, 32 (37.6%) had stabilized, and 20 (23.5%) had worsened, without any deaths. Twenty-two patients (25.9%) obtained a full recovery. Prospective hemorrhage (HR 0.191, p = 0.003) was the adverse predictor for full recovery. Full recovery primarily occurred within the first 12 months, after which the chance of full recovery decreased. The cumulative percentage of complete recovery at 6 months was 32.7%, and at 1, 3, and 5 years was 40.8%, 43.6%, and 49.2%, respectively.

Conclusions

In this study the hemorrhage rate was relatively high in pediatric brainstem CMs, although the functional outcome was acceptable. The decline in hemorrhage risk and the identified adverse predictors in this study were helpful for clinicians and patients when deciding on treatment. Referral bias and the insufficient follow-up period of the study were highlighted as limitations.