Yin Zhuang, Jun Lin and Huilin Yang
Ji Eun Choi, Ho Jun Seol and Yang-Sun Cho
Endodermal cyst is a rare developmental cyst of the CNS, such as a Rathke cleft and colloid cyst lined by columnar epithelium of presumed endodermal origin. Intracranial endodermal cysts are rare, and most are found in the posterior fossa. The authors report a case of petroclival endodermal cyst with extensive bone destruction. A 12-year-old boy presented with transient facial weakness and headache. Imaging revealed a 3 × 3 × 4–cm, partial rim, enhanced cystic lesion in the petroclival area that was isointense on T1-weighted imaging and hyperintense in T2-weighted imaging. The cyst wall was partially removed and the cyst was obliterated using a lateral approach. Histological examination revealed ciliated, simple-to-pseudostratified cuboidal epithelium with a basement membrane that was consistent with an endodermal cyst, with the rare finding of xanthogranulomatous changes.
Liang Wu, Tao Yang, Xiaofeng Deng, Chenlong Yang, Lei Zhao, Ning Yao, Jingyi Fang, Guihuai Wang, Jun Yang and Yulun Xu
Extradural en plaque meningiomas are very rare tumors in the spinal canal. Most studies on these lesions have been case reports with literature reviews. In this paper, the authors review their experience in a surgical series of 12 patients with histologically proven, purely extradural en plaque meningiomas and discuss their clinical features, radiological findings, and long-term outcomes.
Clinical and imaging data of 12 patients with spinal extradural en plaque meningiomas treated at a single institution were retrospectively analyzed.
There were 5 male and 7 female patients, with a mean age of 39.9 years. The mean follow-up period was 74.8 months. Nine tumors were located in the cervical spine, 1 in the cervicothoracic spine, and 2 in the thoracic spine. All the tumors were confirmed as extradural en plaque meningiomas with sheetlike growth along the dura mater. Gross-total resection of the tumor with a well-demarcated dissection plane was achieved in 4 cases. Subtotal resection was achieved in 8 cases, 2 of whom underwent postoperative low-dose radiation therapy. The symptoms present before the surgery were improved in all cases at the last follow-up evaluation. The postoperative follow-up MRI showed no recurrence or regrowth in 4 cases with gross-total removal and 7 cases with subtotal removal during the mean follow-up periods of 58.0 months and 71.1 months, respectively. One patient experienced recurrence at 88 months after his initial subtotal removal and improved following a revision operation.
Spinal extradural en plaque meningiomas are amenable to surgery if complete removal can be achieved. Because of the encirclement of the dura that is characteristic of the tumors, complete resection is usually difficult, subtotal removal for spinal cord decompression is advised, and follow-up imaging is needed. The risk of long-term recurrence/regrowth of the lesions is low, and a good clinical outcome after total or subtotal removal can be expected.
Xiaofeng Deng, Kai Wang, Liang Wu, Chenlong Yang, Tao Yang, Lei Zhao, Jun Yang, Guihuai Wang, Jingyi Fang and Yulun Xu
Intraspinal hemangioblastomas are relatively uncommon benign tumors. The surgical strategies remain controversial, and the risk factors with regard to clinical outcome are unclear. The purpose of this study was to analyze the clinical characteristics, imaging findings, surgical strategies, and functional outcomes associated with intraspinal hemangioblastomas.
A series of 92 patients who underwent 102 operations for resection of 116 intraspinal hemangioblastomas at a single institution during 2007–2011 were consecutively enrolled in this study. Of these, 60 patients (65.2%) had sporadic hemangioblastomas and 32 (34.8%) had von Hippel-Lindau disease. Preoperatively, 13 patients underwent digital subtraction angiography (DSA), 15 patients underwent 3D CT angiography (3D CTA), and none underwent preoperative embolization. Clinical characteristics, imaging findings, and operative records were analyzed. The advantages and disadvantages of DSA and 3D CTA were compared. For identification of risk factors that affect prognosis, logistic analysis was performed.
The male/female patient ratio was 1.8:1.0 (59 male and 33 female patients). Of the tumors, 41% were intramedullary, 37% were intramedullary-extramedullary, and 22% were primarily extramedullary. Three-dimensional CTA and DSA did not differ significantly in the ability to identify the feeding arteries (p = 1.000) and image qualities (p = 0.367). However, compared with 3D CTA, the effective x-ray dose of spinal DSA was 2.73 times higher and the mean amount of contrast media injected was 1.88 times higher. Spinal DSA was more time consuming (mean 120 minutes) than 3D CTA (scanning time < 1 minute). No complications were observed after 3D CTA; acute paraparesis developed in 1 patient after DSA.
Gross-total resection was achieved for 109 tumors (94.0%), and resection was subtotal for 7 tumors. Mean duration of follow-up was 50 months (range 24–78 months). At the most recent follow-up visit, the functional outcome was improved for 38 patients (41.3%), remained stable for 40 (43.5%), and deteriorated for 14 (15.2%). Logistic analysis showed that subtotal resection was a risk factor affecting prognosis (p = 0.003, OR 12.833, 95% CI 2.429–67.806).
The authors' study suggests that safe and effective treatment of intraspinal hemangioblastomas can be achieved for most patients, even without preoperative embolization. Gross-total resection, when safe to perform, leads to better outcomes. Compared with spinal DSA, 3D CTA is a promising technique because it is noninvasive, takes less time to perform, requires lower x-ray doses and less contrast media, results in fewer complications, and offers high accuracy for delineating the feeding arteries.
Da Li, Yang Yang, Shu-Yu Hao, Liang Wang, Jie Tang, Xin-Ru Xiao, Hui Zhou, Gui-Jun Jia, Zhen Wu, Li-Wei Zhang and Jun-Ting Zhang
The aim of this study was to evaluate the pre- and postoperative rehemorrhage risk, neurological function outcome, and prognostic factors of surgically treated brainstem cavernous malformations (CMs) with long-term follow-up.
The authors conducted a retrospective review of the clinical data from 242 patients with brainstem CMs that were surgically treated between 1999 and 2010. Patient charts, imaging findings, and outcomes were examined.
The study included 242 patients, with a male-to-female ratio of 1.3 and mean age of 32.6 years. The mean modified Rankin Scale scores on admission, at discharge, at 3 and 6 months after surgery, and at recent evaluation were 2.2, 2.6, 2.3, 1.8, and 1.5, respectively. The preoperative calculated annual hemorrhage and rehemorrhage rates were 5.0% and 60.9%, respectively. The complete resection rate was 95%. Surgical morbidity occurred in 112 patients (46.3%). Eighty-five patients (35.1%) demonstrated worsened condition immediately after surgery; 34 (41.0%) and 51 (61.4%) of these patients recovered to their baseline level within 3 and 6 months after surgery, respectively. At a mean follow-up of 89.4 months, the patients' condition had improved in 147 cases (60.7%), was unchanged in 70 cases (28.9%), and had worsened in 25 cases (10.3%). A total of 8 hemorrhages occurred in 6 patients, and the postoperative annual hemorrhage rate was 0.4%. Permanent morbidity remained in 65 patients (26.9%). The adverse factors for preoperative rehemorrhage were age ≥ 50 years, size ≥ 2 cm, and perilesional edema. The risk factors for postoperative hemorrhage were developmental venous anomaly and incomplete resection. The independent adverse factors for long-term outcome were increased age, multiple hemorrhages, ventral-seated lesions, and poor preoperative status. Favorable, complete improvement in the postoperative deficits over time was correlated with good preoperative neurological function and continuing improvement thereafter.
Favorable long-term outcomes and significantly low postoperative annual hemorrhage rates were achieved via surgery. Total resection should be attempted with an aim of minimal injury to neurological function; however, postoperative deficits can improve during the postoperative course. Close follow-up with radiological examination is proposed for patients with adverse factors predictive of rehemorrhage.
Peng Li, Fu Zhao, Jing Zhang, Zhenmin Wang, Xingchao Wang, Bo Wang, Zhijun Yang, Jun Yang, Zhixian Gao and Pinan Liu
The aim of this study was to evaluate the clinical features of spinal schwannomas in patients with schwannomatosis and compare them with a large cohort of patients with solitary schwannomas and neurofibromatosis Type 2 (NF2).
The study was a retrospective review of 831 patients with solitary schwannomas, 65 with schwannomatosis, and 102 with NF2. The clinical, radiographic, and pathological data were extracted with specific attention to the age at onset, location of tumors, initial symptoms, family history, and treatment outcome.
The male-to-female ratio of patients with schwannomatosis (72.3% vs 27.7%) was significantly higher than that of patients with solitary schwannomas (53.3% vs 46.7%) and NF2 (54.0% vs 46.0%), respectively (chi-square test, p = 0.012). The mean age at the first spinal schwannoma operation of patients with NF2 (24.7 ± 10.2 years) was significantly younger than that of patients with solitary schwannomas (44.8 ± 13.2 years) and schwannomatosis (44.4 ± 14.1 years; 1-way ANOVA, p < 0.001). The initial symptoms were similar among the 3 groups, with pain being the most common. The distribution of spinal tumors among the 3 groups was significantly different. The peak locations of spinal schwannomas in patients with solitary schwannomas were at C1–3 and T12–L3; in schwannomatosis, the peak location was at T12-L5. A preferred spinal location was not evident for intradural-extramedullary tumors in NF2. Only a slight prominence in the lumbar area could be observed. The patients in the 3 groups obtained similar benefits from the operation; the recovery rates in the patients with solitary schwannomas, NF2, and schwannomatosis were 50.1%, 38.0%, and 53.9%, respectively. The prognosis varied among spinal schwannomas in the patients with schwannomatosis. Up until the last date of follow-up, most patients with schwannomatosis (81.5%) had undergone a single spinal operation, but 12 patients (18.5%) had undergone multiple spinal operations. Patients with nonsegmental schwannomatosis or those with early onset disease seemed to have a poor prognosis; they were more likely to undergo multiple spinal operations. Small cauda equina nodules were common in patients with schwannomatosis (46.7%) and NF2 (86.9%); these small schwannomas appeared to have relatively static behavior. Two patients suspicious for schwannomatosis were diagnosed with NF2 with the detection of constitutional NF2 mutations; 1 had unilateral vestibular schwannoma, and the other had suspicious bilateral trigeminal schwannomas.
The clinical features of spinal schwannomas vary among patients with solitary schwannomas, NF2, and schwannomatosis. Spinal schwannomas of patients with NF2 appear to be more aggressive than those in patients with solitary schwannomas and schwannomatosis. Spinal schwannomas of schwannomatosis predominate in the lumbar area, and most of them can be treated successfully with surgery. The prognosis varies among spinal schwannomas of schwannomatosis; some patients may need multiple operations due to newly developed schwannomas. Sometimes, it is difficult to differentiate schwannomatosis from NF2 based on clinical manifestations. It is prudent to perform close follow-up examinations in patients with undetermined schwannomatosis and their offspring.
Fu-Shan Xue, Gui-Zhen Yang, Hui-Xian Li and Ya-Yang Liu
Peixi Liu, Qingzhu An, Xi Chen, Jun Huang, Guo-Yuan Yang and Wei Zhu
Coil embolization is a safe, efficient, and minimally invasive technique for the treatment of intracranial aneurysms. However, coil embolization is associated with a higher risk of recurrence than clip ligation. In this study, the authors explore a new approach through the promotion of endothelial progenitor cells (EPCs) to optimize endothelialization of the aneurysm neck and reduce the risk of recurrence.
A coiled aneurysm model was created in 48 adult male Sprague-Dawley rats via microsurgery. Half of these animals were treated with rosuvastatin (20 mg/kg) in saline via gavage for 10, 20, or 30 days. The other half were administered saline without rosuvastatin. An additional 15 rats underwent “mock surgery” (identical anesthesia and saline gavage but no surgery). The endothelial repair process in the coiled aneurysms was evaluated via flow cytometry, im-munostaining, and electronic microscopy. The mock surgery group was used for comparison in flow cytometry studies. The effects of rosuvastatin on viability and functioning of Sprague-Dawley rat bone marrow-derived EPCs were also explored via MTT, migration, and tube formation assays.
The aneurysm neck repair score was significantly higher in the rosuvastatin-treated rats than in the untreated rats (p < 0.05). The circulating EPC count was increased and maintained at a higher level in rosuvastatin-treated rats compared with the aneurysm rats that did not receive rosuvastatin (p < 0.05). Immunostaining showed that the aneurysm neck endothelium was more integrated and the number of kinase insert domain receptor-positive cells was increased in the rosuvastatin-treated rats. Further study demonstrated that rosuvastatin promoted EPC proliferation, migration, and tube formation.
Rosuvastatin promoted endothelialization of the coiled aneurysm neck via induction of EPCs, suggesting that promoting endothelialization provides an additional therapeutic opportunity during vascular endothelium repair.
Teng-yu Li, Yu-lun Xu, Jun Yang, James Wang and Gui-Huai Wang
The aim of this study was to investigate the clinical characteristics, imaging features, differential diagnosis, treatment options, and prognosis for primary spinal epidural cavernous hemangiomas.
Fourteen patients with pathologically diagnosed non–vertebral origin cavernous hemangiomas who had undergone surgery at Beijing Tiantan Hospital between 2003 and 2012 were identified in the hospital's database. The patients' clinical data, imaging characteristics, surgical treatment, and postoperative follow-up were analyzed retrospectively.
There were 9 males and 5 females with an average age of 51.64 years. The primary epidural cavernous hemangiomas were located in the cervical spine (2 cases), cervicothoracic junction (2 cases), thoracic spine (8 cases), thoracolumbar junction (1 case), and lumbar spine (1 case). Hemorrhage was confirmed in 4 cases during surgery. Preoperatively 5 lesions were misdiagnosed as schwannoma, 1 was misdiagnosed as a meningioma, and 1 was misdiagnosed as an arachnoid cyst. Preoperative hemorrhages were identified in 2 cases. Three patients had recurrent cavernous hemangiomas. The initial presenting symptoms were local pain in 5 cases, radiculopathy in 6 cases, and myelopathy in 3 cases. Upon admission, 1 patient had radicular symptoms and 13 had myelopathic symptoms. The average symptom duration was 18 months. All patients underwent surgery; complete resection was achieved in 8 cases, subtotal resection in 4 cases, and partial resection in 2 cases. Postoperative follow-up was completed in 10 cases (average follow-up 34 months); 1 patient died, 5 patients showed clinical improvement, and 4 patients remained neurologically unchanged.
Total surgical removal of spine epidural cavernous hemangiomas with a chronic course is the optimum treatment and carries a good prognosis. Secondary surgery for recurrent epidural cavernous hemangioma is technically more challenging. In patients with profound myelopathy from acute hemorrhage, even prompt surgical decompression can rarely reverse all symptoms.
Xiaohui Ren, Junmei Wang, Mengqing Hu, Haihui Jiang, Jun Yang and Zhongli Jiang
Intracranial and intraspinal malignant peripheral nerve sheath tumors (MPNSTs) are rarely reported because of their extremely low incidence. Knowledge about these tumors is poor. In this study the authors aimed to analyze the incidence and clinical, radiological, and pathological features of intracranial and intraspinal MPNSTs.
Among 4000 cases of intracranial and intraspinal PNSTs surgically treated between 2004 and 2011 at Beijing Tiantan Hospital, cases of MPNST were chosen for analysis and were retrospectively reviewed. To determine which parameters were associated with longer progression-free survival (PFS) and overall survival (OS), statistical analysis was performed.
Malignant PNSTs accounted for 0.65% of the entire series of intracranial and intraspinal PNSTs. Twenty-four (92.3%) of these 26 MPNSTs were primary. Radiologically, 26.9% (7 of 26) of the MPNSTs were misdiagnosed as nonschwannoma diseases. Twenty-one patients were followed up for 1.5 to 102 months after surgery. Twelve patients experienced tumor recurrence, and median PFS was 15.0 months. The 2- and 3-year PFS rates were 47.7% and 32.7%, respectively. Five patients died of tumor recurrence, and median OS was not available. The 2- and 3-year OS rates were 74.7% and 64.0%, respectively. Univariate analysis revealed that female sex, total tumor removal, and primary MPNSTs were significantly associated with a better prognosis. Multivariate analysis revealed that only total removal was an independent prognostic factor for both PFS and OS.
Malignant PNST within the skull or spinal canal is a rare neoplasm and is seldom caused by benign schwannomas. Radiologically, intracranial or intraspinal MPNST should be differentiated from meningioma, chordoma, fibrous dysplasia of bone, and ear cancer. Total resection whenever possible is necessary for the prolonged survival of patients, especially males.