Clinical management and prognosis of spinal myxopapillary ependymoma: a single-institution cohort of 72 patients.

PURPOSE: Myxopapillary ependymoma (MPE) was classified as grade 2 tumor in the 2021 World Health Organization central nervous system classification because of its high recurrence probability. This study aimed to investigate predictive factors and management of tumor recurrence. METHODS: Seventy-two patients with spinal MPE underwent initial surgical treatment at our hospital between 2011 and 2021. Kaplan-Meier curves and Cox regression were used to analyze the correlation between clinical variables and progression-free survival (PFS). RESULTS: The median age at diagnosis was 33.5 years (range 8-60 years). Twenty-one patients (29.2%) had preoperative spinal drop metastases. Gross total resection (GTR) was performed in 37 patients (51.4%). The median follow-up was 7.2 years, and the follow-up rate was 88.9% (64 of 72 cases). Twelve of the 64 patients (18.9%) relapsed, and preoperative drop metastasis occurred in 7 patients (58.3%). The estimated 5-year and 10-year PFS rates were 82% and 77%, respectively. Univariate analysis showed that GTR was associated with improved PFS (hazard ratio [HR] 0.149, p = 0.014), while preoperative drop metastasis (HR 3.648, p = 0.027) and tumor involvement sacrococcygeal region (HR 7.563, p = 0.003) were associated with tumor recurrence. Adjuvant radiotherapy (RT) was significantly associated with improved PFS in patients with preoperative drop metastasis (p = 0.039). CONCLUSION: Complete surgical resection under the premise of protecting neurological function is an important factor in reducing spinal MPE recurrence. Adjuvant RT is recommended when the tumor invades the capsule with preoperative drop metastasis or adhesion to the nerve and cannot reach GTR.

Long-term outcomes of foramen magnum decompression with duraplasty for Chiari malformation type I in adults: a series of 297 patients.

OBJECTIVE: Foramen magnum decompression with duraplasty (FMDD) is one of the most frequently utilized surgical treatments for Chiari malformation type I (CMI) in adults. However, its long-term outcomes remain controversial. The object of this study was to evaluate the long-term outcomes of FMDD in adults with CMI. METHODS: In total, 297 adults with CMI who had undergone FMDD at the authors' institution between 2011 and 2020 were included in this retrospective study. Long-term (> 1 year) outcomes were evaluated using the Chicago Chiari Outcome Scale (CCOS), visual analog scale (VAS), and Japanese Orthopaedic Association (JOA) scale. RESULTS: The median patient age was 44 years (range 18-65 years). The mean clinical follow-up period was 67 months (range 14-123 months). Compared with preoperative conditions, the postoperative syringomyelia regression rate was 91.3% (242/265), and the cerebellar tonsil ascended in 18.2% of patients (54/297), was stable in 64.3% (191/297), and continuously descended in 17.5% (52/297). Long-term clinical follow-up data were acquired from 267 patients. According to the CCOS score, the patient's condition improved in 62.5% of cases (167/267), was stable in 31.8% (85/267), and worsened in 5.6% (15/267). According to the VAS score, the patient's condition improved in 59.5% of cases (110/185), remained unchanged in 27.6% (51/185), and worsened in 13.0% (24/185) among the follow-up patients with relevant data. According to the JOA score, the patient's condition improved in 40.1% of cases (107/267), remained unchanged in 50.2% (134/267), and worsened in 9.7% (26/267). Notably, there was no association between clinical outcomes and syringomyelia regression (p = 0.227) or changes in the cerebellar tonsillar position (p = 0.323). CONCLUSIONS: FMDD is a simple, safe, and effective surgical procedure for adult CMI that yields significant and sustained improvement in clinical and radiological outcomes. However, clinical improvement does not always correlate with syringomyelia regression and cerebellar tonsillar shift.

Spinal cysticercosis: a rare cause of myelopathy.

BACKGROUND: Neurocysticercosis is a neuroinfectious disease caused by the larval stage of the tapeworm Taenia solium. Isolated spinal cysticercosis is rare, with limited cases having been reported in the literature. This entity poses great diagnostic and therapeutic challenges. METHODS: This retrospective study included seven patients pathologically diagnosed with spinal cysticercosis. The clinical manifestations, radiological features on magnetic resonance imaging (MRI), treatment, and outcomes were analyzed. RESULTS: This case series consisted of four male and three female patients, with an average age of 34.9 ± 10.9 years. Clinically, six patients manifested with localization-related myelopathy. There were four solid lesions, one cystic-solid lesion, and three cystic lesions. The solid and cystic-solid lesions showed characteristic MRI features: 1) within the lesion, there was a mural nodule with isointensity on T1WI and iso- to hyperintensity on T2WI; 2) the signals at the periphery of the mural nodule were variable, ranging from hypointense to hyperintense on T2WI; and 3) ring-like or cyst wall enhancement could be present, and dot-like enhancement could be noted in the mural nodule. Complete resection of the responsible lesion was achieved in all patients, and oral albendazole was administered in a patient with one more suspected homologous lesion. After a mean follow-up period of 56.7 ± 35.1 months, the patient's symptoms mostly regressed. CONCLUSION: Spinal cysticercosis is an extremely rare cause of myelopathy. Characteristic MRI features can facilitate preoperative diagnosis. Clinicians should be aware of this entity, and it should be included in the differential diagnosis of myelopathy.

Autophagy triggers endoplasmic reticulum stress and C/EBP homologous protein-mediated apoptosis in OGD/R-treated neurons in a caspase-12-independent manner.

We reported that a high level of autophagy was initiated by oxygen-glucose deprivation (OGD) and was maintained in neurons even after oxygen-glucose deprivation followed by reoxygenation (OGD/R), accompanied by neuronal apoptosis. This study focused on autophagy-induced apoptosis and its signaling network, especially the role of endoplasmic reticulum stress (ERS). Analysis of primary cultured cortical neurons from mice showed that the autophagy-induced apoptosis depended on caspase-8 and -9 but not on caspase-12. This finding did not mean that the endoplasmic reticulum did not participate in this process. Increases in the levels of endoplasmic reticulum (ER) biomarkers and binding immunoglobulin protein (BiP) were induced by autophagy in OGD/R-treated neurons. In addition, as an apoptotic transcription factor induced by ER stress, C/EBP homologous protein (CHOP) expression was significantly increased in neurons after OGD/R. This result suggested that the autophagy-BiP-CHOP-caspase (8 and 9)-dependent apoptotic signaling pathway at least partly participated in autophagy-induced apoptosis in primary cortical neurons. It revealed that ER induced apoptosis in neurons suffering from OGD/R injury in an ER stress-CHOP-dependent manner rather than a caspase-12-dependent manner. However, more research on signaling or cross-linking networks and intermediate links is needed. The realization of caspase-12-independent BiP-CHOP neuronal apoptosis pathway has expanded our understanding of the neuronal apoptosis network, which may eventually provide endogenous interventional strategies for OGD/R injury after stroke.NEW & NOTEWORTHY ER stress induced by autophagy mediates caspase-8- and caspase-9-dependent apoptosis pathways by regulating CHOP in neurons exposed to OGD/R. We hypothesized that the autophagy-BiP-CHOP-caspase (8 and 9)-dependent apoptotic signaling pathway at least partly participated in autophagy-induced apoptosis in primary cortical neurons.

Clinical characteristics and surgical outcomes of ependymomas in the upper cervical spinal cord: a single-center experience of 155 consecutive patients.

Ependymomas occurring in the upper cervical spinal cord (above the level of the C4 segment) are rare entities with great therapeutic challenges. This study was aimed to investigate the clinicoradiological characteristics and the prognosis in a large cohort of upper cervical ependymomas from a single institution. This retrospective study enrolled 155 patients with primary ependymomas in the upper cervical spinal cord. The pre- and post-operative clinical and magnetic resonance imaging profiles were collected. The neurological outcomes and survival events were evaluated, and potential independent risk factors were analyzed. There were 82 females and 73 males, with an average age of 43.1 ± 11.3 years. Immediately post-operatively, 118 (76.1%) patients experienced neurological deterioration and 32 (20.7%) patients remained unchanged. Three months after surgery, 61 (39.4%) patients showed deteriorated neurological functions compared to the pre-operative baseline levels. After an average follow-up period of 56.0 ± 24.7 months, the neurological functions were worse than the baseline status in 37 (23.9%) patients and improved in 33 (21.3%) patients, respectively. Logistic regression analysis identified that lower age (≤ 42 years) and lower pre-operative MMS (I-II) were independent protective factors for predicting favorable neurological functions. Multivariate Cox regression analysis revealed that incomplete resection was the only independent risk factor associated with a shorter progression-free survival. Age and pre-operative functional status affect the long-term neurological outcomes, and incomplete resection was associated with a shorter survival. Our findings indicate that gross total resection should be the goal of surgical treatment of upper cervical ependymomas.

Chordoid glioma: an entity occurring not exclusively in the third ventricle.

Chordoid gliomas are extremely rare entities, which are generally considered occurring exclusively in the third ventricle. Despite the low-grade histological grade, aggressive behaviors have been reported in literatures. Due to the low morbidity, the origins, clinical, and radiological features, management and prognosis are still yet to be well elucidated. We retrospectively reviewed the clinical profiles from a series of 6 patients with chordoid gliomas. All patients underwent surgical treatment, and the diagnoses were based on histopathological examinations. Magnetic resonance imaging (MRI) was performed perioperatively. Follow-up outcomes were presented. This case series consisted of three male and three female patients (age range 27-67 years; mean age 43.3 years). MRI results showed tumors in the third ventricle (4/6), temporal-parietal-occipital lobe involving the lateral ventricle (1/6), and cerebellar hemisphere (1/6). Three tumors were solid, and the others were cystic-solid. Hydrocephalus was present in one patient. The T1-weighted imaging showed hypo- to isointensity, and T2-weighted imaging showed iso- to hyperintensity; enhancement was homogeneous (4/6) or heterogeneous (2/6). Diffusion-weighted imaging showed no evidence of restricted diffusion. Magnetic resonance spectrum showed an elevated choline value and reduced N-acetylaspartate value. Gross total resection was achieved in all patients, and during an average follow-up period of 35.8 months, no recurrence was noted. Chordoid gliomas can occur outside the third ventricle with a great diagnostic challenge. The MRI characteristics suggest a low-grade tumor, and the accurate diagnosis depends on pathological criteria. Complete surgical resection is associated with a favorable outcome.

Clinical characteristics and surgical outcomes of spinal myxopapillary ependymomas.

Spinal myxopapillary ependymoma (SME) is a rare pathological variant of ependymoma, which most commonly occurs in the cauda equina and filum terminale of the spinal cord. Although SME is considered as a benign entity, histologically corresponding to WHO grade I, local recurrence and metastasis have been reported in many cases. The purpose of this large-scale, single-center study was to investigate the clinical characteristics and surgical outcomes of SME. A total of 34 consecutive patients diagnosed with SME were enrolled in this retrospective study. All patients underwent magnetic resonance imaging (MRI) and were treated with surgical resection. Individual clinical data were collected, and surgical outcomes were evaluated during the follow-up period. There were 21 males and 13 females, with an average age of 29.97 years. Clinical symptoms included back pain (82.4%), weakness (44.1%) and numbness (20.6%) in extremities, and sphincter dysfunction (26.5%). The tumor locations included lumbar segments (52.9%), thoracolumbar segments (23.5%), lumbosacral segments (17.6%), and thoraco-lumbo-sacral segments (5.9%). On MRI, all SMEs appeared hypointense on T1-weighted imaging and hyperintense on T2-weighted imaging, with remarkable enhancement. Gross total resection was achieved in 18 patients, and subtotal resection (STR) was achieved in 16 patients. After an average follow-up period of 41.5 months, recurrence was noted in 5 patients (5 of 16) who underwent STR. SMEs have some characteristic features on MRI which can facilitate the preoperative diagnosis. Complete surgical resection is the best treatment of choice with a favorable outcome. In cases of incomplete resection, postoperative radiotherapy may be an effective alternative.

Shunt dependency syndrome and acquired Chiari malformation secondary to cerebrospinal fluid diversion procedures: a 9-year longitudinal observation.

BACKGROUND: Shunt dependency syndrome is a rare long-term complication of cystoperitoneal (CP) shunting for intracranial arachnoid cysts, which is characterized by acute intracranial hypertension with normal-sized or small ventricles. Additionally, acquired Chiari type I malformations (ACIM) could be infrequently secondary to extrathecal shunt drainage of cerebrospinal fluid. CASE REPORT: We described a 12-year-old boy who developed shunt dependency syndrome following a CP shunting for treating a temporal arachnoid cyst. To manage this rare complication, we placed a lumboperitoneal (LP) shunt. During the follow-up period, shunt-induced ACIM and concomitant syringomyelia were noted. CONCLUSION: Shunt dependency syndrome is a rare complication secondary to CP shunting in the treatment of temporal arachnoid cysts, and LP shunting is an effective option to relieve the intracranial hypertension. However, the clinicians should be alert to the ACIM as a rare late complication of cerebrospinal fluid diversion procedures, and the potential protecting effect of the programmable valve should be emphasized.

Mixed gangliocytoma-pituitary adenoma in the sellar region: a large-scale single-center experience.

BACKGROUND: Mixed gangliocytoma-pituitary adenoma is an extremely rare tumor occurring in the sellar region, histologically composed of both gangliocytic and pituitary adenomatous architectures. The histogenesis of these tumors remains unknown, and the diagnosis, treatment, and prognosis are yet to be fully understood. METHODS: We retrospectively reviewed the clinical, radiological, and histopathological profiles from a series of 20 patients with sellar mixed gangliocytoma-pituitary adenomas. All patients underwent surgical tumorectomy via an endoscopic or microscopic transsphenoidal approach. Perioperative magnetic resonance imaging (MRI) and computed tomography (CT) data were reviewed. Immunohistochemical stains and electron microscopy examination were performed. Follow-up outcomes were presented. RESULTS: This case series consisted of 13 females and seven males (age range, 20-59 years; mean age, 42.3 ± 11.2 years). Preoperative endocrine examination showed elevated growth hormone (GH) in nine patients and hyperprolactinemia in eight patients. The tumors were positive for GH in 15 cases, prolactin in 13 cases, adrenocorticotropic hormone in three cases, and thyroid-stimulating hormone in one case. Gross total resection was achieved in 15 patients, and subtotal resection in five patients. During an average follow-up period of 42.5 ± 29.0 months, no recurrence was noted. CONCLUSIONS: The clinical and neuroimaging features of sellar mixed gangliocytoma-pituitary adenomas are non-specific, and invasion into the cavernous sinus is common. Surgical resection via a transsphenoidal approach is the preferred treatment, and the surgical outcomes are favorable. Moreover, our histopathological findings are more likely to support the theory that mixed gangliocytoma-pituitary adenoma originates from the neuronal transdifferentiation of adenomatous cells.

Clinical features and long-term outcomes of primary spinal malignant melanoma: a single center experience.

Primary malignant melanomas are very rare tumors in the spinal canal. In this study, the authors review their experience in a series of seven patients with histologically proven primary spinal malignant melanoma (PSMM) and discuss the clinical features, treatment strategy, and long-term outcomes. Clinical data of seven patients with PSMM treated at a single institution were retrospectively analyzed. There were three male and four female patients, with a mean age of 44 years. The mean duration of illness was 5.4 months. The tumors showed hyperintensity in six cases on T1-weighted image (WI) and isointensity or hypointensity in five cases on T2WI. Gross total resection (GTR) of the tumor was achieved in two cases, and subtotal resection (STR) was achieved in five cases. Four STR patients underwent postoperative local radiation therapy. Postoperative MRI results showed no tumor recurrence in all four female patients after an average follow-up period of 64.5 months. Three male patients had tumor recurrence and dissemination after postoperative 14.7 months (8-24 months), and all died 16.3 months (10-25 months) after initial diagnosis. PSMM should be considered in the differential diagnosis of a middle-aged patient with spinal lesion if the tumor shows hyperintensity on T1WI and hypointensity or isointensity on T2WI on MRI. STR followed by radiotherapy is not excessively associated with deterioration of the final outcome compared to GTR. Our study suggests that PSMM might have female predominance in favorable outcome. Surgical resection followed by adjuvant radiotherapy and regular follow-up are recommended.

Clinical features and long-term outcomes of pediatric spinal meningiomas.

Pediatric spinal meningiomas are very rare. Most studies on pediatric spinal meningiomas are case reports with literature reviews. This study presented a surgical series of 14 pediatric patients with histologically proven sporadic spinal meningiomas, including 5 WHO grade II tumors. In this series, there were 9 male and 5 female patients with a mean age of 11.1 years. Seven tumors were located in the cervical spine, 5 in the thoracic spine, and 2 in the lumbar spine. Gross total resection (GTR) of the tumor with a well-demarcated dissection plane was achieved in 11 cases, subtotal resection was achieved in 2 cases, and partial resection was performed in 1 case. The symptoms were improved in 10 cases at the last follow-up and the current status of 2 patients worsened and 1 patient had no change compared to the preoperative presentation. One patient died of progressive tumor regrowth after partial resection and adjuvant radiotherapy. The postoperative follow-up magnetic resonance imaging showed tumor recurrence in one of the 11 GTR cases during the mean follow-up period of 72.5 months. Recurrence/regrowth of the residual tumors was observed in both the 2 STR cases. Although pediatric spinal meningiomas have a higher incidence of WHO grade II meningiomas, they are amenable to surgery if it is possible to achieve GTR. A good clinical outcome after GTR can be expected, even for WHO grade II meningiomas. Effect of adjuvant radiochemotherapy for controlling the tumors is still unclear and requires further investigation.

Pediatric infratentorial meningiomas: a series of 19 cases and review of the literature.

PURPOSE: Pediatric infratentorial meningiomas are extremely rare. In this article, we present a series of 19 cases operated at our institution in the last 8 years. METHODS: During the 8-year period from January 2008 to December 2015, we encountered 21 cases suffered from infratentorial meningiomas. Two patients were excluded. The clinical profiles, radiological features, surgical procedures, intraoperative findings, and outcomes were extracted from the patient records and neuroimaging data. RESULTS: The 19 cases with pediatric infratentorial meningiomas account for about 12.8% of all pediatric intracranial meningiomas. The age distribution of the patients ranged from 7 to 18 years. There were 9 male and 10 female patients. Cranial nerve defects were the first common signs and symptoms found in most cases (n = 14). One meningioma localized in cerebellum, one originated in jugular foramen. Meningioma involving internal auditory canal was only seen in one of the six patients with CPA meningioma. One tumor was totally ossified. In all, total resection was achieved in 14 patients, subtotal resection in 5 patients. Gamma knife was used for the recurrent and subtotally resected tumors. Conventional radiotherapy was applied for high-grade meningiomas (WHO grade II and WHO grade III). CONCLUSION: Pediatric infratentorial meningiomas are different from supratentorial ones in many aspects, such as onset age, gender ratio, and neuroradiological characteristics. Surgical excision is challenging. According to the locations of tumors, different surgical approaches would be chosen to maximally resect the lesions without damage to brain stem and cranial nerves. Gamma knife and conventional radiotherapy could be used as postoperative adjuvant therapies. Long-term clinical follow-up and serial imaging are recommended.

Spinal meningeal melanocytomas: clinical manifestations, radiological and pathological characteristics, and surgical outcomes.

Meningeal melanocytoma is a rare benign tumor, most frequently located in the posterior fossa and spinal canal. The aim of this study was to investigate the clinical manifestations, radiological features, management, and follow-up data of spinal meningeal melanocytomas. We present the clinical data and long-term outcomes from a consecutive surgical series of 16 patients with pathologically diagnosed spinal meningeal melanocytomas. All of the patients underwent surgical resection. Pre and postoperative MRI was performed. Follow-up data and neurological functional assessment is presented and discussed. The mean age at diagnosis was 42.0 years, with a significant male predominance. The primary clinical symptoms were weakness or numbness of the extremities. The appearance of melanocytoma on MRI is typically isointense to hyperintense on T1-weighted images, hypointense on T2-weighted images, and contrast enhancement tends to be remarkable and homogeneous. In most cases, gross total resection is achievable; however, in rare cases with dumbbell-shaped tumors involving the extraspinal region, a staging operation and subtotal resection should be attempted. During a mean follow-up period of 58.1 months, the symptoms were completely relieved in all the patients, and no tumor progression or recurrence was noted. Melanocytic tumors of the central nervous system have a typical appearance on MRI scans, varying with the content and distribution of melanin. However, the differential diagnosis between malignant melanoma and melanocytoma still depends on pathological criteria. Spinal meningeal melanocytoma has a benign course, and it is amenable for gross total resection. The outcome is favorable following complete resection.

[The clinical characteristics and microsurgery treatment of patients with spinal cord glioblastoma].

OBJECTIVE: The aim is to explore the clinical characteristics of spinal cord glioblastoma and the therapeutic effect of microsurgery. METHODS: The clinical data of 18 patients with spinal cord glioblastoma from January 2011 to December 2014 in Beijing Tiantan Hospital were retrospectively analyzed including the clinical characteristics, the microsurgery treatment and the postoperative radiochemotherapy. RESULTS: There were 12 cases for subtotal resection, 4 cases for partial resection and 2 cases for biopsy of the intraspinal tumors under microscope. The nervous system symptoms were improved in 11 cases, no changes in 5, deterioration in 2 and no deaths within 3 months after the operation. Among those, 5 cases (100%) with preoperative McCormick grade Ⅰ, 4 (57%) with grade Ⅱ, 2 (50%) with grade Ⅲ, and 0 with grade Ⅳ had improved. There were McCormick gradeⅠ8 cases, grade Ⅱ 5 cases, grade Ⅲ 2 cases and grade Ⅳ 3 cases within 3 months after the operation. Seventeen patients were followed up from 6 to 36 months, and 1 patients was lost to follow-up. Five patients returned to normal work and study, while 8 died. The median survival time was 16 months. CONCLUSIONS: Spinal cord glioblastoma is highly malignant with low incidence and poor prognosis, which should be performed by early operative treatment and postoperative adjuvant radiochemotherapy.

Treatment strategies and long-term outcomes for primary intramedullary spinal germinomas: an institutional experience.

Primary intramedullary germinomas are very rare tumors in the spinal cord. This study presented a series of 11 patients with histologically proven primary intramedullary spinal germinomas. Their clinical and radiological findings, treatment records and long-term outcomes were reviewed. There were four male and seven female patients with a mean age of 27.1 years. Because germ cell tumors were suspected by frozen-section biopsy, gross total resection was unattempted. Partial resection was performed in four cases, while biopsy was performed in seven cases. Postoperatively, carboplatin and etoposide chemotherapy combined with low dose radiotherapy (30.6 Gy) to local spine was performed in seven cases, followed by radiotherapy alone (40 Gy) in four cases. All 11 patients had a complete response to either combination treatment or single radiotherapy, and the mass effect on the spinal cord vanished. The mean follow-up period was 75.4 months. At the last follow-up, the symptoms were improved in 10 cases and the current status of one patient was unchanged. The postoperative follow-up magnetic resonance imaging showed no recurrence or dissemination in any of the patients. Primary intramedullary germinomas are amenable to adjuvant radiochemotherapy and low dose radiation with etoposide and carboplatin chemotherapy is recommended. When combination therapy cannot be performed, relatively high doses of radiotherapy are advised, and radiation to the craniospinal axis may be unnecessary. A good clinical outcome after combination therapy or radiotherapy alone can be expected, and the risk of long-term recurrence and dissemination is low.

Clinical characteristics and surgical outcomes of primary spinal paragangliomas.

Spinal paragangliomas are extremely rare tumors, most frequently involving the cauda equina and the filum terminale. We aimed to investigate the clinical manifestations, radiological features, management, and follow-up data of primary spinal paraganglioma. We present the clinical data and long-term outcomes from a consecutive surgical series of 19 patients with pathologically diagnosed spinal paragangliomas. All of the patients had undergone surgical resection. Pre- and postoperative magnetic resonance imaging was performed and follow-up data and neurological functional assessment are presented and discussed. The mean age at diagnosis was 47.7 years, with a significant male predominance. The primary clinical symptoms were low back pain and sciatica. Magnetic resonance images (MRI) showed characteristic signs that help differentiate paragangliomas from other spinal tumors, including a "salt & pepper" sign, serpiginous flow void, and a peripheral hypointense rim. Also, a well-encapsulated appearance can be found intraoperatively. During a mean follow-up period of 62.1 months, remnant tumor progression was noted on MRI in three patients with incomplete resection. Pain symptoms were relieved immediately after surgical intervention, while motor and sphincter dysfunction were much slower to improve. Differential diagnosis of paraganglioma based on MR images alone is challenging, but the presence of specific characteristic features provides suggestive clues; however, accurate diagnosis depends on pathological criteria. Despite the benign course, gross total resection is ideal, given an increased risk of recurrence in situ. Timely recognition and surgical treatment should be emphasized to avoid progressive neurological deficits.

Clinical presentation and long-term outcome of primary spinal peripheral primitive neuroectodermal tumors.

Primary spinal peripheral primitive neuroectodermal tumors (pPNETs) are extremely rare entities that predominantly occur in children and young adults. Few studies have reported more than three cases. There are no current optimum treatment strategies due to the paucity of data. Here, we present 13 patients (nine females and four males) with primary intraspinal pPNETs who were surgically treated from April 2008 to February 2014. Histopathologic findings revealed the expression of CD99 in all cases. Limb weakness was the most common initial symptom (11/13, 85 %). The tumors were located mainly at the cervical level (6/13, 46 %) and in the epidural space (10/13, 77 %). The radiological diagnosis was neurinoma or meningioma in most cases (10/13, 77 %). Gross total resection was achieved in 77 % (10/13) of patients. During a mean follow-up of 25.5 months, local relapse occurred in 8 (61.5 %) patients and distant metastases occurred in 8 (61.5 %) patients. The overall 1-year survival rate was 77 % (10/13), and the overall 2-year survival rate was 54 % (7/13). The 2-year survival rate was 57.1 % in patients with adjuvant chemotherapy and 50 % in those without chemotherapy. Gross total resection and adjuvant radiotherapy with or without chemotherapy demonstrated a longer survival period (1-year survival rate: 100 %; 2-year survival rate: 86 %). Our data showed that primary spinal pPNETs are extremely rare and aggressive tumors with a poor prognosis. Radical resection is advocated. Gross total resection combined with adjuvant radiation may help to significantly improve patient survival period. Chemotherapy may also help to slightly prolong patient life.

Clinical characteristics and treatment strategies for idiopathic spinal extradural arachnoid cysts: a single-center experience.

BACKGROUND: Idiopathic spinal epidural arachnoid cysts (SEACs) are relatively rare lesions with diverse clinical manifestations depending on the affected spinal region and nerve roots. To assess the relationship between clinical presentation and surgical outcome, we conducted a retrospective review of medical records and neuroimages from idiopathic SEAC patients. METHODS: Of 15 consecutive patients with idiopathic SEACs, 14 underwent surgical treatment at the Beijing Tiantan Hospital between January 2008 and July 2013. Case histories, clinical presentations, imaging findings, operative findings, and surgical outcomes were reviewed. RESULTS: Of the 15 patients, 5 were children (median age, 13 years) and 10 were adults (median age, 35 years). The male-to-female ratio was 4:1. The median disease duration was 1 year in children and 4 years in adults. The most common manifestations were progressive paraparesis (73.3 %) and radiculopathy (66.7 %). All pediatric lesions affected thoracic segments, while 80 % of adult lesions affected lumbar segments. Fourteen patients underwent microsurgical treatment, with cyst excision in nine (combined with fistula closure in four) and partial cyst excision in five (with fistula closure in three). No recurrences were observed during follow-up (average of 23 months). Excellent outcome was achieved in six cases, good outcome in four, fair in two, and poor in two. CONCLUSIONS: Idiopathic SEAC mainly affect the thoracic segments in children and the lumbar segments in adults, and often results in progressive paraparesis and radiculopathy. Early surgical intervention is suggested for progressive symptomatic patients to restore neurological function.

Intramedullary gangliogliomas: clinical features, surgical outcomes, and neuropathic scoliosis.

Intramedullary spinal cord gangliogliomas are rare tumors composed of glial components and ganglion cells. These gangliogliomas are generally considered as slow-growing tumors, corresponding histologically to WHO grade I or II. There are few reports of large case series of intramedullary spinal cord gangliogliomas from a single center. We retrospectively reviewed a consecutive series of 18 patients with pathologically diagnosed ganglioglioma. Clinical manifestations, radiological features, treatment and follow-up data, and concomitant scoliosis were investigated. The mean age at diagnosis was 27.5 years, with a slight female predominance. The primary clinical symptoms were sensorimotor deficits. Magnetic resonance (MR) imaging manifestations varied considerably. Some associated, but not necessary, features were found, such as young age at onset, large tumor dimension, and bony changes. Scoliosis was observed in seven patients. Remnant tumor progression was observed in five patients during the follow-up period, and no deaths occurred. The last neurological evaluation showed functional improvement from preoperative status in five patients. Differential diagnosis of ganglioglioma based on MR images alone is challenging, but the combination of some characteristic features can be helpful. An accurate diagnosis of ganglioglioma depends on pathological criteria. Despite the benign course of ganglioglioma, considerable growth may affect its resectability and prognosis. The extent of resection should be meticulously planned, and the potential risk of recurrence and neurological deterioration should be evaluated. The concomitant scoliosis is noteworthy.

Surgical outcomes in spinal cord subependymomas: an institutional experience.

Spinal cord subependymomas are very rare. Most studies on spinal cord subependymomas have been case reports with literature reviews. This study presented a surgical series of 13 patients with histologically proven spinal cord subependymomas. Their clinical data, radiological findings, operative records, and follow-up outcomes were reviewed. There were 5 male and 8 female patients with a mean age of 39.5 years. The mean follow-up period was 67.8 months. Four tumors were located in the cervical spine, 5 in the cervicothoracic spine, and 4 in the thoracic spine. Gross total resection (GTR) of the tumor with a well-demarcated dissection plane was achieved in 9 cases, and subtotal resection was achieved in 4 cases. The symptoms present before the surgery were improved in 11 cases at last follow-up and the current status of 2 patients had no change compared to the preoperative presentation at last follow-up. The postoperative follow-up magnetic resonance imaging showed no recurrence in the 9 GTR cases during the mean follow-up period of 70.3 months. No recurrence/regrowth of the residual tumors was observed in the 4 STR cases during the mean follow-up period of 62.0 months. Spinal cord subependymomas are amenable to surgical resection. It is possible to achieve GTR of intramedullary subependymomas that have a well-demarcated dissection plane. When GTR cannot be achieved, STR of the lesion for decompression is advised, and follow-up imaging is needed. A good clinical outcome after GTR or STR can be expected.