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  • 1
    In: Journal of Neurosurgery, Journal of Neurosurgery Publishing Group (JNSPG), Vol. 129, No. 3 ( 2018-09), p. 805-814
    Abstract: While sporadic peripheral schwannomas (SPSs) are generally well treated with surgery, their biology is not well understood. Consequently, treatment options are limited. The aim of this study was to provide a comprehensive description of SPS. The authors describe clinicopathological features and treatment outcomes of patients harboring these tumors, and they assess expression of biomarkers using a clinically annotated tissue microarray. Together, these data give new insight into the biology and management of SPS. METHODS Patients presenting with a primary SPS between 1993 and 2011 (n = 291) were selected from an institutional registry to construct a clinical database. All patients underwent follow-up, and short- and long-term outcomes were assessed. Expression of relevant biomarkers was assessed using a new tissue microarray (n = 121). RESULTS SPSs were generally large (mean 5.5 cm) and frequently painful at presentation (55%). Most patients were treated with surgery (80%), the majority of whom experienced complete resolution (52%) or improvement (18%) of their symptoms. Tumors that were completely resected (85%) did not recur. Some patients experienced short-term (16%) and long-term (4%) complications postoperatively. Schwannomas expressed higher levels of platelet-derived growth factor receptor–β (2.1) than malignant peripheral nerve sheath tumors (MPNSTs) (1.5, p = 0.004) and neurofibromas (1.33, p = 0.007). Expression of human epidermal growth factor receptor–2 was greater in SPSs (0.91) than in MPNSTs (0.33, p = 0.002) and neurofibromas (0.33, p = 0.026). Epidermal growth factor receptor was expressed in far fewer SPS cells (10%) than in MPNSTs (58%, p 〈 0.0001) or neurofibromas (37%, p = 0.007). SPSs more frequently expressed cytoplasmic survivin (66% of tumor cells) than normal nerve (46% of cells), but SPS expressed nuclear survivin in fewer tumor cells than in MPNSTs (24% and 50%, respectively; p = 0.018). CONCLUSIONS Complete resection is curative for SPS. Left untreated, however, these tumors can cause significant morbidity, and not all patients are candidates for resection. SPSs express a pattern of biomarkers consistent with the dysregulation of the tumor suppressor merlin observed in neurofibromatosis Type 2–associated schwannomas, suggesting a shared etiology. This SPS pattern is distinct from that of other tumors of the peripheral nerve sheath.
    Type of Medium: Online Resource
    ISSN: 0022-3085 , 1933-0693
    RVK:
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    Language: Unknown
    Publisher: Journal of Neurosurgery Publishing Group (JNSPG)
    Publication Date: 2018
    detail.hit.zdb_id: 2026156-1
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  • 2
    Online Resource
    Online Resource
    Decker Medicine ; 2018
    In:  DeckerMed CGSO Case-Based Reviews ( 2018-12-11)
    In: DeckerMed CGSO Case-Based Reviews, Decker Medicine, ( 2018-12-11)
    Abstract: Peripheral nerve sheath tumors represent a spectrum of entities ranging from benign tumors, such as schwannoma, to high-grade malignant tumors termed malignant peripheral nerve sheath tumors. In this chapter, we discuss current concepts and problematic areas in the diagnosis, treatment, and management of peripheral nerve sheath tumors. We discuss the major categories of nerve sheath tumors including schwannomas, neurofibromas, and malignant peripheral nerve sheath tumors. The different benign variants and their propensity for malignant transformation are highlighted. We suggest useful guidelines for the surgical management of these challenging cases. Finally, we discuss some of the advances in molecular biology that have provided new insights into the nature of the various peripheral nerve sheath tumors and suggest potential novel targeted therapeutic strategies for these tumors. This review contains 11 figures, 5 tables and 66 references. Key Words: malignant peripheral nerve sheath tumors, nerve sheath tumor, neurofibroma, neurofibromatosis, schwannoma, schwannomatosis, NF1, NF2
    Type of Medium: Online Resource
    Language: Unknown
    Publisher: Decker Medicine
    Publication Date: 2018
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  • 3
    In: Journal of Neurosurgery: Spine, Journal of Neurosurgery Publishing Group (JNSPG), Vol. 20, No. 1 ( 2014-01), p. 108-116
    Abstract: Renal cell carcinoma (RCC) frequently metastasizes to the spine, and the prognosis can be quite variable. Surgical removal of the tumor with spinal reconstruction has been a mainstay of palliative treatment. The ability to predict prognosis is valuable when determining the role and magnitude of surgical intervention in cancer patients. To better identify factors affecting survival in patients undergoing surgery for spinal metastasis from RCC, the authors undertook a retrospective analysis of a large patient cohort at a tertiary care cancer center. Methods Relevant clinical data on a consecutive series of patients who had undergone surgery for spinal metastasis of RCC between 1993 and 2007 at The University of Texas MD Anderson Cancer Center were retrospectively reviewed. Demographic data, histopathological grade of primary tumor, timing of spinal surgery relative to diagnosis, treatment history prior to surgery, neurological status, and systemic disease burden were analyzed to determine the impact of these factors on survival outcome. Results The authors identified 267 patients who met the study criteria. Five-year overall survival (OS) after spine tumor resection was 7.8%, with a median OS of 11.3 months (95% CI 9.5–13.0 months). Patients with Fuhrman Grade 4 RCC had a median OS of 6.1 months (95% CI 3.5–8.7 months), which was significantly lower than the 14.3 months (95% CI 9.1–19.4 months) observed in patients with Fuhrman Grade 3 or less RCC (p 〈 0.001). Patients with preoperative neurological deficits had a median survival of 5.9 months (95% CI 4.1–7.7 months), which was significantly lower than the 13.5 months (95% CI 10.4–16.6 months) observed in patients with a normal neurological examination (p 〈 0.001). Patients whose spine was the only site of metastasis had a median OS of 19 months (95% CI 9.8–28.2 months) after surgery, significantly longer than the 9.7 months (95% CI 8.1–11.3 months) observed in patients with additional extraspinal metastasis sites (p 〈 0.001). Patients with nonprogressing extraspinal metastasis (no metastasis, stable, or concurrent) had a median survival of 20.6 months (95% CI 15.1–26.1 months), compared with 5.6 months (95% CI 4.4–6.8 months) in patients with progressing metastasis (p 〈 0.001). Conclusions The authors identified several factors influencing survival after spine surgery for metastatic spinal RCC, including grade of the original nephrectomy specimen, activity of the systemic disease, and neurological status at the time of surgery. These clinical features may help to identify patients who may benefit from aggressive surgical intervention.
    Type of Medium: Online Resource
    ISSN: 1547-5654
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    Language: Unknown
    Publisher: Journal of Neurosurgery Publishing Group (JNSPG)
    Publication Date: 2014
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  • 4
    Online Resource
    Online Resource
    Journal of Neurosurgery Publishing Group (JNSPG) ; 2017
    In:  Journal of Neurosurgery: Spine Vol. 27, No. 6 ( 2017-12), p. 700-708
    In: Journal of Neurosurgery: Spine, Journal of Neurosurgery Publishing Group (JNSPG), Vol. 27, No. 6 ( 2017-12), p. 700-708
    Abstract: Treatment of epidural spinal cord compression (ESCC) caused by tumor includes surgical decompression and stabilization followed by postoperative radiation. In the case of severe axial loading impairment, anterior column reconstruction is indicated. The authors describe the use of interbody distraction to restore vertebral body height and correct kyphotic angulation prior to reconstruction with polymethylmethacrylate (PMMA), and report the long-term durability of such reconstruction. METHODS A single institution, prospective series of patients with ESCC undergoing single-stage decompression, anterior column reconstruction, and posterior instrumentation from 2013 to 2016 was retrospectively analyzed. Several demographic, perioperative, and radiographic measurements were collected. Descriptive statistics were compiled, in addition to postoperative changes in anterior height, posterior height, and kyphosis. Paired Student t-tests were performed for each variable. Overall survival was calculated using the techniques described by Kaplan and Meier. RESULTS Twenty-one patients underwent single-stage posterior decompression with interbody distraction and anterior column reconstruction using PMMA. The median age and Karnofsky Performance Scale score were 61 years and 70, respectively. Primary tumors included renal cell (n = 8), lung (n = 4), multiple myeloma (n = 2), prostate (n = 2), and other (n = 5). Eighteen patients underwent a single-level vertebral body reconstruction and 3 underwent multilevel transpedicular corpectomies. The median survival duration was 13.3 months. In the immediate postoperative setting, statistically significant improvement was noted in anterior body height (p = 0.0017, 95% confidence interval [CI] −4.15 to −1.11) and posterior body height (p = 0.0116, 95% CI −3.14 to −0.45) in all patients, and improved kyphosis was observed in those with oblique endplates (p = 0.0002, 95% CI 11.16–20.27). In the median follow-up duration of 13.9 months, the authors observed 3 cases of asymptomatic PMMA subsidence. One patient required reoperation in the form of extension of fusion. CONCLUSIONS In situ interbody distraction allows safe and durable reconstruction with PMMA, restores vertebral height, and corrects kyphotic deformities associated with severe pathological fractures caused by tumor. This is accomplished with minimal manipulation of the thecal sac and avoiding an extensive 360° surgical approach in patients who cannot tolerate extensive surgery.
    Type of Medium: Online Resource
    ISSN: 1547-5654
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    Language: Unknown
    Publisher: Journal of Neurosurgery Publishing Group (JNSPG)
    Publication Date: 2017
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  • 5
    In: Journal of Neurosurgery, Journal of Neurosurgery Publishing Group (JNSPG), Vol. 126, No. 1 ( 2017-01), p. 319-329
    Abstract: Malignant peripheral nerve sheath tumors (MPNSTs) are an aggressive group of soft tissue sarcomas that can arise sporadically, in the context of neurofibromatosis Type 1 (NF1) or at a site of prior irradiation. Large series profiling the features and outcomes of sporadic, NF1-associated, and radiation-associated MPNSTs are limited. The goal of this study was to elucidate differences between MPNST etiologies in a large single-institution retrospective study. METHODS Patients (n = 317) were identified through the tumor registry of The University of Texas MD Anderson Cancer Center. Clinicopathological features were retrospectively collected. Features were compared among MPNST subtypes for patients who had sufficient clinical history (n = 289), and clinicopathological features were used to identify adverse predictors of recurrence and survival outcomes. RESULTS Five-year local recurrence-free survival (LRFS), distant recurrence-free survival (DRFS), and disease-specific survival (DSS) estimates were 56.6%, 49.6%, and 53.6%, respectively, for the high-grade MPNST cohort. Five-year DSS was lower in NF1-associated and radiation-associated MPNST than in sporadic MPNST (52%, 47%, and 67%, respectively, p = 0.140). Patients with radiation-associated MPNST had worse 5-year LRFS than those with the sporadic and NF1-associated subtypes (RT-associated vs sporadic, p = 0.010; RT-associated vs NF1-associated, p = 0.232). Truncally located tumors, positive surgical margins, local recurrence, and metastasis were predictors of adverse DSS in multivariate analysis. CONCLUSIONS Radiation-associated MPNSTs are associated with poorer local recurrence-free and disease-specific survival than sporadic and NF1-associated tumors. NF1-associated MPNSTs may have worse survival outcomes owing to large tumor size, compromising truncal location, and lower rate of negative resection margins compared with sporadic tumors.
    Type of Medium: Online Resource
    ISSN: 0022-3085 , 1933-0693
    RVK:
    RVK:
    Language: Unknown
    Publisher: Journal of Neurosurgery Publishing Group (JNSPG)
    Publication Date: 2017
    detail.hit.zdb_id: 2026156-1
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  • 6
    Online Resource
    Online Resource
    Journal of Neurosurgery Publishing Group (JNSPG) ; 1999
    In:  Journal of Neurosurgery Vol. 91, No. 1 ( 1999-07), p. 93-99
    In: Journal of Neurosurgery, Journal of Neurosurgery Publishing Group (JNSPG), Vol. 91, No. 1 ( 1999-07), p. 93-99
    Abstract: Object. This study was undertaken to explore the effects of growth hormone (GH) and the GH-stimulated peptide insulin-like growth factor—1 (IGF-1) on the growth rate of meningiomas. Methods. Polymerase chain reaction and ribonuclease protection assays were used to demonstrate that GH receptor messenger RNA was present in all 14 meningioma specimens studied, regardless of tumor grade. Both wild type (GHRwt) and a previously described exon 3 deletion isoform (GHRd3) of the GH receptor were identified in individual tumor specimens. The importance of the GH receptor was assessed using a GH receptor antagonist (B2036). Blockade of the GH receptor with B2036 reduced serum-induced DNA synthesis, as measured by thymidine incorporation, by 8 to 33% (mean 20%) in primary meningioma cultures. Tumors that expressed the GHRwt and GHRd3 isoforms, or a combination of the two, were all responsive to antagonist treatment. The importance of IGF-1 in stimulating meningioma cell growth was also assessed. It was found that IGF-1 increased thymidine incorporation in primary meningioma cultures in a dose-dependent manner: 1 ng/ml, 5 ng/ml, and 10 ng/ml resulted in increases in thymidine incorporation of 21%, 43%, and 176%, respectively, over baseline values. Conclusions. In these studies the authors demonstrate that activation of the GH/IGF-1 axis significantly increases the growth rate of meningiomas. Blockade of the GH receptor on tumor cells inhibited tumor growth. If these findings are confirmed in animal studies, agents that downregulate the GH/IGF-1 axis might represent a potential adjuvant therapy in the management of patients with meningioma.
    Type of Medium: Online Resource
    ISSN: 0022-3085
    RVK:
    RVK:
    Language: Unknown
    Publisher: Journal of Neurosurgery Publishing Group (JNSPG)
    Publication Date: 1999
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  • 7
    In: Neurosurgical Focus, Journal of Neurosurgery Publishing Group (JNSPG), Vol. 6, No. 5 ( 1999-05), p. E5-
    Abstract: Traditionally, superior sulcus tumors of the lung that involve the chest wall and spinal column have been considered to be unresectable, and historically, patients harboring these tumors have been treated with local radiation therapy with, at best, modest results. The value of gross-total resection remains unclear in this patient population; however, with the recent advances in surgical technique and spinal instrumentation, procedures involving more radical removal of such tumors are now possible. At The University of Texas M. D. Anderson Cancer Center, the authors have developed a new technique for resecting superior sulcus tumors that invade the chest wall and spinal column. They present a technical description of this procedure and results in nine patients in whom stage IIIb superior sulcus tumors extensively invaded the vertebral column. These patients underwent gross-total tumor resection via a combined approach that included posterolateral thoracotomy, apical lobectomy, chest wall resection, laminectomy, vertebrectomy, anterior spinal column reconstruction with methylmethacrylate, and placement of spinal instrumentation. There were six men and three women, with a mean age of 55 years (range 36–72 years). Histological examination revealed squamous cell carcinoma (three patients), adenocarcinoma (four patients), and large cell carcinoma (two patients). The mean postoperative follow-up period was 16 months. All patients are currently ambulatory or remained ambulatory until they died. Pain related to tumor invasion improved in four patients and remained unchanged in five. In three patients instrumentation failed and required revision. There was one case of cerebrospinal leak that was treated with lumbar drainage and one case of wound breakdown that required revision. Two patients experienced local tumor recurrence, and one patient developed a second primary lung tumor. The authors conclude that in selected patients, combined radical resection of superior sulcus tumors of the lung that involve the chest wall and spinal column may represent an acceptable treatment modality that can offer a potential cure while preserving neurological function and providing pain control.
    Type of Medium: Online Resource
    ISSN: 1092-0684
    Language: Unknown
    Publisher: Journal of Neurosurgery Publishing Group (JNSPG)
    Publication Date: 1999
    detail.hit.zdb_id: 2026589-X
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  • 8
    Online Resource
    Online Resource
    Journal of Neurosurgery Publishing Group (JNSPG) ; 1998
    In:  Neurosurgical Focus Vol. 5, No. 2 ( 1998-08), p. E2-
    In: Neurosurgical Focus, Journal of Neurosurgery Publishing Group (JNSPG), Vol. 5, No. 2 ( 1998-08), p. E2-
    Abstract: Anterior approaches to the spine for the treatment of spinal tumors have gained acceptance; however, in most published reports, patients with primary, metastatic, or chest wall tumors involving cervical, thoracic, or lumbar regions of the spine are combined. The purpose of this study was to provide a clear perspective of results that can be expected in patients who undergo anterior vertebral body resection, reconstruction, and stabilization for spinal metastases that are limited to the thoracic region. Outcome is presented for 72 patients with metastatic spinal tumors who were treated by transthoracic vertebrectomy at The University of Texas M. D. Anderson Cancer Center. The predominant primary tumors included renal cancer in 19 patients, breast cancer in 10, melanoma or sarcoma in 10, and lung cancer in nine patients. The most common presenting symptoms were back pain, which occurred in 90% of patients, and lower-extremity weakness, which occurred in 64% of patients. All patients underwent transthoracic vertebrectomy, decompression, reconstruction with methylmethacrylate, and anterior fixation with locking plate and screw constructs. Supplemental posterior instrumentation was required in seven patients with disease involving the cervicothoracic or thoracolumbar junction, which was causing severe kyphosis. After surgery, pain improved in 60 of 65 patients. This improvement was found to be statistically significant (p 〈 0.001) based on visual analog scales and narcotic analgesic medication use. Thirty-five of the 46 patients who presented with neurological dysfunction improved significantly (p 〈 0.001) following the procedure. Thirty-three patients had weakness but could ambulate preoperatively. Seventeen of these 33 regained normal strength, 15 patients continued to have weakness, and one patient was neurologically worse postoperatively. Of the 13 preoperatively nonambulatory patients, 10 could walk after surgery and three were still unable to walk but showed improved motor function. Twenty-one patients had complications ranging from minor atelectasis to pulmonary embolism. The 30-day mortality rate was 3%. The 1-year survival rate for the entire study population was 62%. These results suggest that transthoracic vertebrectomy and spinal stabilization can improve the quality of life considerably in cancer patients with spinal metastasis by restoring or preserving ambulation and by controlling intractable spinal pain with acceptable rates of morbidity and mortality.
    Type of Medium: Online Resource
    ISSN: 1092-0684
    Language: Unknown
    Publisher: Journal of Neurosurgery Publishing Group (JNSPG)
    Publication Date: 1998
    detail.hit.zdb_id: 2026589-X
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  • 9
    In: DeckerMed Surgery, Decker Medicine, ( 2019-1-30)
    Abstract: Peripheral nerve sheath tumors represent a spectrum of entities ranging from benign tumors, such as schwannoma, to high-grade malignant tumors termed malignant peripheral nerve sheath tumors. In this chapter, we discuss current concepts and problematic areas in the diagnosis, treatment, and management of peripheral nerve sheath tumors. We discuss the major categories of nerve sheath tumors including schwannomas, neurofibromas, and malignant peripheral nerve sheath tumors. The different benign variants and their propensity for malignant transformation are highlighted. We suggest useful guidelines for the surgical management of these challenging cases. Finally, we discuss some of the advances in molecular biology that have provided new insights into the nature of the various peripheral nerve sheath tumors and suggest potential novel targeted therapeutic strategies for these tumors. This review contains 11 figures, 5 tables and 66 references. Key Words: malignant peripheral nerve sheath tumors, nerve sheath tumor, neurofibroma, neurofibromatosis, schwannoma, schwannomatosis, NF1, NF2
    Type of Medium: Online Resource
    Language: Unknown
    Publisher: Decker Medicine
    Publication Date: 2019
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  • 10
    Online Resource
    Online Resource
    Journal of Neurosurgery Publishing Group (JNSPG) ; 1998
    In:  Journal of Neurosurgery Vol. 89, No. 4 ( 1998-10), p. 599-609
    In: Journal of Neurosurgery, Journal of Neurosurgery Publishing Group (JNSPG), Vol. 89, No. 4 ( 1998-10), p. 599-609
    Abstract: Object. Anterior approaches to the spine for the treatment of spinal tumors have gained acceptance; however, in most published reports, patients with primary, metastatic, or chest wall tumors involving cervical, thoracic, or lumbar regions of the spine are combined. The purpose of this study was to provide a clear perspective of results that can be expected in patients who undergo anterior vertebral body resection, reconstruction, and stabilization for spinal metastases that are limited to the thoracic region. Methods. Outcome is presented for 72 patients with metastatic spinal tumors who were treated by transthoracic vertebrectomy at The University of Texas M. D. Anderson Cancer Center. The predominant primary tumors included renal cancer in 19 patients, breast cancer in 10, melanoma or sarcoma in 10, and lung cancer in nine patients. The most common presenting symptoms were back pain, which occurred in 90% of patients, and lower-extremity weakness, which occurred in 64% of patients. All patients underwent transthoracic vertebrectomy, decompression, reconstruction with methylmethacrylate, and anterior fixation with locking plate and screw constructs. Supplemental posterior instrumentation was required in seven patients with disease involving the cervicothoracic or thoracolumbar junction, which was causing severe kyphosis. After surgery, pain improved in 60 of 65 patients. This improvement was found to be statistically significant (p 〈 0.001) based on visual analog scales and narcotic analgesic medication use. Thirty-five of the 46 patients who presented with neurological dysfunction improved significantly (p 〈 0.001) following the procedure. Thirty-three patients had weakness but could ambulate preoperatively. Seventeen of these 33 regained normal strength, 15 patients continued to have weakness, and one patient was neurologically worse postoperatively. Of the 13 preoperatively nonambulatory patients, 10 could walk after surgery and three were still unable to walk but showed improved motor function. Twenty-one patients had complications ranging from minor atelectasis to pulmonary embolism. The 30-day mortality rate was 3%. The 1-year survival rate for the entire study population was 62%. Conclusions. These results suggest that transthoracic vertebrectomy and spinal stabilization can improve the quality of life considerably in cancer patients with spinal metastasis by restoring or preserving ambulation and by controlling intractable spinal pain with acceptable rates of morbidity and mortality.
    Type of Medium: Online Resource
    ISSN: 0022-3085
    RVK:
    RVK:
    Language: Unknown
    Publisher: Journal of Neurosurgery Publishing Group (JNSPG)
    Publication Date: 1998
    detail.hit.zdb_id: 2026156-1
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