scholarly journals Management of peripheral nerve sheath tumors: 17 years of experience at Toronto Western Hospital

2018 ◽  
Vol 128 (4) ◽  
pp. 1226-1234 ◽  
Author(s):  
Daipayan Guha ◽  
Benjamin Davidson ◽  
Mustafa Nadi ◽  
Naif M. Alotaibi ◽  
Michael G. Fehlings ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Clinical Presentation ◽  
Extent Of Resection ◽  
Tumor Location ◽  
Benign Lesions ◽  
Toronto Western Hospital ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Western Hospital ◽  
Nerve Sheath

OBJECTIVEA surgical series of 201 benign and malignant peripheral nerve sheath tumors (PNSTs) was assessed to characterize the anatomical and clinical presentation of tumors and identify predictors of neurological outcome, recurrence, and extent of resection.METHODSAll surgically treated PNSTs from the Division of Neurosurgery at Toronto Western Hospital from 1993 to 2010 were reviewed retrospectively. Data were collected on patient demographics, clinical presentation, surgical technique, extent of resection, postoperative neurological outcomes, and recurrence.RESULTSOne hundred seventy-five patients with 201 tumors had adequate follow-up for analysis. There were 182 benign and 19 malignant PNSTs. Of the benign lesions, 133 were schwannomas, 21 of which were associated with a diagnosis of schwannomatosis. There were 49 neurofibromas, and 26 were associated with neurofibromatosis Type 1 (NF1). Patients presenting with schwannomas were significantly older than those with neurofibromas. Schwannomas were more readily resected than neurofibromas, with the extent of resection of the former influenced by tumor location. Patients with benign PNSTs typically presented with a painful mass and less frequently with motor deficits. The likelihood of worsened postoperative motor function was decreased in patients with fully resected tumors or preoperative deficits. Recurrence of schwannomas and neurofibromas were seen more frequently in patients diagnosed with NF3 and NF1, respectively. Subtotal resection was associated with the increased recurrence of all benign lesions.CONCLUSIONSOutcomes following resection of benign PNSTs depend on tumor histopathology, tumor location, and genetic predisposition syndrome. Gross-total resection should be attempted for benign lesions where possible. The management of malignant PNSTs remains challenging, requiring a multimodal approach.

scholarly journals Retrospective canine skin peripheral nerve sheath tumors data with emphasis on histologic, immunohistochemical and prognostic factors

2015 ◽  
Vol 35 (12) ◽  
pp. 965-974 ◽  
Author(s):  
Gisele S. Boos ◽  
Daniele M. Bassuino ◽  
Fabiana Wurster ◽  
Neusa B. Castro ◽  
Tatiane T.N. Watanabe ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Protein S ◽  
Skin Neoplasms ◽  
Tumor Location ◽  
Benign Tumors ◽  
Nerve Sheath Tumor ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
S 100

Abstract: In this retrospective study was determined the frequency of canine skin peripheral nerve sheath tumors (PNST) in cases diagnosed by the Setor de Patologia Veterinária of the Universidade Federal do Rio Grande do Sul (SPV-UFRGS), Brazil, between the years 2000 and 2012. The canine profiles, as well as histological, immunohistochemical and prognostic aspects of the tumors were based on 70 samples, comprising 40 females, 29 males and one unspecified sample. Between 2000 and 2012, 2,984 skin tumors of dogs were diagnosed in the SPV-UFRGS, totaling 2.34% of skin neoplasms in dogs. Animals that comprised the largest amount of samples (43%) were those with no breed (SRD), followed by German Shepherds (10%). Females were more affected than males (40/70 - 57% and 29/70 - 41% respectively). Skin PNST of this research showed predominant localization on the limbs (40% in the forelimbs and 29% in the hindlimbs); affecting adult dogs, mostly aged between 8 and 11 years (54%). The samples were routinely processed for hematoxylin and eosin, and were also evaluated by toluidine blue and Masson's trichrome staining, and immunohistochemistry (IHC) anti-vimentin, -S-100, -GFAP, -actin, von Willebrand factor and neurofilament. Anisocytosis and anisokaryosis, mitotic index, intratumoral necrosis, invasion of adjacent tissues, tumor location, local recurrence and metastasis were related to the diagnosis of benign (49/70) or malignant tumor (21/70). The Antoni A histological pattern was observed more frequently in benign tumors. The immunohistochemistry helped to diagnose PNST, and anti-vimentin and anti-protein S-100 showed the highest rates of immunostaining. Throughout statistical analysis of animals with tumor recurrence, it was found that the chance of an animal with a malignant peripheral nerve sheath tumor to develop recurrence is 4.61 times higher than in an animal that had a benign tumor.

scholarly journals Localized, Plexiform, Diffuse, and Other Variants of Neurofibroma in 12 Dogs, 2 Horses, and a Chicken

2009 ◽  
Vol 46 (5) ◽  
pp. 904-915 ◽  
Author(s):  
S. Schöniger ◽  
B. A. Summers
Keyword(s):  
Peripheral Nerve ◽  
Large Intestine ◽  
Tumor Location ◽  
Growth Patterns ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
Microscopic Features ◽  
Canine Tumors

In humans, neurofibroma and schwannoma are distinct entities within the group of benign peripheral nerve sheath tumors. In the veterinary literature, these tumors are often classified together simply as benign peripheral nerve sheath tumors, and diagnostic criteria for their subclassification are not well established. We describe peripheral nerve sheath tumors with microscopic, immunohistologic, and ultrastructural features similar to those in subtypes of human neurofibroma in 12 dogs, 2 horses, and 1 chicken. Dogs were of different breeds and were aged 2 months to 15 years. The canine tumors were located in the skin, peripheral nerve, tongue, and large intestine. The 2 horses were 11 and 12 years old. The equine tumors were located in the subcutis of the neck and axilla. The chicken was a mature white Leghorn chicken with an ocular neoplasm. Neurofibromas of this study had localized, plexiform, diffuse and combined plexiform and diffuse growth patterns, and microscopic features similar to those in classic, collagenous, cellular, myxoid, and pigmented neurofibromas of humans. One diffuse neurofibroma contained areas of schwannian differentiation (hybrid neurofibroma-schwannoma). Two plexiform neurofibromas occurred together with diffuse ganglioneuromatosis in the large intestine of young dogs, as has also been reported in humans. This investigation shows the existence of identical subtypes of neurofibroma in animals and humans and identifies similarities in tumor location and patient age between animals and humans. This report will allow a more discriminating classification of benign peripheral nerve sheath tumors and probably has a bearing on epidemiology, pathogenesis and prognosis.

scholarly journals Association of tumor location, extent of resection, and neurofibromatosis status with clinical outcomes for 221 spinal nerve sheath tumors

2015 ◽  
Vol 39 (2) ◽  
pp. E5 ◽  
Author(s):  
Michael Safaee ◽  
Andrew T. Parsa ◽  
Nicholas M. Barbaro ◽  
Dean Chou ◽  
Praveen V. Mummaneni ◽  
...  
Keyword(s):  
Clinical Outcomes ◽  
Extent Of Resection ◽  
Tumor Location ◽  
Spinal Nerve ◽  
Tumor Type ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
The Mean

OBJECT Intradural extramedullary spine tumors represent two-thirds of all primary spine neoplasms. Approximately half of these are peripheral nerve sheath tumors, mainly neurofibromas and schwannomas. Given the rarity of this disease and, thus, the limited analyses of clinical outcomes, the authors examined the association of tumor location, extent of resection, and neurofibromatosis (NF) status with clinical outcomes. METHODS Patients were identified through a search of the University of California, San Francisco, neuropathology database and a separate review of current procedural terminology codes. Data recorded included patient age, patient sex, clinical presentation, presence of NF, tumor type, tumor location, extent of resection (gross-total resection [GTR] or subtotal resection [STR]), and clinical follow-up. RESULTS Of 221 tumors in 199 patients (mean age 45 years), 53 were neurofibromas, 163 were schwannomas, and 5 were malignant peripheral nerve sheath tumors. The most common presenting symptom was spinal pain (76%), followed by weakness (36%) and sensory abnormalities (34%). Mean symptom duration was 16 months. In terms of spinal location, neurofibromas were more common in the cervical spine (74% vs 27%, p < 0.001), and schwannomas were more common in the thoracic and lumbosacral spine (73% vs 26%, p < 0.001). Rates of GTR were lower for neurofibromas than schwannomas (51% vs 83%, p < 0.001), regardless of location. Rates of GTR were lower for cervical (54%) than thoracic (90%) and lumbosacral (86%) lesions (p < 0.001). NF was associated with lower rates of GTR among all tumors (43% vs 86%, p < 0.001). The mean follow-up time was 32 months. Recurrence/progression was more common for neurofibromas than schwannomas (17% vs 7%, p = 0.03), although the mean time to recurrence/progression did not differ according to tumor type (45 vs 53 months, p = 0.63). As expected, GTR was associated with lower recurrence rates (4% vs 22%, p < 0.001). According to multivariate analysis, cervical location (OR 0.239, 95% CI 0.110–0.520) and presence of NF (OR 0.166, 95% CI 0.054–0.507) were associated with lower rates of GTR. In a separate model, only GTR (OR 0.141, 95% CI 0.046–0.429) was associated with tumor recurrence. CONCLUSIONS Resection is an effective treatment for spinal nerve sheath tumors. Neurofibromas were found more commonly in the cervical spine than in other regions of the spine and were associated with higher rates of recurrence and lower rates of GTR than other tumor types, particularly in patients with NF Types 1 or 2. According to multivariate analysis, both cervical location and presence of NF were associated with lower rates of GTR. According to a second multivariate model, the only variable associated with tumor recurrence was extent of resection. Maximal safe resection remains ideal for these lesions; however, patients with cervical tumors or NF should be counseled about their increased risk for recurrence.

scholarly journals Biology and Management of Undifferentiated Pleomorphic Sarcoma, Myxofibrosarcoma, and Malignant Peripheral Nerve Sheath Tumors: State of the Art and Perspectives

2018 ◽  
Vol 36 (2) ◽  
pp. 160-167 ◽  
Author(s):  
Brigitte C. Widemann ◽  
Antoine Italiano
Keyword(s):  
Peripheral Nerve ◽  
Clinical Presentation ◽  
State Of The Art ◽  
Current Knowledge ◽  
Future Research ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Pleomorphic Sarcoma ◽  
Nerve Sheath ◽  
Aggressive Clinical Behavior

Undifferentiated pleomorphic sarcomas, myxofibrosarcomas, and malignant peripheral nerve sheath tumors are characterized by complex genomic characteristics and aggressive clinical behavior. Recent advances in the understanding of the pathogenesis of these tumors may allow for the development of more-effective innovative therapeutic strategies, including immunotherapies. This review describes the current knowledge of the epidemiology, clinical presentation, treatment, and pathogenesis of these tumors and highlights ongoing and future research.

The Surgical Management of Symptomatic Benign Peripheral Nerve Sheath Tumors of the Neck and Extremities: An Experience of 442 Cases

Neurosurgery ◽  
2017 ◽  
Vol 81 (4) ◽  
pp. 568-580 ◽  
Author(s):  
Ketan I. Desai
Keyword(s):  
Peripheral Nerve ◽  
Surgical Excision ◽  
Extent Of Resection ◽  
Motor Deficit ◽  
Neurogenic Pain ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
Upper Cervical ◽  
The Mean

Abstract BACKGROUND The occurrence of benign peripheral nerve sheath tumors (PNSTs) is not uncommon. The surgical excision of symptomatic benign PNSTs along with preservation of the affected nerve and its function is an ideal treatment option. OBJECTIVE To analyze the outcome with respect to morbidity, extent of resection, and recurrence, and to review and compare our results with those reported in literature. METHODS A retrospective review of clinical and radiological findings of 442 patients with benign PNSTs involving the neck and extremities treated surgically from 2000 to 2014 was performed. RESULTS In our series, benign PNSTs involved the extremities in 290 (65.6%) patients and the brachial plexus in 146 (33%) patients, and 6 (1.4%) patients had tumors of the extracranial portion of the vagus and hypoglossal nerves in the neck. The mean age of patients was 38 yr. The presenting features were painful mass and paresthesia. Preoperative motor weakness in the extremity was noted in 15.6% of patients. The common nerves involved by the tumors were the ulnar nerve (15.8%), sciatic nerve (12.7%), and upper cervical roots (11.5%). The excision was total in 81.2%, gross total (&gt;90%) in 17.9%, and subtotal (&gt;50%) in 0.9% patients. In 17.6% of patients, there was severe postoperative neurogenic pain. In 28 (6.3%) patients, a new motor deficit was noted following surgery. Recurrence was seen in 2 patients in our series. The mean follow-up was 30.2 mo. CONCLUSION Benign PNSTs have excellent clinical outcome, and the goal for surgical treatment is total to gross total excision of the tumor with neural preservation.

scholarly journals P.098 Evaluation and surgical management of pelvic peripheral nerve sheath tumors: the University of Toronto experience and review of literature

2017 ◽  
Vol 44 (S2) ◽  
pp. S38-S38
Author(s):  
S Wang ◽  
Z Merali ◽  
F Pirouzmand
Keyword(s):  
Peripheral Nerve ◽  
English Language ◽  
Case Series ◽  
Tumor Location ◽  
Presenting Symptoms ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
Imaging Characteristics ◽  
Surgical Technical

Background: Pelvic peripheral nerve sheath tumors (PNST), which includes neurofibroma, schwannoma, and MPNST, are rare tumors located in the retroperitoneum. Methods: The case records of a prospectively maintained database at Sunnybrook Health Sciences Center (SHSC) were reviewed to identify patients with pelvic PNST, managed between 2006 - 2016. Medical records were retrospectively reviewed for patient demographics, presentation, tumor location, symptoms, imaging characteristics, management, and outcome. The surgical technical caveats were described. An English language literature review was performed to describe previously published experiences. Results: The series consisted of 7 patients, ranging from 22 - 74 years of age at presentation. These lesions tend to be large at the time of diagnosis, and presenting symptoms include abdominal, flank, or back pain, as well as leg edema or hydronephrosis from local compression. Most patients in this cohort were managed surgically with midline abdominal transperitoneal exposures. Lastly, 5 tumors were benign schwannomas managed with gross total resection or debulking, while 2 patients had MPNSTs managed with biopsy followed by adjuvant chemoradiation therapy. Conclusions: In this case series, we describe the characteristics, evaluation, and management of 7 patients with pelvic PNST at a major healthcare institution in Toronto, Canada, highlighting the technical aspects of managing this rare and challenging entity.

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