scholarly journals Reclassification of 21 Presumptive Canine Peripheral Nerve Sheath Tumors (PNST) Using a Literature-Based Immunohistochemical Panel

2016 ◽  
Vol 66 (4) ◽  
pp. 455-465 ◽  
Author(s):  
Rubina Sirri ◽  
Silvia Sabattini ◽  
Giuliano Bettini ◽  
Luciana Mandrioli
Keyword(s):  
Peripheral Nerve ◽  
Soft Tissue Sarcomas ◽  
Therapeutic Implications ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
Archived Samples ◽  
Histological Appearance ◽  
S 100 ◽  
Poorly Differentiated

Abstract The aim of this study was to re-evaluate archived samples of canine soft tissue sarcomas (STSs) morphologically consistent with peripheral nerve sheath tumors (PNSTs). In each case, an immunohistochemical panel was applied, including α-SMA, calponin, desmin, S-100, GFAP, NSE and Olig2, in order to assess whether the phenotype was consistent with the tumor histological appearance. Additionally, the expression of EGFR, a marker with potential therapeutic implications in malignant PNSTs, was evaluated. Twenty-one tumors were included. Fourteen cases (66.7%) were positive for one or more muscular markers and were reclassified as perivascular tumors (PWTs). A positive labeling for S-100 was observed in one tumor (4.8%), thus classifi ed as PNST. The other 6 tumors were generically classified as poorly differentiated STSs. No unique histopathological feature was observed within the three groups. NSE and Olig2 labeling was aspecific and not useful for diagnostic purposes. GFAP was negative in all cases. Six cases (28.6%) were positive for EGFR, including the PNST. Even after the application of a wide immunohistochemical panel, distinguishing between PNSTs and PWTs remains a challenge. Finally, a subgroup of cases cannot be classified based on light microscopy alone.

scholarly journals Neoadjuvant Ifosfamide and Epirubicin in the Treatment of Malignant Peripheral Nerve Sheath Tumors

Sarcoma ◽  
2017 ◽  
Vol 2017 ◽  
pp. 1-6 ◽  
Author(s):  
Angela C. Hirbe ◽  
Pippa F. Cosper ◽  
Sonika Dahiya ◽  
Brian A. Van Tine
Keyword(s):  
Neoadjuvant Chemotherapy ◽  
Peripheral Nerve ◽  
Clinical Benefit ◽  
Metabolic Response ◽  
Neoadjuvant Treatment ◽  
Soft Tissue Sarcomas ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
Response To Chemotherapy

Background and Objectives. Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive soft tissue sarcomas with poor overall survival. Response to chemotherapy has been debated for these tumors. Methods. We performed a retrospective analysis of the patients at our institution with a biopsy-proven diagnosis of MPNST that underwent neoadjuvant chemotherapy prior to surgery. Results. We retrospectively identified five patients who received neoadjuvant chemotherapy with epirubicin and ifosfamide that demonstrated a 30% reduction in tumor growth and a 60% response rate by RECIST criteria. Additionally, a metabolic response was observed in all three patients who received serial PET scans during neoadjuvant treatment. The clinical benefit rate, which includes stable disease, was 100%. Conclusions. Our data suggest that MPNSTs do respond to epirubicin and ifosfamide based chemotherapy and prospective studies are warranted to further define the clinical benefit.

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 Malignant Peripheral Nerve Sheath Tumors in Africa: A Clinicopathological Study

ISRN Surgery ◽  
2011 ◽  
Vol 2011 ◽  
pp. 1-6 ◽  
Author(s):  
Peter M. Nthumba ◽  
Paul Irungu Juma
Keyword(s):  
Peripheral Nerve ◽  
Case Reports ◽  
Soft Tissue Sarcomas ◽  
Late Presentation ◽  
Sub Saharan Africa ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
Sub Saharan ◽  
Case Presentations

Introduction. Malignant peripheral nerve sheath tumors (MPNSTs) are rare, aggressive soft tissue sarcomas associated with poor prognosis, that most commonly affect patients aged 20 to 50 years, but have also been reported in children. There is little reported in literature on these tumors in Africa. Materials and Methods. A search of the hospital pathology database between 1992 and 2008 revealed 333 nerve sheath tumors, of which 31 were MPNSTs. Four representative case reports are presented. Discussion. MNPSTs have rarely been reported from sub-Saharan Africa; in this study, they constituted 9.3% of all nerve sheath tumors. The trunk (42%) and limbs (45%) were the most frequently affected anatomical sites. Late presentation of malignant lesions in this environment is exemplified by the four case presentations patients. Conclusions. This report confirms observations from studies on MPNSTs from other environments. Anatomically centrally located MPNSTs may have a higher incidence in sub-Saharan Africa than in the West. Because NF1-associated MPNSTs are difficult to diagnose clinically, and because surgery is the only mode of therapy that offers a complete cure, a lifetime follow-up is important, as this would enable diagnosis of early lesions amenable to surgical extirpation.

scholarly journals Malignant Peripheral Nerve Sheath Tumors in Children with Neurofibromatosis Type 1

2014 ◽  
Vol 2014 ◽  
pp. 1-6 ◽  
Author(s):  
Apostolos Pourtsidis ◽  
Dimitrios Doganis ◽  
Margarita Baka ◽  
Despina Bouhoutsou ◽  
Maria Varvoutsi ◽  
...  
Keyword(s):  
Radiation Therapy ◽  
Peripheral Nerve ◽  
Neurofibromatosis Type 1 ◽  
Soft Tissue Sarcomas ◽  
Neurofibromatosis Type ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
Increased Risk

Purpose. Malignant peripheral nerve sheath tumors (MPNSTs) are rare in children and account for approximately 5–10% of all soft tissue sarcomas in adults. MPNSTs may occur independently but individuals with neurofibromatosis type 1 (NF1) have a significantly increased risk. Our aim is to present patients with MPNST treated in our department.Cases and Results. In this report we present 4 cases of MPNSTs (3 females: 13, 12, and 13 years old and 1 male: 10 years old) arising in patients with NF1. All of them presented with an enlarging mass and pain at diagnosis. Tumor was located in the buttock, the spinal cord, the trunk, and the left leg proximal to the heel. Wide excision of the tumor and radiotherapy were applied to all and adjuvant chemotherapy was given to three of them after the disease was progressed. All four died 32, 18, 10, and 22 months after diagnosis with progressive disease locally and pulmonary metastases in two of them.Conclusions. In conclusion, MPNSTs arising in patients with NF1 are high grade sarcomas with short survival. Individuals with NF1 should be followed closely in order to identify early the development of MPNSTs. Aggressive surgery and complete excision significantly improves disease-free survival. The usefulness of radiation therapy in MPNSTs is not determined although all patients will receive radiation therapy at some stage of the disease. The role of chemotherapy is unclear.

scholarly journals Treatment of malignant peripheral nerve sheath tumors in pediatric NF1 disease

2020 ◽  
Vol 36 (10) ◽  
pp. 2453-2462
Author(s):  
Enrico Martin ◽  
Uta E. Flucke ◽  
J. Henk Coert ◽  
Max M. van Noesel
Keyword(s):  
Peripheral Nerve ◽  
Radiation Effects ◽  
Soft Tissue Sarcomas ◽  
Standard Of Care ◽  
Response Rates ◽  
Neurofibromatosis Type ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath

Abstract Background Malignant peripheral nerve sheath tumors (MPNSTs) are rare yet highly aggressive soft tissue sarcomas. Children with neurofibromatosis type 1 (NF1) have a 10% lifetime risk for development of MPNST. Prognosis remains poor and survival seems worse for NF1 patients. Methods This narrative review highlights current practices and pitfalls in the management of MPNST in pediatric NF1 patients. Results Preoperative diagnostics can be challenging, but PET scans have shown to be useful tools. More recently, functional MRI holds promise as well. Surgery remains the mainstay treatment for these patients, but careful planning is needed to minimize postoperative morbidity. Functional reconstructions can play a role in improving functional status. Radiotherapy can be administered to enhance local control in selected cases, but care should be taken to minimize radiation effects as well as reduce the risk of secondary malignancies. The exact role of chemotherapy has yet to be determined. Reports on the efficacy of chemotherapy vary as some report lower effects in NF1 populations. Promisingly, survival seems to ameliorate in the last few decades and response rates of chemotherapy may increase in NF1 populations when administering it as part of standard of care. However, in metastasized disease, response rates remain poor. New systemic therapies are therefore desperately warranted and multiple trials are currently investigating the role of drugs. Targeted drugs are nevertheless not yet included in first line treatment. Conclusion Both research and clinical efforts benefit from multidisciplinary approaches with international collaborations in this rare malignancy.

scholarly journals Multiple Peripheral Nerve Sheath Tumors in the Small Intestine of a Horse

1996 ◽  
Vol 33 (6) ◽  
pp. 727-730 ◽  
Author(s):  
N. Kirchhof ◽  
W. Scheidemann ◽  
W. Baumgärtner
Keyword(s):  
Small Intestine ◽  
Peripheral Nerve ◽  
Ganglion Cells ◽  
Glial Fibrillary Acid Protein ◽  
Distal Small Intestine ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
Acid Protein ◽  
S 100

Multiple neurofibromas, schwannomas, and hyperplastic enteric plexuses were observed in the distal jejunum and ileum of a 6-year-old pinto gelding. The animal was presented because of an acute episode of colic. Three meters of distal small intestine, partially incarcerated in the epiploic foramen, were surgically removed. Numerous tumor nodules up to 10 mm in diameter were found adjacent to a Meckel's diverticulum, predominantly located in the subserosa of a hypertrophic segment. Histologically, tumors were well demarcated and composed of interlacing fascicles formed by spindloid cells. Adjacent enteric plexuses were hyperplastic. Immunohistochemically, all tumors were positive for vimentin and S-100. Desmin immunoreactivity was only observed in larger tumors (>500 μm). Glial fibrillary acid protein was demonstrated nearly exclusively in smaller ones. Immunostaining for neurofilament was restricted to entrapped ganglion cells. Based on conventional light microscopic examination and immunohistochemical evaluation, the lesion was diagnosed as multiple benign peripheral nerve sheath tumors in the small intestine.

Malignant Peripheral Nerve Sheath Tumors

2018 ◽  
Author(s):  
Marie-Noëlle Hébert-Blouin
Keyword(s):  
Decision Making ◽  
Soft Tissue ◽  
Peripheral Nerve ◽  
Soft Tissue Sarcomas ◽  
Nerve Sheath Tumor ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Adjuvant Therapies ◽  
Nerve Sheath ◽  
Schwann Cell Differentiation

Malignant peripheral nerve sheath tumors (PNSTs) are soft tissue sarcomas arising from a peripheral nerve or a pre-existing benign nerve sheath tumor or are sarcomas with features of Schwann-cell differentiation. Differentiating between benign and malignant PNSTs can be challenging. The chapter begins with a case example and then discusses assessment, investigations (including imaging), and diagnosis of malignant PNSTs, as well as the steps involved in decision-making about management of a malignant PNST. The surgical principles and goals for resection of a malignant PNST, the adjuvant therapies used in treatment, and the complications and outcomes of treatment are presented.

Phase 1 combination therapy with pexidartinib (PEX) and sirolimus (S) to target tumor-associated macrophages in pigmented villonodular synovitis, malignant peripheral nerve sheath tumors, and other soft tissue sarcomas.

2019 ◽  
Vol 37 (15_suppl) ◽  
pp. 11055-11055 ◽  
Author(s):  
Gulam Abbas Manji ◽  
Brian Andrew Van Tine ◽  
Shing Mirn Lee ◽  
Alexander Raufi ◽  
Parag Patwardhan ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Dose Reduction ◽  
Soft Tissue Sarcomas ◽  
Pigmented Villonodular Synovitis ◽  
Villonodular Synovitis ◽  
Phase 2 ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
Pigmented Villonodular

11055 Background: No effective therapy exists for unresectable malignant peripheral nerve sheath tumors (MPNSTs). We previously reported that the combination of PEX and the mTOR inhibitor S synergistically inhibited MPNST growth (CCR 20: 3146, 2014) by depleting M2 TAMs and by inhibiting receptor tyrosine kinases (RTKs), including c-KIT, PDGFR, CSF1R. We characterized the safety, tolerability, recommended phase 2 dose (RP2D) of PEX plus S in all sarcoma sub-types. Methods: Patients (pts) received PEX plus S orally in 28 days cycle as per Table. The RP2D was determined using the time-to-event continual reassessment method (TITE-CRM) in advanced sarcoma who have progressed on standard therapy. DLT was defined as any need for a dose reduction. Results: 24 pts were accrued (Acr) of which 18 were evaluable (MPNST – 6, pigmented villonodular synovitis (PVNS) – 3, leiomyosarcoma – 5, and other – 9). The mean age was 46y, 56% were male, and 67% had greater than 2 prior therapies. Most common ( > 20%) grade 2 or higher TEAEs were anemia (33%), WBC count decrease (28%), fatigue, neutropenia, and lymphopenia (22% each). There were 5 dose limiting toxicities (DLT): 2 for elevated LFTs both of which resolved with dose reduction, 2 for supra-therapeutic S trough levels, and 1 for grade 5 dehydration at dose level (DL) 3. Four subjects experienced a partial response (PR; -44% to -77% by RECIST, 18 – 61 wks on therapy). Seven subjects experienced stable disease (SD; +19.7% to -20.7% by RECIST; 9.4 – 30 wks on therapy). Five subjects progressed on therapy and two subjects experienced early DLTs and did not undergo tumor assessment. The RP2D is DL 3 (S 2mg/PEX 1000mg) with an estimated probability of DLT of 26.7% as determined by TITE-CRM. This recommendation is based on a target DLT rate of 25%. TAMs and immune subtypes from available tissue specimens and historical controls will be presented. Conclusions: 1000mg of PEX in combination with 2mg of S daily has an acceptable safety profile. Objective responses and durable SD was observed in PNVS and MPNST patients justifying proceeding with a multi-center single arm phase 2 study in advanced MPNST. Clinical trial information: NCT02584647. [Table: see text]

Point Mutation of neu Oncogene in Animal Peripheral Nerve Sheath Tumors

2001 ◽  
Vol 38 (6) ◽  
pp. 679-688 ◽  
Author(s):  
G. Stoica ◽  
S. I. Tasca ◽  
H.-T. Kim
Keyword(s):  
Peripheral Nerve ◽  
Point Mutation ◽  
Animal Species ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
S 100 ◽  
Polymerase Chain ◽  
Neu Oncogene ◽  
Antoni Type

Thirty-four peripheral nerve sheath tumors of four domesticated animal species were characterized and assayed for point mutation of the neu oncogene. Based on their morphoimmunophenotype, 32 tumors were classified as schwannomas. Schwannoma morphology was characterized by the presence of Antoni type A and B pattern and immunoreactivity for S-100 protein and vimentin. Two anaplastic and metastatic tumors originating from spinal cord root, immunonegative for S-100 protein and positive for vimentin, were classified as malignant peripheral nerve sheath tumors (MPNSTs). Four malignant schwannomas and two MPNSTs expressed a point mutation of the neu oncogene by the polymerase chain reaction-restriction fragment length polymorphism method. The finding of neu oncogene mutation could be a useful diagnostic genetic marker in the malignant form of peripheral nerve sheath tumors in animals.

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