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Updates on the diagnosis and treatment of intracranial nerve malignant peripheral nerve sheath tumors  [cached]
L'Heureux-Lebeau B,Saliba I
OncoTargets and Therapy , 2013,
Abstract: Bénédicte L'Heureux-Lebeau,1 Issam Saliba2 1University of Montreal, 2Department of Otolaryngology Head and Neck Surgery, Montreal University Hospital Center (CHUM), University of Montreal, Montreal, Quebec, Canada Background: Malignant peripheral nerve sheath tumors (MPNSTs) are rare entities and MPNSTs of intracranial nerves are even more sporadic. MPNSTs present diagnosis and treatment challenges since there are no defined diagnosis criteria and no established therapeutic strategies. Methods: We reviewed literature for MPNST-related articles. We found 45 relevant studies in which 60 cases were described. Results: We identified 60 cases of intracranial nerve MPNSTs. The age ranged from 3 to 75 years old. Male to female ratio was 1.5:1. The most involved cranial nerves (CNs) were CN VIII (60%), CN V (27%), and CN VII (10%). Most of the MPNSTs reported (47%) arose sporadically, 40% arose from a schwannoma, 8% arose from a neurofibroma, and 6% arose from an unspecified nerve tumor. Twenty patients had a history of radiation exposure, four patients had neurofibromatosis type 1 (NF1), four patients had neurofibromatosis type 2 (NF2), and NF2 was suspected in two other patients. Twenty-two patients were treated with radiotherapy and presented a higher survival rate. Seventy-two percent of patients died of their disease while 28% of patients survived. One-year survival rate was 33%. Forty-five percent of tumors recurred and 19% of patients had metastases. Conclusion: MPNSTs involving CNs are very rare. Diagnosis is made in regards to the histological and pathological findings. Imaging may help orient the diagnosis. A preexisting knowledge of the clinical situation is more likely to lead to a correct diagnosis. The mainstay of treatment is radical surgical resection with adjuvant radiotherapy. Since these tumors are associated with a poor prognosis, a close follow-up is mandatory. Keywords: malignant peripheral nerve sheath tumor, MPNST, neurofibroma, malignant schwannoma
Malignant Peripheral Nerve Sheath Tumors of the Scalp: Case Report and Review of Literature  [PDF]
Touria Bouhafa, Abderahmann Elmazghi, Hayat Baissel, Hind El Fatmi, Afaf Amarti, Khalid Hassouni
International Journal of Clinical Medicine (IJCM) , 2014, DOI: 10.4236/ijcm.2014.515123

Malignant peripheral nerve sheath tumors of the scalp are rare lesions of the nervous system.  Only 16 cases have been reported to date. In this report, we present a case of a malignant peripheral nerve sheath tumor (MPNST) of the scalp and retrospectively analyze the clinical features, imaging findings, pathological features, and prognoses of these tumors.

The 4q12 Amplicon in Malignant Peripheral Nerve Sheath Tumors: Consequences on Gene Expression and Implications for Sunitinib Treatment  [PDF]
Jan Zietsch,Nicolas Ziegenhagen,Frank L. Heppner,David Reuss,Andreas von Deimling,Nikola Holtkamp
PLOS ONE , 2012, DOI: 10.1371/journal.pone.0011858
Abstract: Malignant peripheral nerve sheath tumors (MPNST) are highly aggressive tumors which originate from Schwann cells and develop in about 10% of neurofibromatosis type 1 (NF1) patients. The five year survival rate is poor and more effective therapies are needed. Sunitinib is a drug targeting receptor tyrosine kinases (RTK) like PDGFRα, c-Kit and VEGFR-2. These genes are structurally related and cluster on chromosomal segment 4q12.
Malignant Peripheral Nerve Sheath Tumors: Differentiation Patterns and Immunohistochemical Features - A Mini-Review and Our New Findings  [cached]
Aitao Guo, Aijun Liu, Lixin Wei, Xin Song
Journal of Cancer , 2012,
Abstract: Malignant peripheral nerve sheath tumors (MPNST) represent a group of highly heterogeneous human malignancies often with multiple histological origins, divergent differentiation patterns, and diverse immunohistochemical presentations. The differential diagnosis of MPNST from other spindle cell neoplasms poses great challenges for pathologists. This report provides a mini-review of these unique features associated with MPNST and also presents the first cases of MPNST with six differentiation patterns.
Molecular heterogeneity in malignant peripheral nerve sheath tumors associated with neurofibromatosis type 1
Thomas Laura,Mautner Victor-Felix,Cooper David N,Upadhyaya Meena
Human Genomics , 2012, DOI: 10.1186/1479-7364-6-18
Abstract: Neurofibromatosis type-1 (NF1), resulting from NF1 gene loss of function, is characterized by an increased risk of developing benign and malignant peripheral nerve sheath tumors (MPNSTs). Whereas the cellular heterogeneity of NF1-associated tumors has been well studied, the molecular heterogeneity of MPNSTs is still poorly understood. Mutational heterogeneity within these malignant tumors greatly complicates the study of the underlying mechanisms of tumorigenesis. We have explored this molecular heterogeneity by performing loss of heterozygosity (LOH) analysis of the NF1, TP53, RB1, PTEN, and CDKN2A genes on sections of 10 MPNSTs derived from 10 unrelated NF1 patients. LOH data for the TP53 gene was found to correlate with the results of p53 immunohistochemical analysis in the same tumor sections. Further, approximately 70% of MPNSTs were found to display intra-tumoral molecular heterogeneity as evidenced by differences in the level of LOH between different sections of the same tumor samples. This study constitutes the first systematic analysis of molecular heterogeneity within MPNSTs derived from NF1 patients. Appreciation of the existence of molecular heterogeneity in NF1-associated tumors is important not only for optimizing somatic mutation detection, but also for understanding the mechanisms of NF1 tumorigenesis, a prerequisite for the development of specifically targeted cancer therapeutics.
Survivin Expression and Prognostic Significance in Pediatric Malignant Peripheral Nerve Sheath Tumors (MPNST)  [PDF]
Rita Alaggio, Riccardo Turrini, Daniela Boldrin, Anna Merlo, Claudio Gambini, Andrea Ferrari, Patrizia Dall'Igna, Cheryl M. Coffin, Annalisa Martines, Laura Bonaldi, Gian Luca De Salvo, Paola Zanovello, Antonio Rosato
PLOS ONE , 2013, DOI: 10.1371/journal.pone.0080456
Abstract: Malignant peripheral nerve sheath tumors (MPNST) are very aggressive malignancies comprising approximately 5–10% of all soft tissue sarcomas. In this study, we focused on pediatric MPNST arising in the first 2 decades of life, as they represent one the most frequent non-rhabdomyosarcomatous soft tissue sarcomas in children. In MPNST, several genetic alterations affect the chromosomal region 17q encompassing the BIRC5/SURVIVIN gene. As cancer-specific expression of survivin has been found to be an effective marker for cancer detection and outcome prediction, we analyzed survivin expression in 35 tumor samples derived from young patients affected by sporadic and neurofibromatosis type 1-associated MPNST. Survivin mRNA and protein expression were assessed by Real-Time PCR and immunohistochemical staining, respectively, while gene amplification was analyzed by FISH. Data were correlated with the clinicopathological characteristics of patients. Survivin mRNA was overexpressed in pediatric MPNST and associated to a copy number gain of BIRC5; furthermore, increased levels of transcripts correlated with a higher FNCLCC tumor grade (grade 1 and 2 vs. 3, p = 0.0067), and with a lower survival probability (Log-rank test, p = 0.0038). Overall, these data support the concept that survivin can be regarded as a useful prognostic marker for pediatric MPNST and a promising target for therapeutic interventions.
DNA copy number changes in high-grade malignant peripheral nerve sheath tumors by array CGH
Stine H Kresse, Magne Sk?rn, Hege O Ohnstad, Heidi M Naml?s, Bodil Bjerkehagen, Ola Myklebost, Leonardo A Meza-Zepeda
Molecular Cancer , 2008, DOI: 10.1186/1476-4598-7-48
Abstract: Considerable more gains than losses were observed, and the most frequent minimal recurrent regions of gain included 1q24.1-q24.2, 1q24.3-q25.1, 8p23.1-p12, 9q34.11-q34.13 and 17q23.2-q25.3, all gained in five of seven samples. The 17q23.2-q25.3 region was gained in all five patients with poor outcome and not in the two patients with disease-free survival. cDNA microarray analysis and quantitative real-time reverse transcription PCR were used to investigate expression of genes located within these regions. The gene lysyl oxidase-like 2 (LOXL2) was identified as a candidate target for the 8p23.1-p12 gain. Within 17q, the genes topoisomerase II-α (TOP2A), ets variant gene 4 (E1A enhancer binding protein, E1AF) (ETV4) and baculoviral IAP repeat-containing 5 (survivin) (BIRC5) showed increased expression in all samples compared to two benign tumors. Increased expression of these genes has previously been associated with poor survival in other malignancies, and for TOP2A, in MPNSTs as well. In addition, we have analyzed the expression of five micro RNAs located within the 17q23.2-q25.3 region, but none of them showed high expression levels compared to the benign tumors.Our study shows the potential of using DNA copy number changes obtained by array CGH to predict the prognosis of MPNST patients. Although no clear correlations between the expression level and patient outcome were observed, the genes TOP2A, ETV4 and BIRC5 are interesting candidate targets for the 17q gain associated with poor survival.Malignant peripheral nerve sheath tumors (MPNSTs) are rare tumors that arise sporadically or as part of the neurofibromatosis type 1 (NF1) or -2 (NF2) autosomal inherited disorder. The NF1/von Recklinghausen neurofibromatosis, caused by germ line mutations of the NF1 tumor suppressor gene, is one of the most common autosomal dominant inherited disorders, occurring at a frequency of one in every 4,000 individuals [1]. Patients with this disease have an increased risk of benign
Malignant peripheral nerve sheath tumors (MPNST) – Clinicopathological study and treatment outcome of twenty-four cases
Madhabananda Kar, SV Suryanarayana Deo, Nootan Shukla, Ajay Malik, Sidharth DattaGupta, Bidhu Mohanti, Sanjay Thulkar
World Journal of Surgical Oncology , 2006, DOI: 10.1186/1477-7819-4-55
Abstract: A retrospective analysis of 24 MPNST patients, treated from 1994 to 2002, in the department of Surgical Oncology at All India Institute of Medical Sciences, New Delhi, was done. A combination of gross, histopathological and immunohistochemical findings, and proliferation markers (MIB1) were considered for diagnosis and grade of the MPNST. Survival analysis was done by the Kaplan-Meier method and differences were evaluated with the log-rank test. Multivariate analysis was carried out by using Cox's proportional hazards model by using SPSS (Version 9, Chicago, Illinois) software.MPNST constituted 12% of all soft tissue sarcomas, where 21% (5/24) of patients had associated Von Recklinghausen's disease (VRHD). A higher incidence of male preponderance and multifocal MPNST were noted in the present series. At a mean follow-up of 38 months, 13 (54 %) patients had relapse of disease and 5-year over all and disease free survival were 58% and 35% respectively. In univariate analysis, sex (p = 0.05), tumor depth (p < 0.03), and cellular differentiation (p < 0.002) were shown to be adverse prognostic factors for disease free survival and sex (p = 0.04), cellular differentiation (p < 0.0004), and tumor grade (p = 0.05) for overall survival. However, in multivariate analysis, cellular differentiation (p < 0.005) and tumor grade (p < 0.01) emerged as independent prognostic factors for both disease free and overall survival, respectively. Postoperative radiotherapy (RT) has shown a definite role in both disease free and overall survival in this study.MPNSTs constituted a significant proportion (12%) of soft tissue sarcoma in our medical center. Heterogeneous differentiation and multifocality of the tumor were few distinct features of MPNST. Sex and cellular differentiation were noticed as the new adverse prognostic factors and adjuvant radiotherapy has been proved to be a significant treatment tool in the current series.Malignant peripheral nerve sheath tumor (MPNST) is a rare vari
Slow Spinal Cord Compression Inducing by Malignant Peripheral Nerve Sheath Tumors in Cotonou  [PDF]
D. Gnonlonfoun, C. Adjien, J. Nyangui Mapaga, L. Hode, G. Goudjinou, A. Sowanou, R. Domingo, P. Gnigone, G. Mambila, D. Affanou, P. H. Kouna Ndouongo, D. Houinato
Neuroscience & Medicine (NM) , 2018, DOI: 10.4236/nm.2018.91004
Abstract: MPNST is a very uncommon malignant type of neoplasm. It is often associated with neurofibromatosis type 1 (von Recklinghausen disease). It involves large anatomical regions, and thus takes on varied clinical presentations. However, bone location of MPNST, particularly in the spinal canal has been poorly described in the literature. We hereby report the case of a 29-year old young man with MPNST in the spinal canal. He presented a slow spinal cord compression confirmed by spinal MRI. MPNST was revealed through histologic and immune histochemical features after tumor resection.
Sulindac derivatives inhibit cell growth and induce apoptosis in primary cells from malignant peripheral nerve sheath tumors of NF1-patients
Silke Frahm, Andreas Kurtz, Lan Kluwe, Faris Farassati, Reinhard E Friedrich, Victor F Mautner
Cancer Cell International , 2004, DOI: 10.1186/1475-2867-4-4
Abstract: Exisulind and Sulindac Sulfide showed a dramatic time- and dose-dependent growth inhibitory effect with IC50-values of 120 μM and 63 μM, respectively. The decrease in viability of the tested cells correlated with induction of apoptosis. Treatment with 500 μM Exisulind and 125 μM Sulindac Sulfide for a period of 2 days increased the rate of apoptosis 21-27-fold compared to untreated cells. Reduced expression of RAS-GTP and phosphorylated ERK1/2 was detected in treated MPNST cells. Moreover, elevated levels of phosphorylated SAPK/JNK were found after drug treatment, and low activation of cleaved caspase-3 was seen.Our results suggest that this class of compounds may be of therapeutic benefit for Neurofibromatosis type 1 patients with MPNST.The malignant peripheral nerve sheath tumor (MPNST) is one of the most aggressive neoplasias of soft tissue, characterized by neurological deficits, pain and a rapid increase in size. Surgical removals or amputations do not prevent from recurrences with increased morbidity and fatality. More than 50% of individuals with MPNSTs also have neurofibromatosis type 1 (NF1), and approximately 10% of NF1 patients develop MPNSTs, of whom only 21% survive for five years after diagnosis [1,2]. NF1 is a common genetic disease with an incidence of 1:3500 [3,4], caused by mutations of the NF1 tumor suppressor gene located on chromosome 17q11.2 [5]. One proposed function of the NF1 gene product neurofibromin is the downregulation of activated RAS, based on the conversion of RAS-GTP to RAS-GDP via its GTPase enzymatic activity [6]. However, the loss of NF1 gene function is not the unique molecular lesion in these tumors, inactivation of p53 and p16 gene regions seem to play a crucial role in the malignant transformation of MPNSTs [7,8]. To this date, there is no effective treatment of NF1 patients with this malignancy [9]. Our interest focused on the therapeutic use of oral, non-toxic agents which may be able to control the progression of MPNSTs.Su
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