eRAM
encyclopedia of Rare Disease Annotation for Precision Medicine
| Disease | malignant peripheral nerve sheath tumor |
| Comorbidity | C0085113|neurofibromatosis |
| Sentences | 39 |
| PubMedID- 23972085 | Chemotherapy for the treatment of malignant peripheral nerve sheath tumors in neurofibromatosis 1: a 10-year institutional review. |
| PubMedID- 24426497 | Here we report a rare case of malignant peripheral nerve sheath tumour, not associated with neurofibromatosis type 1 and arising in the retroperitoneum. |
| PubMedID- 24396458 | The majority of gastric malignant peripheral nerve sheath tumors are associated with neurofibromatosis (13). |
| PubMedID- 24009526 | Recently, 23 human mpnsts in patients with inherited neurofibromatosis type 1 (nf1; heterozygous germline nf1 mutation) were examined using high resolution acgh [41] (figure s3b). |
| PubMedID- 23292448 | Since the bi-allelic inactivation of both neurofibromin 1 (nf1) gene alleles (nf1(-/-)) in schwann cells (scs) is common in both benign plexiform neurofibromas (pns) and malignant peripheral nerve sheath tumors (mpnsts) in patients with neurofibromatosis type 1 (nf1), other genetic alterations in scs may be required for tumor progression of pns to mpnsts. |
| PubMedID- 23242059 | Purpose: the aim of this study was to compare the diagnostic performance of (1)(8)f-fluorodeoxyglucose (fdg) pet/ct and whole-body mri for the detection of malignant peripheral nerve sheath tumors (mpnsts) in patients with neurofibromatosis type 1, and to evaluate a panel of imaging-based criteria serving that purpose. |
| PubMedID- 22745048 | Metronomic therapy for malignant peripheral nerve sheath tumor in neurofibromatosis type 1. |
| PubMedID- 25621889 | Rassf2 is also downregulated in malignant peripheral nerve sheath tumors (mpnsts) associated with neurofibromatosis type 1 relative to non-malignant plexiform neurofibromas [41], probably as a consequence of its hypermethylation. |
| PubMedID- 23101443 | Role of resection of malignant peripheral nerve sheath tumors in patients with neurofibromatosis type 1. |
| PubMedID- 25317349 | malignant peripheral nerve sheath tumors in children with neurofibromatosis type 1. |
| PubMedID- 20205747 | malignant peripheral nerve sheath tumor associated with neurofibromatosis type 1, with metastasis to the heart: a case report. |
| PubMedID- 23244685 | Molecular heterogeneity in malignant peripheral nerve sheath tumors associated with neurofibromatosis type 1. |
| PubMedID- 24097302 | 18f-fdg pet/ct for detection of malignant peripheral nerve sheath tumours in neurofibromatosis type 1: tumour-to-liver ratio is superior to an suvmax cut-off. |
| PubMedID- 25452937 | This study examines the outcomes of adjuvant radiation therapy in mpnst in patients with and without neurofibromatosis type 1 (nf1) and reviews the literature on use of radiation for mpnst. |
| PubMedID- 24392233 | Studied mpnst in patients with and without neurofibromatosis and reported 5-year survival rates of 16% and 53%, respectively [6]. |
| PubMedID- 21731277 | Intraoral presentation of multiple malignant peripheral nerve sheath tumors associated with neurofibromatosis-1. |
| PubMedID- 25810463 | Approximately half of mpnsts develop in patients with neurofibromatosis type 1 (nf1), while the other half develop sporadically (evans et al, 2002; ferner & gutmann, 2002; carli et al, 2005). |
| PubMedID- 20664435 | Conclusion: neurofibromatosis type 1 with malignant peripheral nerve sheath tumors complicating pregnancy requires an experienced, multidisciplinary team of care offering an aggressive evaluation to rule out malignant transformation or recurrence when there is any change in clinical status of a patient, as this may signal a potentially fatal change in the lesion. |
| PubMedID- 24932270 | Approximately 50% of mpnsts occur in patients with neurofibromatosis type 1 (nf1), and 10% of these are radiation-induced, while the remainder affect individuals without a known genetic predisposition. |
| PubMedID- 23047517 | Metastatic malignant peripheral nerve sheath tumor in neurofibromatosis type 1: a geriatric patient report. |
| PubMedID- 22084762 | Approximately 50% of mpnst occur in patients with neurofibromatosis type 1 [7]. |
| PubMedID- 21196210 | malignant peripheral nerve sheath tumors (mpnsts), often found associated with neurofibromatosis type 1 (nf1), are aggressive tumors that pose significant diagnostic and therapeutic challenges. |
| PubMedID- 24343189 | malignant peripheral nerve sheath tumor in neurofibromatosis type 1. |
| PubMedID- 25810689 | The diagnosis was not changed in 2 cases (1 rhabdomyosarcoma (rms) and 1 malignant peripheral nerve sheath tumor in a patient with neurofibromatosis-1), and in the remaining 3 (2 retroperitoneal rms and 1 solitary fibrous tumor in the thigh), the final conclusions remained equivocal. |
| PubMedID- 23483452 | We used genome-wide serum mirna expression analysis to investigate the role of serum mirnas in distinguishing malignant peripheral nerve sheath tumor (mpnst) patients with and without neurofibromatosis type 1 (nf1) from nf1 patients. |
| PubMedID- 22869071 | A novel nf1 mutation in a chinese patient with giant cafe-au-lait macule in neurofibromatosis type 1 associated with a malignant peripheral nerve sheath tumor and bone abnormality. |
| PubMedID- 20686424 | The role of angiogenesis in the transformation of plexiform neurofibroma into malignant peripheral nerve sheath tumors in children with neurofibromatosis type 1. |
| PubMedID- 24166582 | Benign whole body tumor volume is a risk factor for malignant peripheral nerve sheath tumors in neurofibromatosis type 1. |
| PubMedID- 24250026 | Until 50% of observed mpnsts occur in patients with neurofibromatosis 1 (nf1). |
| PubMedID- 24516579 | Approximately half of mpnsts manifest in patients with neurofibromatosis type 1 (nf1; von recklinghausen disease) [1], and patients with nf1 have a 5–10% lifetime risk of mpnst [2], [3]. |
| PubMedID- 23161774 | Survival meta-analyses for >1800 malignant peripheral nerve sheath tumor patients with and without neurofibromatosis type 1. |
| PubMedID- 21246207 | Current knowledge also showed that most mpnsts are associated with neurofibromatosis type 1 [2, 3]. |
| PubMedID- 22348274 | Cases were neurofibromatosis 1 adults with mpnsts and controls were patients without mpnsts individually matched by age and sex (1 : 3). |
| PubMedID- 26018702 | Foreign body abscess mimicking a malignant peripheral nerve sheath tumor in a patient with neurofibromatosis type 1. |
| PubMedID- 20634933 | For the vast majority of cases, the aetiology is unknown, although there are certain genetic associations, such as the 10% lifetime risk of malignant peripheral nerve sheath tumour (mpnst) in individuals with familial neurofibromatosis, caused by mutations in the nf1 gene [13]. |
| PubMedID- 24527089 | In total, ~50% of mpnst cases arise in patients with neurofibromatosis type 1 (nf1; von recklinghausen’s disease) and the five-year overall survival rate is 34% (1). |
| PubMedID- 26445379 | Diagnostic accuracy of pet/ct-guided percutaneous biopsies for malignant peripheral nerve sheath tumors in neurofibromatosis type 1 patients. |
| PubMedID- 23196734 | Association of mpnst with neurofibromatosis type 1 (nf1) has been frequently reported as a result of malignant transformation of a pre-existing plexiform neurofibroma. |
| PubMedID- 23209032 | mpnsts occur frequently in patients with neurofibromatosis type 1 (nf1), in which nf1 gene deficiency leads to ras hyperactivation. |
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