eRAM
encyclopedia of Rare Disease Annotation for Precision Medicine
| Disease | neurofibromatosis type 1 |
| Symptom | C0751689|peripheral nerve sheath tumors |
| Sentences | 8 |
| PubMedID- 23244685 | Molecular heterogeneity in malignant peripheral nerve sheath tumors associated with neurofibromatosis type 1. |
| 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- 20074816 | Plexiform neurofibromas are peripheral nerve sheath tumors associated with neurofibromatosis type 1. |
| PubMedID- 23101443 | Role of resection of malignant peripheral nerve sheath tumors in patients 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- 26445379 | Diagnostic accuracy of pet/ct-guided percutaneous biopsies for malignant peripheral nerve sheath tumors in neurofibromatosis type 1 patients. |
| 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- 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. |
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