Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0729233 (Thoracic)
 6,478 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report two cases of ventrolateral thoracic and lumbar meningoceles associated with neurofibromatosis type 1, and present a review of the literature. Thoracic and lumbar meningoceles are rare lesions which are associated with neurofibromatosis in 60% to 85% of cases. Large meningoceles may cause pain, neurological signs, or respiratory complaints when located intrathoracically. Surgery is indicated in such cases, as well as when small meningoceles enlarge with time. The etiology of meningoceles in neurofibromatosis remains controversial. The authors postulate that ventrolateral lesions of the thoracic and lumbar spine are secondary to congenital mesodermal dysplasia and hypoplastic bone changes. MRI is the diagnostic method of choice, since it most adequately visualizes the pathological anatomy of the spinal cord, meninges, and adjacent structures in every sectional plane. CT scans are particularly useful in assessing the spinal bony changes.
 ...
PMID:Thoracic and lumbar meningocele in neurofibromatosis type 1. Report of two cases and review of the literature. 747 16

We present a 25-year-old male patient with neurofibromatosis type 1 (NF1). Thoracic intra-dural extra-medullary tumoral mass was excised gross-totally and the patient was referred to oncology unit. Histopathological diagnosis was malignant peripheral nerve sheath tumor (MPNST), a rare sarcoma with a dismal prognosis. Tumor recurred in its previous site with an adjacent apical mass in the left lung 7 weeks following initial surgery and repeat surgery was performed with complete removal of intra-dural tumor. We report the first patient with intra-dural MPNST localized proximal to conus medullaris; in upper thoracic spine. It must always be considered the possibility of a rare but a devastating tumor, MPNST beside schwannomas and neurofibromas in patients with NF1 when an intra-spinal mass is diagnosed.
 ...
PMID:A case of intra-dural malignant peripheral nerve sheath tumor in thoracic spine associated with neurofibromatosis type 1. 1659 31