Nerve Tissue Neoplasms (Subscribe)  

Definition Neurofibromatosis type I (NF-1), along with Neurofibromatosis type II (a.k.a. MISME syndrome), Tuberous Sclerosis, Sturge-Weber, and Von Hippel-Lindau compromise the phakomatosis or neurocutaneous syndromes, all of which have neurologic and dermatologic lesions. This grouping is an artifact of an earlier time in medicine, before the distinct genetic basis of each...

Case report of a 5 year old child with Neurofibromatosis Type 1 who developed a Charcot knee
Lokiec et al; JBJS B 1998 80: 468

Discussion: - also called malignant schwannoma or fibrosarcoma of nerve sheath; - extracompartmental high grade tumor that develops from sheaths (Schwann cells) of peripheral nerves; - usually is a primary lesion occuring along proximal major nerve trunks, ...

Search String site:www.ncbi.nlm.nih.gov dystrophic (curvature OR scoliosis) (neurofibroma OR neurofibromatosis) scoliosis
22 papers cited July 2007

Primitive Neuroectodermal Tumor (PNET) is a soft tissue sarcoma classically described under small round blue cell tumors (SRBCT). There is considerable clinical and histologic overlap between this tumor and Ewing's sarcoma (ES). For many years, pathologists struggled to make a diagnostic distinction. Now with sophisticated molecular biological analysis, it turns...

Neurofibromatosis, Von Recklinghausen's disease, is an autosomal dominant inherited disorder of mesenchymal tissue, where fibrous skin tumours, neurilemmomas and areas of skin pigmentation ( cafe-au-lait spots ) are typical features. Neurolemmomas can affect cranial or peripheral nerves.
Bone radiographs may show disturbance of bone architecture which can immitate polyostotic fibrous dysplasia,...

- 2 subtypes: idiopathic and dystrophic; - dysplastic type: (dystrophic) - short, sharply angulated curve which involve only few vertebra; - associated w/: ...

This is a descriptive category describing a large number of malignant tumors that tend to occur in childhood. They are united by having a similar histologic appearance, that is, small round blue cells. Subtle clues may be present to distinguish between the tumors. The pathologist is assisted by immunohistochemistry, electron...

Rajagopal KL, Lakhkar BN. Solitary giant neurilemoma in the psoas muscle. Indian J Radiol Imaging 2002;12:551-552
Full Text available
The case of a 38-year old male with a giant neurilemoma in the left psoas muscle is reported, which was not associated with von Recklinghausen's disease. Ultrasound and CT showed a large cystic...

Neurofibromatosis is often associated with a dysplastic progressive scoliosis. Enlargement of the neural foramina by erosion of adjecent neurofibomas occasionally predisposes to the adjacent attached rib entering the spinal canal. We describe such a case in which partial rib excision and later posterior spinal fusion resulted in complete recovery (full...

Surgical Correction of Dystrophic Spinal Curves in Neurofibromatosis: A Review of 56 Patients.
Spine. 24(21):2247, November 1, 1999. (abstract) Parisini, Patrizio MD *; Di Silvestre, Mario MD *; Greggi, Tiziana MD *; Paderni, Stefania MD *; Cervellati, Stefano MD *; Savini, Romolo MD *+
Conclusions. The severe dystrophic curve with anterior vertebral...

Spine 1997, vol. 22, no23, pp. 2770-2776 (25 ref.)
Abstract: When evaluating patients with neurofibromatosis and scoliosis, a careful search for the evidence of dystrophic changes should done, because prognosis and management depend highly on the presence of these changes. Preoperative imaging studies including computed tomography or magnetic-resonance imaging are recommended,...

Can J Surg 2002 45: #3, 181 Conclusion: In children with scoliosis but no NF1 there is a range of mild scalloping whereas those with NF1 have severe scalloping

The neurofibromatoses are genetic disorders of the nervous system that primarily affect the development and growth of neural (nerve) cell tissues. These disorders cause tumors to grow on nerves and produce other abnormalities such as skin changes and bone deformities. National Institute of Neurological Disorders and Stroke Highly Reputable