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neurofibroma/phosphatase

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13 resultats

Atypical neurofibroma and osteosclerotic metastasis.

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35-year-old male presented with multiple swellings in left leg, headache, weakness of limbs for 4 months, and blurring of vision for the last 15 days. On examination, he was pale, cachexic with generalized lymphadenopathy and lower motor neuron type weakness of limbs sparing right upper limb. Blood

Paraspinal neurofibromas and hypertrophic neuropathy in Noonan syndrome with multiple lentigines.

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BACKGROUND Noonan syndrome with multiple lentigines (NSML), formerly known as LEOPARD syndrome, is an autosomal-dominant disorder characterised by lentigines, EKG abnormalities, ocular hypertelorism, pulmonic stenosis, abnormal genitalia, growth retardation and deafness. There is significant

Targeted Inhibition of the Dual Specificity Phosphatases DUSP1 and DUSP6 Suppress MPNST Growth via JNK.

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In neurofibromatosis type 1 (NF1) and in highly aggressive malignant peripheral nerve sheath tumors (MPNSTs), constitutively active RAS-GTP and increased MAPK signaling are important in tumorigenesis. Dual specificity phosphatases (DUSPs) are negative regulators of MAPK signaling that

Conditional Inactivation of Pten with EGFR Overexpression in Schwann Cells Models Sporadic MPNST.

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The genetic mechanisms involved in the transformation from a benign neurofibroma to a malignant sarcoma in patients with neurofibromatosis-type-1- (NF1-)associated or sporadic malignant peripheral nerve sheath tumors (MPNSTs) remain unclear. It is hypothesized that many genetic changes are involved

In situ distribution of the beta-subunit of platelet-derived growth factor receptor in nonneoplastic tissue and in soft tissue tumors.

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The distribution of the beta-subunit of platelet-derived growth factor receptor (PDGFR-beta) was assessed by a sensitive immunoalkaline phosphatase technique using the monoclonal antibody PR7212. Frozen tissue sections of several nonneoplastic human tissues were stained along with 42 soft tissue

Culture and phagocytic characteristics of Schwann cells in vitro. A possible model substrate for cultivation of M. leprae.

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Tissue cultures of five acoustic and four spinal schwannomas demonstrated good growth and migration of Schwann cells within two to four days. Two types of cells corresponding to Antoni type A tissue and Antoni type B tissue were clearly recognized. Both these cell types showed avid phagocytosis when

Loss of heterozygosity in tumor cells of a recurrent mandibular giant cell granuloma in neurofibromatosis type 1.

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Neurofibromatosis type 1 (NF1) is an autosomal dominant inherited disease affecting about 1:3000 humans. Neurofibromas are benign soft tissue tumors. Giant cell granuloma (GCG) is a benign tumor-like lesion that is preferentially located in the jaws. GCG can develop in NF1 patients. A 7-year-old

Extraskeletal osteoclastomas responsive to dexamethasone treatment in Paget bone disease.

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Giant cell tumors (GCTs) of bone, also called osteoclastomas, complicate Paget bone disease (PBD), though infrequently. Giant cell reparative granulomas (GCRGs), which are histologically similar to GCTs, also occur rarely in pagetic patients. A 45-yr-old black woman with neurofibromatosis, type I,

Adult hypophosphataemic osteomalacia with Fanconi syndrome presenting in a patient with neurofibromatosis.

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A patient is described with neurofibromatosis, increasing walking problems and low back pain. Initially, osteoporosis or pressure caused by a neurofibroma was suspected. However, the progressively increasing alkaline phosphatase activity and hypophosphataemia found during laboratory investigations

[Immunohistochemical findings in intrathoracic tumors. III. Detection of carcinoembryonic antigen in tumor tissue].

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35 intrathoracic tumors were investigated for the presence of carcinoembryonic antigen. Additionally, activity of alkaline phosphatase and nonspecific esterase was determined in correlation to the presence of carcinoembryonic antigen. Carcinoembryonic antigen was detected in 60% of all tumors and in
The genetic evolution from a benign neurofibroma to a malignant sarcoma in patients with neurofibromatosis type 1 (NF1) syndrome remains unclear. Schwann cells and/or their precursor cells are believed to be the primary pathogenic cell in neurofibromas because they harbor biallelic neurofibromin 1

EGFR and erbB2 in malignant peripheral nerve sheath tumors and implications for targeted therapy.

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Malignant peripheral nerve sheath tumors (MPNSTs) are sarcomas with poor prognosis and limited treatment options. Evidence for a role of epidermal growth factor receptor (EGFR) and receptor tyrosine kinase erbB2 in MPNSTs led us to systematically study these potential therapeutic targets in a larger

Bone mineral metabolism in patients with neurofibromatosis type 1 (von Recklingausen disease).

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The neurofibromatosis type 1 (NF1) is characterized by specific cutaneous features (neurofibromas, "café-au-lait" spots of the skin) and alterations of several tissue (nervous, vascular) and bone deformities, such as scoliosis, congenital pseudoarthrosis and bone dysplasia of tibia. Moreover,
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