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Neuroblastoma growth factors derived from neurofibroma (NF1): participation of uridine in a neuroblastoma growth.

Human glioma cell extracts were found to elicit a marked growth-promoting activity on human neuroblastoma cells. This activity was also detected in the extracts of neurofibroma type 1 (NF1; von Recklinghausen neurofibromatosis) comprising aberrant Schwann cell growth. The purified substance from the NF1 extracts by HPLC on ODS columns was identical to a pyrimidine nucleoside, uridine, the chemical structure of which was identified by gas chromatography-mass spectrometry. The authentic uridine showed a strong growth-promoting activity on human neuroblastoma cells. Other purine or pyrimidine nucleotides, their derivatives, and ribose sources for their syntheses were employed to test the activity; a purine nucleoside, adenosine, showed a stronger activity than uridine. The current study raises the possibility that human neuroblastoma cells may be affected by dysfunctions of the de novo pathway of both purine and pyrimidine nucleotide biosyntheses.

Cell Division↗

Treatment of multiple facial neurofibromas with dermabrasion.

Three patients with multiple facial neurofibromas were treated by dermabrasion with good cosmetic results. Wound healing was entirely normal and no complications were observed. There was no evidence of accelerated regrowth of tumors during follow-up.

Adult↗

Stimulation of folliculo-sebaceous proliferations by neurofibromas: a report of two cases.

Two unrelated patients, a woman aged 44 and a man aged 56, with a type I neurofibromatosis, showed comedo-like formations at the top of some neurofibromas. One of them was excised in each case and they were studied histopathologically. The comedo-like formation of the first case mimicked the epithelial component of a fibrofolliculoma, and that of the second case had the structure of a primary late microcomedo and showed also anagen hair differentiation in the form of a follicular bulb and papilla. Both events are considered to be the result of a stimulation phenomenon and to reflect the essential role of the stroma in many cutaneous epithelial hyperplasias, hamartomas and possibly in some neoplasms.

Adult↗

Neurofibromas: location by scanning with Tc-99m DTPA. Work in progress.

The accumulation of technetium-99m diethylenetriamine pentaacetic acid (Tc-99m DTPA) in benign soft-tissue neurofibromatosis tumors is reported. In a series of 16 patients with clinical stigmata of neurofibromatosis, 28 sites of abnormal soft-tissue localization of the isotope observed scintigraphically were documented to be sites of soft-tissue tumor by clinical and/or radiographic (predominantly computed tomographic) correlations. The smallest lesion detected was a 1.5-cm subcutaneous neurofibroma. Normal physiologic nonrenal distribution of the Tc-99m DTPA was established by scintigraphic imaging of a control population.

Adolescent↗

Central neurofibromas in two dogs.

Two dogs each had a fibrous tumor of the central nervous system. One tumor involved the spinal cord and ventral nerve root, and the other involved the midbrain and posterior brain stem. Both tumors had spindle-shaped cells arranged in rhythmic patterns. There was perivascular tumor cell infiltration in the adjacent parenchyma. The tumor cells strongly resembled fibroblasts and seemed to produce collagen. Because both tumors were near nerve roots and because their histological appearances were characteristic of nerve sheath tumors they were classified as central neurofibromas.

Animals↗

Resection of small plexiform neurofibromas in neurofibromatosis type 1 children.

BACKGROUND: Plexiform neurofibromas (PNF) are benign tumors of the peripheral nerve which mostly develop in patients with neurofibromatosis type 1 (NF1). Surgical interventions are usually not applied to children with small tumors. These are rather restricted to debulking of larger tumors in adults that cause clinical complications or aesthetic disfigurement. In most cases, a total resection of PNF is not possible due to the network-like growth of the tumors. PATIENTS AND METHODS: Early surgical intervention was carried out for 9 small PNFs in 7 NF1 children. Tumor resection was performed following the graphical delineation of the affected skin and according the MRI findings. RESULTS: Total resection was achieved for all 9 PNF without causing any neurological or organic deficit. Annual magnetic resonance tomography over a period of four years did not reveal any relapse of the tumors. CONCLUSIONS: Early surgical intervention for small superficial PNFs in NF1 children have various advantages and may especially be considered a strategy to prevent progression.

Journal Article↗

Multiple cervical and intrathoracic plexoid neurofibromas. Case report.

A 29-year-old male with known von Recklinghausen's disease is presented. The main symptom of the patient was paroxysmal episodes of pain and numbness in the right upper hand for the last 10 years. Cervical and mediastial magnetic resonance imaging (MRI) revealed 3 large tumors originating from the right vagus nerve and another of the same origin contralaterally. Surgical resection of the masses in the right hemithorax was performed via right posterolateral thoracotomy. The postoperative course was uneventful and symptoms recessed. Plexoid neurofibromas were diagnosed at histological examination. The mass in the left hemithorax is under surveillance according to the patient's preference. The clinical, radiological, surgical, and histopathological features of this rare case are discussed.

Adult↗

Storiform neurofibroma (Bednar tumor). A case report.

Clinical data and morphological features of the storiform neurofibroma, presenting as an exophytic tumor of the trunk in 30 years old white man are described. This benign tumor, is microscopically similar to dermatofibrosarcoma protuberans and differs from the letter by the presence of melanin containing cells.

Adult↗

[The storiform M-8333/1,3 neurofibroma].

Attention concentrated on features of storiform neurofibroma failed to find an unambiguous universal interpretation. 15 consecutively published cases were collectively revised with partial conclusions from a comparison of findings and references: Nosological identity of dermatofibrosarcoma protuberans and its "pigmented variant" is substantiated but fails to solve histogenetic embarrassment of both items. Characteristic storiform structure is connected with layered very flat cells. No doubt they remind of spindle-shaped fibroblasts in cross section but flat dendritic cells remain an essential component no matter whether pigmented or not. Open to discuss is whether fibroblasts belong to adaptable cells which generally can get a flat shape among dendritic cells. Their pigmentation may have local origin according to paraneuronal theory.

Fibrosarcoma↗

[Vater Paccini's neurofibroma. Report of 3 cases].

The authors study three clinical cases of Pacinian neurofibromas. All of them were located in the fingers. A bibliographic review was performed. The morphologic, clinical and histological features are reviewed and stressed its benign behavior and special localization.

Adolescent↗

[Schwannoma and neurofibroma of the neck].

Three schwannomas and one neurofibroma of the neck were treated surgically in our department during the last years (1980-1987). These tumors are relatively rare. The different clinical and histopathological criteria of the two tumors are discussed. In spite of these differences, in most cases a correct diagnosis is made only during surgery. Therefore, the possibility of temporary or permanent damage to major nerves is always present and the patient should be informed accordingly.

Adult↗

Neurofibromatosis with central neurofibroma of the mandible: review of the literature and report of case.

The clinical, radiographic, and etiological factors of neurofibromatosis have been discussed. A rare case of a large neurofibroma of the mandible in association with systemic neurofibromatosis and a characteristic mandibular dysplasia has been presented. The unique coexistence of mandibular dysplasia associated with sphenoidal and orbital dysplasia should be considered in the diagnosis of multiple neurofibromatosis.

Bone Diseases↗

[Multiple retroperitoneal neurofibroma. Report of a case].

Tumors of nervous origin in the retroperitoneum are rare without specific symptoms which diagnosis is usually very difficult. The authors report a case of an asymptomatic patient with two benign retroperitoneal neurofibromas, one of them calcified and located near the inferior pole of the left kidney; the other tumor was closely adhered to the pancreatic tail in the supramesocolic region. Both were resected with appropriated surgical margins. The histopathologic and immunohistochemical studies established the correct diagnosis of the lesions. The authors comment the diagnostic tests of these tumors, their possible malignant degeneration and their surgical treatment.

Adult↗