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Axons induce differentiation of neurofibroma Schwann-like cells.

Neurofibromatosis type 1 (NF-1, von Recklinghausen's disease) is characterized by the focal accumulation of Schwann-like cells (SLC) to form subcutaneous and plexiform neurofibromas and schwannomas. The aim of the present study was to determine whether NF-SLC are competent to differentiate in the presence of axons. Five dermal neurofibromas from five patients with NF-type 1 were enzymatically dissociated and the resultant cells were co-cultured with fetal rat dorsal root ganglion neurons. The cultures were studied by indirect immunofluorescence microscopy using antibodies against galactocerebroside (galC), P0 glycoprotein, human nerve growth factor receptor (NGFR) and human myelin-associated glycoprotein (MAG). SLC were strongly NGFR+ but galC- and MAG-SLC for the 2 weeks of coculture. After 3 weeks in vitro, SLC-NGFR was down-regulated but some of the spindle shaped cells had become galC+. MAG-SLC first appeared after 5 weeks in vitro but P0 glycoprotein was never detected when studied up to 6 weeks. Our data demonstrate that axons induce SLC to down-regulate surface NGFR and to express some myelin components in a qualitatively normal fashion.

Animals↗

Haemorrhagic necrosis of the grey matter of the spinal cord due to accidental injection of iopamidol in a patient with multiple neurofibromas; a clinico-pathological study.

Cervical laminectomy was performed in a 34-year-old man with multiple spinal neurofibromas because of a slowly progressive medullary compression. Four weeks later a rapid deterioration necessitated iopamidol myelography by left lateral cervical puncture at C2 level. Despite the establishment of adequate spinal fluid contact, resulting in imaging of the subarachnoid space, part of the contrast medium entered the spinal cord, thus delineating a syrinx from the upper cervical extending to the upper thoracic level. After the puncture the patient developed triplegia, involving the left arm and both legs and a paresis of the right arm. He died from aspiration pneumonia. Autopsy revealed haemorrhagic necrosis of the spinal grey matter. This adverse effect of myelography is argued to have been conditioned by the extreme immobility and displacement of the spinal cord due to the presence of multiple neurofibromas. The deterioration four weeks after the operation was probably caused by a further compression of the spinal cord.

Adult↗

Case report 828: Plexiform neurofibroma of the tibial nerve invading the medial and lateral gastrocnemius muscles and plantaris muscle.

A 5-year-old boy presented with a several-year history of swelling of his calf and muscular weakness. MRI demonstrated abnormal signal and diffuse enlargement of the gastrocnemius muscle. Pathological examination revealed plexiform neurofibroma, and the diagnosis of neurofibromatosis was subsequently made. The manifestations of neurofibromatosis and the peripheral nerve sheath tumors encountered in this disease are discussed. Special attention is paid to the MR features of the plexiform neurofibroma which is characteristic of this disorder.

Child, Preschool↗

Melanotic neurofibroma: a case report with ultrastructural study.

The authors report a case of a solitary melanotic neurofibroma involving the temporomalar area in a 17-year-old man. Light-microscopic study reveals a benign neurofibroma including melanin deposits. Electron microscopy shows typical premelanosomes and melanosomes within Schwann cell cytoplasm. The literature is reviewed, and theories of histogenesis for this unusual tumor are discussed.

Adolescent↗

Tactile-like structures in neurofibromas. An ultrastructural study.

The light- and electron-microscopic features of four neurofibromas are described. In each case, whorled structures resembling tactile corpuscles were observed. Ultrastructurally, these lamellated formations were composed predominantly of perineurial cells, although many had a "central core" containing either a myelinated or unmyelinated axon, or several Schwann cells unassociated with axons. Our findings provide further evidence for the participation of perineurial cells as well as Schwann cells in the histogenesis of neurofibromas.

Adult↗

Tactile-like corpuscles in neurofibromas: immunohistochemical demonstration of S-100 protein.

Tactile-like corpuscles in neurofibroma tissues were examined by the indirect immunoperoxidase method using rabbit anti-S-100 sera. In all five cases examined, positive staining was observed in their constituent cells, both in the flattened cytoplasm and nuclei. On examination of the normal peripheral nervous system, positive staining was exclusively confined to Schwann cells in the nerve bundles and Schwann-related cells such as lamellar cells in the Meissner corpuscle, whereas staining was negative in perineurial cells. Electron-microscopic examination showed ultrastructural similarity between constituent cells of the tactile-like corpuscles in neurofibroma and lamellar cells in the Meissner corpuscle. These findings may indicate Schwannian nature of the tactile-like corpuscles.

Adolescent↗

Angiographic demonstration of gastrointestinal neurofibromas in von Recklinghausen's disease.

Two cases of intestinal neurofibromas which were demonstrated during mesenteric arteriography are described. In 1 patient, tumors located in the jejunum and distal ileum were the apparent source of gastrointestinal bleeding. In another case, a neurofibroma of the proximal jejunum was an incidental finding during evaluation for severe pancreatitis and bleeding from esophageal varices.

Adult↗

A case of neurofibroma located in the retroperitoneum involving the uncinate process of the pancreas.

A case of solitary neurofibroma in the retroperitoneum involving the uncinate process of the pancreas, which has been reported in only one case in Japan and less than 20 cases in the world literature, is described. A 57-year-old male complained of back pain and microhematuria. Plain and enhanced abdominal computed tomography (CT) indicated an 8 x 6 cm hypovascular tumor located in the uncinate process. We initially assumed it to be a cancer of the pancreas. His physical examination and blood chemistry result were normal. Various diagnostic imaging methods however, disclosed a rare type of tumor. The tumor was resected by pancreaticoduodenectomy and histological examination revealed it to be neurofibroma.

Humans↗

Neurofibroma with increased uptake of [F-18]-fluoro-2 deoxy-D-glucose interpreted as a metastatic lesion.

We report a patient with a solitary spinal neurofibroma in the posterior mediastinum interpreted as a metastatic tumor. A 46-year-old female with rectal cancer who had undergone operation and subsequent adjuvant chemotherapy two years previously was referred to our department for a follow-up whole body FDG-PET study. PET scan revealed a mass with increased uptake of FDG (SUV = 4.6) in the posterior mediastinum. MRI examination showed a dumbbell neurogenic tumor originating from the intercostal nerve at T6 level. A subsequent CT-guided biopsy demonstrated a neurofibroma.

Diagnosis, Differential↗

A rapidly growing benign intrathoracic neurofibroma after lung lobectomy.

A 67-year-old male underwent a right upper lung lobectomy for lung cancer in January 1993. Follow-up chest X-rays revealed a progressive and rapidly growing intrathoracic mass in the right thorax. The mass, however, did not resemble a tumor recurrence, and the patient complained only of shortness of breath. Computerized tomography and magnetic resonance imaging confirmed the presence of the intrathoracic mass and its associated compression of the residual lung. A right thoracotomy was performed in January 1998, and a mass found arising from the sympathetic nerve trunk was resected. Microscopic examination revealed stellate or spindle-shaped cells in myxoid stroma with sparsely distributed collagen fibers. Immunohistochemically, the cells were positive for neuron-specific enolase, and the tumor was identified as neurofibroma. The patient did not suffer from von Recklinghausen's disease, and there was no family history of the disease. After resection of the neurofibroma, the compressed lung was able to re-expand, and the patient's shortness of breath disappeared. At one year postoperative, the patient remains well, and there is no evidence of recurrence.

Aged↗

Mesenteric plexiform neurofibroma associated with Recklinghausen's disease.

We present a rare case of plexiform neurofibroma involving the ileal mesentery of a 10-year-old boy with Recklinghausen's disease. MRI showed multiple ring-like structures in the masses on T2-weighted and contrast enhanced T1-weighted images. Probably these findings reflect enlarged peripheral nerves with myxoid degeneration. MRI is useful for the diagnosis of mesenteric plexiform neurofibroma because of this characteristic appearance.

Child↗

Diffuse, retroperitoneal mesenteric and intrahepatic periportal plexiform neurofibroma in a 5-year-old boy.

We present a case of plexiform neurofibroma involving the retroperitoneum, mesentery, and liver in a 5-year-old boy who underwent evaluation for extent of a palpable left neck mass. The mass had intrathoracic extension with great vessel encasement and extension into the abdomen. Abdominal CT revealed a diffuse low-attenuation non-enhancing mass encasing the retroperitoneal vessels with serpiginous extension into the liver along the portal vein. This spread pattern of plexiform neurofibroma is an unusual manifestation of neurofibromatosis in a young child.

Child, Preschool↗

Plexiform neurofibroma during and after pregnancy.

Neurofibromatosis is a genetic disease that affects multiple organ systems, and has a wide range of clinical features. Neurofibromatosis in pregnancy is encountered with a frequency of 1:2434 to 1:18,500 deliveries. Plexiform neurofibromas are rare subcutaneous nodules or amorphous overgrowth of subcutaneous tissues, which may affect the underlying bone, and produce deformities. We present a patient with a giant plexiform neurofibroma of the thigh which got larger in pregnancy, and shrunk a bit in the postpartum period.

Adult↗

Localized orbital neurofibromas.

Of nine patients (five men and four women, 25 to 55 years old) with localized orbital neurofibromas, only one had other systemic findings consistent with neurofibromatosis. The neurofibromas originated from sensory nerves of the orbit, producing gradual proptosis with expansion of the orbital walls in most cases. Mild orbital discomfort occurred in some patients. Preoperative and postoperative anesthesia in the distribution of the involved sensory nerves was also encountered. Five patients had multiple tumors within the same orbit. Four patients had tumors or a pedicle of the tumor extending into the superior orbital fissure. Two patients were initially thought to have fibrotic pseudotumors. Surgical excision is the treatment of choice for these tumors.

Adult↗

Neurofibroma and schwannoma of the oral cavity. A clinical and ultrastructural study.

The clinical parameters of fifty-five cases accessioned as neurofibroma and schwannoma of the oral cavity were reviewed. An electron microscope study was performed on several cases. The component cells of neurofibroma were spindle shaped, with bundles of collagen fibrils between cells. Junctional complexes were observed between adjacent cells. Component cells of the schwannoma showed numerous convoluted cytoplasmic processes. Reduplication of basal lamina was frequently observed and formed the material in the hyalinized area of Verocay bodies.

Adolescent↗

Plexiform neurofibroma of major salivary glands in children.

Two cases of plexiform neurofibroma of major salivary glands in children are presented. The sites were the sublingual glands in one and the parotid and submaxillary glands in the other. The four previously reported cases in children, all involving the parotid, are briefly reviewed. A sarcomatous component was present in one case. Although plexiform neurofibroma of the major salivary glands is unusual, the possibility should be considered in those patients with von Recklinghausen's disease who develop a major salivary gland tumor.

Child, Preschool↗

Solitary neurofibroma of the mandible and infratemporal fossa in a young child. Report of a case.

The first case of a rare and unusual solitary neurofibroma of the mandible that caused bony erosion and extension into the adjacent soft tissues of the infratemporal fossa is presented. The clinical, radiographic, and histopathologic features of the neurofibroma are reviewed. The diagnostic procedures and the surgical treatment of this tumor in a young child are discussed.

Child, Preschool↗