Search PubMed⌕ Search

SEARCH · Search PubMed

Results for “NEUROFIBROMA”

Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 343 records · Page 19Linked to original sources
PubMed2000

Parapelvic neurofibroma of the kidney.

We report the first case of neurofibroma causing obstruction by external compression of the upper renal pelvis. A 33-year-old woman reported a 1-year history of dull lumbar pain on the right side. A right renal parapelvic cyst was suspected. The patient underwent retroperitoneoscopic exploration. The right renal parapelvic lesion was identified as a soft tissue tumor. The tumor was removed with a retroperitoneoscopic procedure. Histopathological findings revealed neurofibroma.

Adult↗
PubMed1999

Pacinian neurofibroma of the hand: a case report and literature review.

Pacinian neurofibroma is a rare, benign tumor not associated with von Recklinghausen's disease (neurofibromatosis). Histologically, it is composed of a proliferation of structures resembling normal pressure receptors. A case of pacinian neurofibroma of the hand in a 4-year-old child is presented, along with a review of the literature.

Adipose Tissue↗
PubMed1997

[A rare case of a primary intrabronchial neurofibroma].

HISTORY AND CLINICAL FINDINGS: A 42-year-old woman, a smoker for many years, had suffered from dry cough for some time. She was admitted because of haemoptyses, the first one month previously. Physical examination was unremarkable. INVESTIGATIONS: Chest radiography in two planes showed no abnormality. Computed tomography revealed a space-occupying lesion adjacent to the right main bronchus, strongly suspicious of a central bronchial carcinoma. Bronchoscopy showed a smoothly circumscribed tumour in the upper lobe bronchus which almost occluded its lumen. Multiple biopsies failed to establish the benignity/malignity of the tumour. TREATMENT AND COURSE: The tumour was removed by upper lobe resection. There was no macroscopic intraoperative evidence of infiltration. Histology provided the surprising diagnosis of a benign neurofibroma. CONCLUSION: Thoracotomy should, if at all possible, be performed whenever biopsy of a pulmonary mass fails to establish its benignity. Intrapulmonary neurofibroma is very rare, unless it is part of v. Recklinghausen's disease.

Adult↗
PubMed2001

Multicentric reticulohistiocytosis with neurofibroma-like nodules.

We describe a patient with multicentric reticulohistiocytosis (MR), who presented with 2 different clinical types of lesions. Some were characteristic for MR in that they were deeply set, small, firm papules. Many others were larger, soft, and even sessile, like neurofibromas. Histopathologic features of both types of lesions revealed MR. To our knowledge, this is the first report of lesions of MR mimicking neurofibromas.

Adrenal Cortex Hormones↗
PubMed2004

Isolated neurofibroma of the orbit with extensive myxoid changes: a clinicopathologic study including MRI and electron microscopic findings.

Isolated orbital neurofibroma (INO) without systemic neurofibromatosis is rare. Previous reports including MRI with elaboration of the ultrastructural features of the cellular and extracellular components in INO do not exist. A 44-year-old male presented with painless, slowly progressive proptosis (6 mm) and downward displacement (3 mm) of the left eye of two years duration. MRI revealed a suprabulbar mass hypointense to fat, isointense to brain on T1-weighted images with small hyperintense areas following contrast enhancement. T2-weighted images showed a predominantly hyperintense mass with a few hypointense islands. The lesion was excised using an anterior superior transseptal orbitotomy. Histopathologically, large areas of a paucicellular myxoid stroma containing mucopolysaccharides made up approximately three-quarters of the mass. Focal hypercellular areas showed marked collagen deposition. Blood vessels were distributed at the periphery of the lesion and in association with nodules of nerve sheath cells. Ultrastructurally, perineural and fibroblast-like cells were loosely arranged in a myxoid stroma. Schwann cells were embedded in a dense collagenous matrix. Variable differentiation of Schwann cells, perineural cells and fibroblast-like cells associated with a more solid (collagenous) or loose (myxoid) extracellular matrix may determine the characteristic, though not specific, MRI features in isolated neurofibromas of the orbit.

Adult↗
PubMed2002

Pigmented (melanotic) neurofibroma. Report of an unusual case with immunohistochemical, ultrastructural and cytogenetic analyses.

In the spectrum of neurofibromas, pigmented tumors are rare variants usually showing only faint, macroscopically obvious pigmentation. We report a case of a huge pigmented neurofibroma with extended, macroscopically striking pigmentation in a patient with stigmata of neurofibromatosis. The immunohistochemical and ultrastructural findings support a melanotic line of differentiation besides schwann cell differentiation and indicate a phenotypic neoplastic spectrum between tumorous schwann cells and melanocytes. Using comparative genomic hybridization, striking chromosomal aberrations were not detected. High level amplifications of the known chromosomal regions, including genes of major enzymes responsible for melanin synthesis, appear to be unlikely. However, smaller chromosomal defects might have been overlooked by the limited resolution of this screening method. Therefore, other mechanisms up-regulating melanogenesis, such as mutations in regulatory genes, have to be considered.

Adult↗
PubMed1999

EVI2B, a gene lying in an intron of the neurofibromatosis type 1 (NF1) gene, is as the NF1 gene involved in differentiation of melanocytes and keratinocytes and is overexpressed in cells derived from NF1 neurofibromas.

The EVI2B gene is one of three genes embedded in intron 27b of the neurofibromatosis type 1 (NF1; M. Recklinghausen) gene, which are transcribed in the direction opposite that of the NF1 gene. The function of EVI2B and its relation to NF1 symptoms is unknown. Here, the amounts of NF1 and EVI2B mRNA were investigated in detail in cells involved in NF1 manifestations as café-au-lait macules and neurofibromas. These investigations showed that aside from the NF1 gene, EVI2B is involved in melanocyte and keratinocyte differentiation. Whereas in NF1 melanocytes from café-au-lait macules, EVI2B expression was not altered, in fibroblast-like cells derived from neurofibromas, an increased level of EVI2B mRNA was found. We investigated whether this increase was attributable to an influence of NF1 gene expression on the expression of the EVI2B gene, as suggested by the fact that the EVI2B primary transcript is antisense to the NF1 primary transcript. Investigations of cells derived from patients with different amounts of NF1 pre-mRNA showed no correlation between the amount of NF1 pre-mRNA and the increased level of EVI2B mRNA.

Adolescent↗
PubMed1994

Histopathological variants of neurofibroma. A study of 114 lesions.

Although neurofibroma is a relatively common tumor, some histopathologic variants are so rare that they are not well known. In a study of 130 neural cutaneous tumors seen between 1986 and 1991 in the department of dermatology at the University of Heinrich-Heine, we identified 114 neurofibromas of different types. We present herein the histopathological features of these tumors. The differentiating features from other neural tumors--melanocytic or mesenchymal tumors that display "neuroid" features--are also discussed. also discussed.

Adipose Tissue↗
PubMed2004

Intraneural dendritic cell neurofibroma with pseudorosettes.

Dendritic cell neurofibroma with pseudorosettes (DCNWPR) is a recently proposed variant of neurofibroma. However, its peripheral nerve sheath origin has subsequently been questioned, and it has been suggested that the neoplasm could represent a hitherto undescribed variant of melanocytic nevus with neural differentiation. Here we report a case of DCNWPR that arose almost exclusively within the confinement of the perineurium in the skin. This observation gives further evidence that this entity is a peripheral nerve sheath tumor and is unrelated to melanocytic neoplasms.

Biomarkers, Tumor↗
PubMed1988

Expression of glial fibrillary acidic protein (GFAP) in peripheral nerve sheath tumors. A comparative study of immunoreactivity of GFAP, vimentin, S-100 protein, and neurofilament in 38 schwannomas and 18 neurofibromas.

Immunoreactivity of glial fibrillary acidic protein (GFAP) in 38 schwannomas and 18 neurofibromas was evaluated and compared with the reactivity of vimentin, S-100 protein, and neurofilament protein. All cases were positive for vimentin and S-100 protein. GFAP was positively stained in the neoplastic cells of 15 of 38 schwannomas (38%) and in two of 18 neurofibromas (11%). The extensively stained GFAP-positive tumors tended to be deeply situated in the body. The GFAP-positive cells were usually spindle-shaped and appeared preferentially in the perivascular region of hyalinized, thick blood vessels.

Adolescent↗
PubMed2000

Localized neurofibroma of the orbit: a radiographic and histopathologic study.

PURPOSE: To demonstrate the clinical, radiographic, intraoperative and histopathologic presentation of a localized neurofibroma of the orbit. METHODS: Clinicopathologic case report. RESULTS: A 25-year-old man was examined for inferior displacement of the right globe. Physical examination and neuro-imaging identified a localized solid tissue mass of the superior orbit. Histopathologic evaluation after complete surgical resection proved the lesion to be a localized neurofibroma. CONCLUSIONS: The patient's history, clinical presentation, and imaging studies were strongly characteristic for this infrequently encountered orbital lesion. Several imaging features, including multilobulation, multiplicity, ring-configured contrast enhancement, and magnetic resonance imaging (MRI) signal intensity heterogeneity, when present in combination, may be reasonably diagnostic of this tumor.

Adult↗
PubMed1984

Cervical chordoma presenting with intervertebral foramen enlargement mimicking neurofibroma: CT findings.

A cervical chordoma, confirmed at surgery, presented on plain spine films as a focal enlargement of the intervertebral foramen and mimicked the characteristic appearance of cervical neurofibroma. Computed tomography (CT) of the cervical spine was obtained immediately following metrizamide myelography; it demonstrated a soft-tissue mass in the enlarged intervertebral foramen that extended posteriorly to compress the spinal cord and anteriorly to compress the hypopharynx. The mass was sharply demarcated, inhomogeneous, and low in attenuation; CT density measured between those of cerebrospinal fluid and muscle. Although this CT appearance is not specific for cervical chordoma, it is unusual for neurofibroma. Cervical chordoma should be considered in the differential diagnosis of focal enlargement of cervical intervertebral foramina.

Adult↗
PubMedDate not supplied

Spinal neurilemmomas and neurofibromas: central dot sign in postgadolinium MRI.

The MR studies of three histologically proven spinal neurilemmomas and neurofibromas were reviewed retrospectively. There were two benign neurilemmomas (schwannomas) and one neurofibroma. The common characteristic of these cases was a central low intensity focus ("dot") seen on postcontrast T1-weighted imaging. The low intensity foci corresponded histologically to a congeries of changes including edema, microcysts, foam cells, hyalinization of blood vessels, old hemorrhage, and dystrophic calcification.

Adult↗
PubMed2004

Isolated plexiform neurofibroma: treatment with three-dimensional conformal radiotherapy.

OBJECTIVES: To present a case of an unusual benign tumor of the tongue treated successfully with radiotherapy. STUDY DESIGN: Case report. METHODS: Retrospective chart review. RESULTS: A 60-year-old man presented with a painful submucosal lesion of the tongue base. Computed tomography showed an infiltrative soft-tissue mass involving the left base of the tongue. Operative biopsy revealed plexiform neurofibroma. Because of the patient's operative risk and the potential morbidity of surgical resection, he was treated with three-dimensional conformal radiotherapy (3DCRT). His treatment was accomplished using a five-field arrangement treating exclusively the mass lesion to a total tumor dose of 60 Gy. After treatment, the patient's tongue pain resolved, and he noted minimal transient xerostomia. Serial follow-up radiographic examinations showed the base of tongue mass to be slightly smaller 4 months after treatment. The most recent follow-up magnetic resonance image reveals a further decrease in size of the mass. The patient is now over 3 years out from treatment. CONCLUSIONS: Solitary plexiform neurofibroma of the tongue base is a rare tumor. These benign neoplasms are usually treated with either observation or surgical excision. This case demonstrates that, when significant symptoms necessitate active management, these lesions may be successfully treated with minimal morbidity using 3DCRT. The ability of this technique to deliver a conformal radiation dose to the tumor volume while sparing the surrounding normal tissues may expand the application of radiotherapy in the treatment of these benign lesions of the head and neck.

Biopsy↗
PubMed2002

Netting operation to control neurofibroma of the face.

When neurofibroma involves the face, it can lead to a most disfiguring, destructive, and debilitating condition. Because of the unique aesthetic and functional properties of the face, the surgeon might hesitate to remove all of the involved soft tissues and facial nerves in a radical procedure. Involvement of the craniofacial skeleton makes the treatment more difficult and complex. The treatment usually consists of excising the symptomatic lesion, and often these lesions are incompletely corrected. Regrowth after partial excision is frequent and leads to recurrence of deformity. The force of gravity plays a major role in the appearance of deformity, leading to facial tissue drooping and bulging as the mass grows and gains weight. The aim of this operation was to contain the residual mass after excision within a tight net against the force of gravity, thus limiting and preventing drooping or bulging of the facial soft tissue while preserving muscles and nerves associated with facial expression. Polytetrafluoroethylene (Teflon) mesh, used as a net, was evaluated as a replacement material for subcutaneous tissue and a substitute for superficial fascia destroyed by tumor infiltration. The mesh was suspended in the superior-posterior direction, capturing the residual mass like a net capturing fish. From 1989 to 1999, a total of eight patients underwent the netting procedure after partial excision of neurofibroma of the face. The follow-up period was 3 to 10 years. The use of Teflon mesh proved to be compatible with the surrounding tissue, endurable at follow-up, and consistent with expectations. Follow-up computed tomographic scans revealed no further visible tumor growth, and the area was contained under the mesh net with satisfactory postoperative facial contour. The advantages of this procedure are the avoidance of radical excision of facial soft tissue, preservation of remnant facial expression, and prevention of progression of facial dysmorphism.

Adolescent↗
PubMed2003

Quadriplegia in a 10 year-old boy due to multiple cervical neurofibromas.

STUDY DESIGN: A case report of a child with quadriplegia as a result of neurofibromatosis type I who had cervical laminectomy for spinal cord decompression followed by occipitocervical instrumentation is described. OBJECTIVES: To describe the consequences of severe neurofibromatosis type I and an effective surgical technique of occipitocervical instrumentation. SUMMARY OF BACKGROUND DATA: Neurofibromatosis type I is one of the most commonly inherited genetic disorders in the human population. Extensive intraspinal involvement by neurofibromas can cause significant distortion of normal spinal structure as well spinal cord compression. Extensive laminectomy (with subsequent risk of postsurgical kyphosis) is often required for adequate decompression of the spinal cord. METHODS: The clinical and radiographic presentation of a child with severe neurofibromatosis type I resulting in quadriplegia is described. The severe neurologic deficit was caused by compression of the spinal cord by intraspinal neurofibromas. Extensive laminectomy was required to adequately decompress the spinal cord. Occipitocervical fusion from the occiput to C6 was done to stabilize the spine and prevent future kyphosis. RESULTS: Decompression of the spinal cord led to complete neurologic recovery, and instrumentation of the cervical spine was successful in preventing the development of postlaminectomy kyphosis in this pediatric patient. CONCLUSIONS: The reported case emphasizes the need for treating acute neurologic symptoms caused by spinal cord compression in neurofibromatosis type I as well as addressing the future risk of spinal deformity following laminectomy.

Cervical Vertebrae↗
PubMed2005

Radiotherapy and radiosurgery for benign neurofibromas.

The purpose of this study was to evaluate the efficacy of radiotherapy (RT) and stereotactic radiosurgery (SRS) for neurofibromas. We studied 4 patients treated with RT (3 patients) or SRS (1 patient) and followed from 1.7 to 14.8 years. The tumor remained locally controlled in all patients. No significant complications related to treatment were observed. RT and SRS are likely to locally control neurofibromas in patients who require treatment and are not good candidates for complete resection.

Adult↗