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At least 235 records · Page 13Linked to original sources

Long-term outcome after removal of spinal neurofibroma.

Spinal neurofibromas are uncommon, comprising approximately 3% of all spinal tumors. They occur both sporadically and in association with neurofibromatosis 1 (NF1; von Recklinghausen's disease). This study presents the clinical characteristics of 32 patients who underwent surgery for symptomatic spinal neurofibromas. Twenty-two of these patients showed clinical signs of NF1. The patients were typically younger (median age 31 years) than those with spinal schwannomas. The tumors were located mainly in the cervical region and tended to grow both extra- and intradurally. Patients with NF1 were prone to develop new spinal neurofibromas. A life-table analysis showed a reduced survival rate for these patients compared to that of the general population.

Adolescent↗

Plexiform neurofibroma of the cauda equina. Case report.

Plexiform neurofibroma of the cauda equina has been reported only twice previously. The authors report the first pediatric patient in whom such a tumor has been found. A 4-year-old boy presented with low-back pain that radiated bilaterally into the L-4 and L-5 dermatomes. A dermal sinus noted at the midthoracic level was surrounded by a hemangiomatous lesion. Magnetic resonance imaging confirmed the presence of the dermal sinus and revealed a well-defined lumbosacral mass that showed heterogeneous intensity with irregular enhancement. Intraoperatively, a solid mass, which engulfed the entire cauda equina, could not be dissected from the roots. The dermal sinus tract, however, was excised from the thoracic spine. The patient underwent radiotherapy to control the tumor and relieve his pain. Plexiform neurofibromas of the cauda equina are characterized by an insidious and progressive clinical course. The tumor mass may engulf all the roots of the cauda equina. No plexiform neurofibroma of the cauda equina has been reported to be associated with neurofibromatosis Type 1. The authors assume that the thoracic-level dermal sinus observed in this child was an incidental finding.

Cauda Equina↗

[Spinal neurinomas and neurofibromas in Senegal].

Authors present 13 cases of spinal neurinomas and neurofibromas out of 418 non- pottic, non-traumatic medullary compression operated between 1965 and 1995. All cases were histologically documented. Neurinomas and neurofibromas represent 3.1% of all non pottic, non traumatic medullary compression and 12.8% of tumour medullary compression operated in our service. The illness started with radicular pains (6 cases) and spinal ache (5 cases) and most of the time, the medullary compression diagnosis is completed at the spasmodic paraplegia status. No patient has done any C.T. scan but in the other hand a myelography has been completed in all cases, resulting in the blockage of the contrast product which in 4 cases was of an epidural type, and in 2 cases of intra dural type. All patients were operated through a posterior approach, which ended in 10 cases to a total removal, and in 3 cases to a partial one. 7 extradural localizations were noticed and 3 neurinomas, 3 neurofibromas and 1 neurofibrosarcoma were histologically identified. A post operative follow-up was done on 11 patients and a satisfactory result was obtained on 6 of them. In 2 cases, the neurological status remained unchanged. In 2 cases a deterioration occurred and a patient with neurofibrosarcoma passed away.

Adolescent↗

[Endobronchial neurofibroma].

Neurofibroma belongs to the benign tumors and it is categorised as neuroectodermal tumor. Neurofibromas are most frequently found in the posterior mediastinum, their endobronchial localisation is rather rare. Our paper presents two cases of endobronchial neurofibromas with diverse clinical and X-ray symptomatology. Both patients underwent surgery and the etiology of tumours was assessed by histological examination of the preoperative biopsy.

Adult↗

The ultrastructure of peripheral neurofibroma: the role of mast cells and their interaction with perineurial cells.

The authors analyze the ultrastructure of mast cells and perineurial cells when both are present in neurofibroma of the nerve sheath. Samples of pathologic tissue taken from three patients with neurofibroma of a peripheral nerve sheath were analyzed by light and transmission electron microscopy. The observations document the characteristics of the tumor cells (Schwann cells and perineurial cells) as well as the presence of numerous mast cells, typically in close contact with the perineurial cells and never with the Schwann cells. Many electron-dense vesicles were found between the cells; these vesicles are created when the cell membrane of the mast cell buds, and then they come into contact with the adjacent perineurial cell. Endocytosis vesicles are often present in the cytoplasm of perineurial cells. Analysis of these observations led the authors to assume the existence of a metabolic interaction between the two cell type in contact with each other and an active role of the mast cells in the evolution of the tumor. The following two theories are plausible: either the mast cells actively stimulate tumor growth, or they alter the phenotype of the tumor cell. These findings could have interesting clinical applications. The use of treatment protocols which inhibit mast cell activity could, in theory, stop either the proliferation of the neurofibroma or its malignant transformation.

Cell Division↗

[Isolated neurofibroma of the stomach. Case report and review of the literature].

An uncommon case of gastric neurofibroma is described: it was an incidental finding during assessment for abdominal pain, possibly due to pancreatitis, in a 58 year old man, with no sign of von Recklinghausen's disease. The generic diagnosis of gastric wall neoplasia was made by CT scanning; the neoplasm was resected with wedge resection of gastric wall. Histological and ultrastructural examination revealed a neurofibroma. Gastrointestinal stromal tumors are rare occurrence and usually are of smooth muscle derivation: a small percentage arises from nerve sheet, but such a distinction is never sharp. Neurogenic gastric tumors are usually benign and only 10% of von Recklinghausen associated neurofibromas can undergo malignant transformation. Wide excision of the tumor appears therefore the treatment of choice.

Humans↗

Plexiform neurofibroma of the cheek mucosa. A case report.

The case reported deals with a solitary plexiform neurofibroma affecting the cheek submucosa. Neurofibroma is an uncommon tumor which rarely appears in oral cavity but it represents the most common neurogenic tumor. Furthermore, plexiform variety is less frequent. Clinically, oral neurofibromas usually appears as anodyne and asintomatic lesions. Sometimes, they produce nervous compression. In this case, tumor is big but asintomatic. There is no definitive radiologic image. It has association with polyglandular syndromes and phacomatosis. The treatment of choice is excision. There are doubts of the surgical results so that some authors are looking for new non-surgical treatments. The clinical characteristics, epidemiology, diagnosis and treatment are described as soon as a bibliographic revisión.

Aged↗

A case of bathing trunk nevus studded with neurofibroma-like papules.

There is a significant association between bathing trunk nevus and neurofibromatosis. However, not all neurofibroma-like papules detected clinically in cases with bathing trunk nevus may be a neurofibromas and histopathological confirmation is essential for definition of these lesions. We report a 21 year old white male patient with a bathing trunk nevus studded with neurofibroma-like papules of melanocytic nature. Histopathological examination of both papular and flat lesions showed diffuse melanocytes with plentiful eosinophilic cytoplasm and round nuclei in the dermis. Immunohistochemistry showed strong positivity of dermal melanocytes for S-100 protein. The clinical and histopathological findings and positivity of dermal melanocytes for S-100 protein were consistent with bathing trunk melanocytic nevus.

Adult↗

[Melanotic neurofibroma, clinical and histopathologic diagnosis. Case report].

Melanotic neurofibroma is a rare benign tumor, derived from peripheral nerve sheath, whose originality consists in the presence of melanic pigment. The clinical diagnosis is difficult to establish, requiring the histopathological exam to make the difference between melanotic neurofibroma and the other pigmented tumors. Although, sometimes neither the anatomopathological exam can establish the final diagnosis, requiring supplementary studies. Melanotic neurofibroma has a good prognosis and the malignization is rare. The elective treatment is surgical, represented by the complete excision of the tumor.

Adult↗

Simultaneous neurofibroma and schwannoma of the sciatic nerve.

The authors report a case of simultaneously occurring neurofibroma and schwannoma of the sciatic nerve and discuss the complementary aspects of MR and US. The schwannoma was well-defined and showed distal enhancement on sonographic evaluation, whereas the neurofibroma was ill-defined; both tumors were hypoechoic. T1- and T2-weighted MR images revealed similar signal characteristics of the two tumors, but intense enhancement following administration of gadolinium-DTPA distinguished the schwannoma from the neurofibroma.

Female↗

Plexiform neurofibroma of the tongue: a case report of a child.

A three-year-old girl with a lingual plexiform neurofibroma treated by total excision is presented. Despite their occurrence in the head and neck region, neural sheath tumors are rarely encountered in the oral cavity. It is reported that 4-7% of patients affected by neurofibromatosis display oral manifestations. Neurofibromatosis is characterized by café-au-lait spots and cutaneous neurofibromas. Plexiform neurofibroma is said to be indicative of von Recklinghausen's disease (VRD) even though it may be the only manifestation of the disease. Generally, surgical resection represents the treatment of choice and the diagnosis can only be confirmed after histological examination. Affected patients need regular follow-up to detect malignant degeneration, an early recurrence or appearance of other manifestations of VRD.

Cafe-au-Lait Spots↗

Huge plexiform neurofibroma of the head and liver--case report.

Neurofibromatosis (NF) is a hereditary autosomal dominant disorder. Von Recklinghausen first described NF in 1882, which is now classified as Neurofibromatosis 1 (NF-1). NF-1 is the most commonly encountered NF which affects 1 in 4000 persons. Clinical manifestations of NF-1 include: generalized cutaneous neurofibroma, pigmented skin patches (cafe-au-lait spots), pigmented iris hamartoma (Lisch nodules), skeletal abnormally, CNS tumors, etc. The subject of this case study is a young adult male with a huge plexiform neurofibroma involving both the liver and head regions. The head tumor measured 10 x 8 x 3.5 cm3 in size, weighted approximately 180g with overlying hyperpigmented skin and an underlying congenital skull defect. A CT scan and MRI of the head and neck revealed a well defined lobulated tumor and deformed external ear. A abdominal sonogram, CT scan and MRI showed a huge plexiform neurofibroma with liver invasion. Lisch nodules and multiple cafe-au-lait spots were also found. Surgical removal of the head tumor along with an external ear reconstruction was performed. Satisfactory cosmetic results and improved hearing were achieved.

Adolescent↗

[Immunohistochemical and ultrastructural studies on tactile-like corpuscles in neurofibromas].

Morphological characteristics of tactile-like corpuscles (tactoid bodies, pseudomeissnerian corpuscles) which are occasionally present in neurofibromas have already been detailed. However, there has yet been controversy on the cytogenesis of these lamellated structures. Probably, one of the most important problems is whether tactile-like corpuscles are composed of Schwann cells or of perineurial cells. In this study, seven cases of neurofibromas with tactile-like corpuscles were examined by rabbit or mouse polyclonal antisera to S-100 protein which could be regarded, at least in the peripheral nervous system, as a specific marker for Schwann cells. Since recent extensive studies have revealed that S-100 protein is a mixture of two predominant dimeric components with S-100 alpha and S-100 beta subunits, and the S-100 alpha subunit is absent from Schwann cells, tactile-like corpuscles were also examined by a mouse monoclonal antibody (ASA-1) specific for the S-100 alpha subunit. For immunohistochemical analysis, peripheral nerves, Meissner corpuscles and epidermal melanocytes were also examined in parallel. By the immunoperoxidase method using rabbit or mouse polyclonal antisera to S-100 protein, tactile-like corpuscles were intensely stained in sections from all seven cases. Both the flattened cell bodies and the eccentrically located nuclei of their constituent cells were stained. In the background neurofibroma tissue surrounding the tactile-like corpuscles, spindle-shaped cells were stained variably. In three of the seven cases, numerous melanin-containing cells which often surrounded the tactile-like corpuscles were also stained. In normal peripheral nerves, positive staining was confined to Schwann cells and their processes.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

Vascular changes in cutaneous neurofibromas.

A variety of vascular changes has been associated with neurofibromatosis, and morphological alterations of arteries have been described previously. However, little attention has been paid to structural modifications of the microvasculature in neurofibromas themselves. Small vessels of 10 cutaneous neurofibromas, excised from patients with neurofibromatosis, were studied by using transmission electron microscopy and compared with normal skin vessels. The major alterations were: (1) increased thickness of the cytoplasm of endothelial cells which send numerous long processes to the lumen and, in some instances, obliterate it; (2) larger numbers of endothelial cell processes on the abluminal surface which are apposed to pericyte processes; (3) increased amounts of filaments and free ribosomes in the cytoplasm of endothelial cells, and (4) abundant pericytes with bundles of cytoplasmic filaments and many small subplasmalemmal densities. Mitotic figures were not seen in any of the vascular elements. These changes were interpreted to be the result of a generalized malformative process of blood vessels in neurofibromas rather than a consequence of the action of a tumor angiogenesis factor.

Adolescent↗

[Neurofibroma of the tongue: a clinical case].

The Authors report a case of neurofibroma of the tongue, observed by them. Its peculiarities and the differential diagnosis with neurinoma and other affections are discussed, mostly pointing out its rarity, epidemiological data and histogenesis. After a review of the literature concerning these forms, the Authors dwell upon the histopathologic characters of neurofibroma, the peculiarities of the case reported by them, considering the real rareness of an isolated report of neurofibroma, typical element of Recklinghausen's disease.

Humans↗

[Solitary tumors of the vagus nerve in the carotid sulcus: neurofibroma and schwannoma. Apropos of 2 cases].

Neurinoma of the vagus is a rare disease (less than a hundred cases published) and even more exceptional when it develops in the carotid cervical sulcus. Indeed, nevous tumors of the neck are usually situated in a high position, in the lateo-pharyngeal space. Clinical diagnosis is difficult: a chronical enlarged lymph node is the usual suggestion. Nevertheless, two signs, albeit inconsistent are very important: cough and bradycardia on palpation of the tumefaction. Enucleation, when possible, is the best procedure for exeresis, leaving the recurrent fibers intact. But in most cases, the tumor cannot be separated from the nervous fibers and the vagus nerve must be severed at both ends. This results in definitive paralysis of the homolateral vocal cord. Microscopically, it is not always easy to distinguish between neurofibroma and schwannoma because both tumors share a common cytogenetic origin and many intermediary forms are found. Only electronic microscopy can solve litigious cases. The importance of this distinction is not only speculative, a schwannoma assumes a solitary course but a neurofibroma may belong to Recklinghausen's disease. Both schwannoma and the rare solitary neurofibroma of the cervical portion of the vagus nerve can be surgically removed, their prognosis is favorable. nervous tumors of the neck in Recklinghausen's Disease must not be operated on account of possible aggravation.

Adult↗

Malignant change in cutaneous neurofibromas--case reports.

Neurofibromatosis is the most common single gene disorder to affect the nervous system, with an estimated incidence of 1 in 3000 live births. Neurofibromatosis (NF) may be classified into von Recklinghausen NF (NFI) and bilateral acoustic NF (NFII) based on the distribution of lesions. The most common lesion associated with the von Recklinghausen type is the neurofibroma. Various complications are associated with neurofibromatosis, the most feared of which is malignant change in the neurofibroma. This article describes the study of 7 cases of proven malignant change in neurofibromas with regards to presentation, clinical progress and treatment followed by a review of the present literature.

Adult↗

neu proto-oncogene mutation is specific for the neurofibromas in a N-nitroso-N-ethylurea-induced hamster neurofibromatosis model but not for hamster melanomas and human Schwann cell tumors.

Point mutations of the transmembrane domain coding region of the neu proto-oncogene in N-nitroso-N-ethylurea-induced hamster neurofibromas were found at high frequency (93%; 14 of 15). They involved codons 659 as well as 658, the latter not having been reported previously in rat tumors. The mutational change was seen even in the early stage neurofibroma. On the other hand, no mutations were detected in melanomas or Wilms' tumors induced in the same N-nitroso-N-ethylurea-treated animals, even when the melanomas demonstrated extensive schwannian differentiation. Moreover, any human Schwann cell tumors including neurofibroma, schwannoma, and malignant schwannoma did not show the mutation of c-erbB-2 gene (0 of 34), which is homologous to the hamster neu. Since high expression of neu mRNA is evident in the hamster Schwann cell at the late gestational and neonatal stages, transplacental administration of N-nitroso-N-ethylurea is considered to interact directly to carcinogenesis of the hamster Schwann cell through neu gene mutation.

Amino Acid Sequence↗