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At least 217 records · Page 12Linked to original sources
PubMed2006

Hemimacroglossia caused by isolated plexiform neurofibroma: a case report.

OBJECTIVES: The present case is a diffuse isolated plexiform neurofibroma of the tongue that was not associated with neurofibromatosis that we treated with intraoral surgery. STUDY DESIGN: A case report. METHODS: We present a 5-year-old girl with isolated plexiform neurofibroma of the tongue with infiltration of the tongue base and review treatment approaches for this very rare tumor. RESULTS: An intraoral approach appears to be a good alternative method that allows both an acceptable exposure for total excision and limited postoperative sequelae. CONCLUSIONS: Isolated plexiform neurofibroma of the tongue is very rare. It causes a massive macroglossia that is progressive with advance of years and disturbs the patient's speech, occlusion, and tongue movements. Involvement of the tongue may be associated with infiltration into the deep structures of the neck by the tumor, thereby presenting considerable operative difficulties.

Biopsy, Needle↗
PubMed1996

Mesenteric plexiform neurofibroma: computed tomography appearance.

Gastrointestinal involvement in von Recklinghausen's disease is usually in the form of neurofibromas and leiomyomas. Very rarely, plexiform neurofibromas may be seen involving the bowel wall and myenteric plexus with secondary minor involvement of the mesentery. This report describes the computed tomography morphology of a plexiform neurofibroma extensively involving the mesentery with minor involvement of the bowel wall.

Child↗
PubMed1985

Solitary neurofibroma of the esophagus.

A rare case of solitary neurofibroma located in the esophagus is reported. A large (4.2 X 4.0 X 3.0 cm) submucosal tumor was surgically removed from the midportion of the esophagus of a 64-year-old woman. Light and electron microscopic examination, and immunohistochemistry of S-100 protein in the tumor tissue confirmed neurofibroma. The patient had no evidence of von Recklinghausen's neurofibromatosis. A review of literature failed to find other reports of solitary neurofibroma of the esophagus with reliable histological diagnosis.

Diagnosis, Differential↗
PubMed1986

Perineurial cell tumor and the significance of the perineurial cells in neurofibroma.

The authors attempted to clarify the exact cell components of neurofibroma by immunohistochemical and ultrastructural studies. Materials were randomly selected, 40 cases of neurilemoma and neurofibroma (-tosis) in addition to 2 cases of tumors composed exclusively of perineurial cells and three cases of normal peripheral nerve. The applied markers included antisera of S-100 protein for Schwann cells, blood coagulation factor XIIIa for endoneurial fibroblasts or perineurial cells, and laminin and collagen type IV for the basement membrane. S-100 protein was demonstrated only in normal or neoplastic Schwann cells, but not in perineurial cells. On the other hand, factor XIIIa was often recognized in endoneurial fibroblasts and perineurial cells, but not in Schwann cells. Neurofibroma was basically composed of a mixture of Schwann cells, perineurial cells, and endoneurial fibroblasts, the population of each type of cell differing according to the case and area within a given tumor. Perineurial cell tumor exclusively composed of perineurial cells, though rare, appears to be a definite entity, and its characteristic histological and ultrastructural features were described.

Adult↗
PubMedDate not supplied

Surgical treatment of a left ventricular neurofibroma.

Primary cardiac neurofibroma is a rare occurrence. We describe a case of left ventricular neurofibroma in a 56-year-old woman with Von Recklinghausen disease. Resection of the tumor with concomitant mitral valve replacement yielded a satisfactory clinical result, and histological examination of the resected tissue confirmed benign neurofibroma. The anatomic distribution of the vagus nerve plexus, which penetrates the epicardium and myocardium and courses over the left ventricular subendocardial surface, provides a tissue source from which this neurogenic tumor may arise.

Cardiac Surgical Procedures↗
PubMed1980

Bizarre cutaneous neurofibromas.

Bizarre cutaneous neurofibroma is an uncommon benign neoplasm. Microscopically, it is usually characterized by stellate and polyhedral cells embedded in a myxoid stroma, and less commonly by solid sheets of epithelioid cells. Cellular pleomorphism and mitotic figures are regular features and have resulted in erroneous diagnosis of malignancy. The mucinous material has the staining characteristics of a sulfated mucosubstance, probably chondroitin sulfate B. Terms previously applied to this lesion include nerve sheath myxoma. Pacinian neurofibroma, myxoid neurofibroma, and neurotheceoma.

Adolescent↗
PubMed1977

Incidence of neurofibroma in cattle in abattoirs in New South Wales.

The incidence of neurofibroma in slaughtered cattle in New South Wales during a four-year period is reported. Most of the cases were in adult Hereford females over 5 years old. In 24 of the herds to which neurofibroma cases were traced there was evidence that the disease had previously occurred in the herd. However, the data does not indicate close familial relationship among cases of neurofibroma.

Abattoirs↗
PubMed2001

Plexiform neurofibroma of the submandibular salivary gland in a child.

Plexiform neurofibromas in major salivary glands are rarely described. In the literature, most reported tumours have been present in the parotid gland region. A three-year-old boy with a family history of neurofibromatosis presented with a rapidly growing left submandibular mass. The clinical diagnosis was that of a neurofibroma rather than a primary salivary gland tumour. Resection of the lesion revealed a plexiform neurofibroma involving the submandibular gland. Although these tumours have a neurogenic rather than a salivary gland origin, they must be considered in the differential diagnosis of a salivary gland lesion in a patient with a history of neurofibromatosis.

Child, Preschool↗
PubMedDate not supplied

Management of plexiform neurofibroma of the larynx.

We report the ninth case of plexiform neurofibroma of the larynx, which occurred in a 2-year-old with multiple café au lait spots and obstructive sleep apnea. In discussing this clinical problem, we have attempted to make the following points. A patient's having more than six café au lait spots of greater than 1.5-cm diameter is diagnostic of von Recklinghausen's disease. It is much more difficult to completely excise plexiform neurofibroma than nonplexiform neurofibroma. The association of juvenile xanthogranuloma with von Recklinghausen's disease may be a risk factor for the later development of leukemia.

Child, Preschool↗
PubMed1981

Specific estradiol binding in schwannomas, meningiomas, and neurofibromas.

The cytoplasmic fractions of schwannomas (acoustic neuromas), meningiomas, and neurofibromas were assayed for the presence of estrogen receptors. Specific estradiol binding was detected in 7 of 16 schwannomas, 7 of 10 meningiomas, and 1 of 6 neurofibromas. A nontumorous vestibular nerve was also studied and showed no estradiol binding. In the tumors, the concentration of the estradiol binding sites as estimated by saturation binding analysis covered a wide range of values (21 to 2430 fmol/g of tumor) but, overall, meningiomas contained the highest amount of estradiol binder. A Scatchard plot analysis of one of the schwannoma specimens demonstrated high affinity estradiol binding (Ka = 1.695 X 10(10) M-1). Although there were more females than males in each tumor category, the overall incidence of estradiol binding was similar in males (5 of 11, 45%) and in females (10 of 21, 48%). In 5 cases, progestin binding was also measured and was detected in two meningiomas (both from female patients); one meningioma and two neurofibromas showed no progestin binding. A discussion is presented of the possible role of estradiol in the pathogenesis or modulation of meningeal and Schwann cell tumors as well as in the genetic disorder neurofibromatosis.

Adolescent↗
PubMed1983

Large intramedullary neurofibroma of the conus medullaris: case report.

A case of a very large intramedullary neurofibroma of the conus medullaris extending over 3 1/2 segments is reported. The tumor was successfully treated by midline myelotomy and radical excision with no exacerbation of the preoperative neurological deficit. Intramedullary neurofibroma is extremely rare, accounting for less than 1% of spinal neurofibromas. Myelotomy and radical excision under the operating microscope are recommended in such cases.

Adult↗
PubMed2002

A solitary huge neurofibroma of the soft palate.

Neurofibroma of the soft palate, an extremely rare tumour, is probably not yet reported in the literature. We report the first case of an isolated neurofibroma of the soft palate not associated with von Recklinghausen's disease (VRD), which is also, probably, the first reported case of neurofibroma of the soft palate. The tumour was completely removed from the soft palate after performing tracheostomy, necessitated due to difficulties in intubation.

Female↗
PubMed1975

Scintigraphic diagnosis of acoustic neurofibromas.

Radioisotope brain scanning gave positive results in 57 out of 68 acoustic neurofibromas in three British neurosurgical centres. Acoustic neurofibromas over 2 cm in diameter may be expected to give a positive scintigraphic result, and tumours of this size also erode the petrous bone. Since most are 3-4 cm in diameter, correlation between the results of petrous radiography and cerebral scintigraphy often permits a specific diagnosis of acoustic neurofibroma to be made. No other radiological investigation may be necessary preoperatively.

Adult↗
PubMed2005

Plexiform neurofibroma of the oesophagus: a mimicker of malignancy.

We report the first case of a plexiform neurofibroma of the oesophagus, presenting with dysphagia in a 67-year-old man known to suffer from von Recklinghausen's neurofibromatosis. The clinical symptoms and radiological findings mimicked malignancy, and raised considerable concern. Numerous investigations failed to elucidate the benign nature of the condition. The diagnosis was finally achieved at surgery and the patient was successfully treated by oesophageal resection. Plexiform neurofibromas, though only found in 20-30% of affected individuals are pathognomonic of von Recklinghausen's neurofibromatosis. This report highlights the tendency of plexiform neurofibromas to grow extensively and encase surrounding structures, thereby mimicking a neoplastic process.

Aged↗
PubMed2006

Neurofibroma with psammoma bodies.

Neurofibromas are benign tumours of the nerve sheath. Histologically they vary depending on their contents of cells, myxoid stroma and collagen. A 41-year old male with radicular pain had a tumour involving the posterior chest wall. Microscopically it resulted to be a neurofibroma with abundant psammoma bodies. Although these bodies are very frequent in some neoplasias, to our knowledge they have not been described in neurofibromas to date.

Adult↗
PubMed2005

High frequencies of plexiform neurofibromas, mental retardation, learning difficulties, and scoliosis in Brazilian patients with neurofibromatosis type 1.

A clinical study of Brazilian patients with neurofibromatosis type 1 (NF1) was performed in a multidisciplinary Neurofibromatosis Program called CEPAN (Center of Research and Service in Neurofibromatosis). Among 55 patients (60% females, 40% males) who met the NIH criteria for the diagnosis of NF1, 98% had more than six café-au-lait patches, 94.5% had axillary freckling, 45% had inguinal freckling, and 87.5% had Lisch nodules. Cutaneous neurofibromas were observed in 96%, and 40% presented plexiform neurofibromas. A positive family history of NF1 was found in 60%, and mental retardation occurred in 35%. Some degree of scoliosis was noted in 49%, 51% had macrocephaly, 40% had short stature, 76% had learning difficulties, and 2% had optic gliomas. Unexpectedly high frequencies of plexiform neurofibromas, mental retardation, learning difficulties, and scoliosis were observed, probably reflecting the detailed clinical analysis methods adopted by the Neurofibromatosis Program. These same patients were screened for mutations in the GAP-related domain/GRD (exons 20-27a) by single-strand conformation polymorphism. Four different mutations (Q1189X, 3525-3526delAA, E1356G, c.4111-1G>A) and four polymorphisms (c.3315-27G>A, V1146I, V1317A, c.4514+11C>G) were identified. These data were recently published.

Adolescent↗
PubMed2006

Oral plexiform neurofibroma not associated with neurofibromatosis type I: case report.

An unusual case of isolated plexiform neurofibroma arising in the oral cavity without other clinical manifestations or family history of neuro-fibromatosis-1 (NF-1) is described. The tumor was histopathologically analyzed and an immunohistochemical panel comprising S-100 protein, epithelial membrane antigen (EMA), collagen IV, and CD34 was performed. Typical features of plexiform neurofibroma characterized by enlarged nerve fascicles composed of elongated nuclei and scant cytoplasm cells were identified. Subjacent to the oral epithelium, tactile-like bodies were also detected. On the basis of this report, we would like to emphasize that plexiform neurofibroma can occur in the oral cavity as a benign isolated tumor in patients without other stigmata of NF-1.

Adult↗