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Neurofibroma of the palatal mucosa. A case report.

Neurofibromas have not been reported in the periodontal literature. In this case report, a 27-year-old female presented with a complaint of a lump in the maxillary left palatal tissue; periodontal evaluation revealed a mass 15 x 8 x 4 mm on the palatal mucosa. After removal, the region healed without recurrence. The patient was referred to her physician for a physical, and no evidence of neurofibromatosis was found elsewhere, suggesting that this case represented an example of an isolated oral neurofibroma lesion.

Adult↗

Presacral solitary giant neurofibroma without neurofibromatosis type 1 presenting as pelvic mass--case report.

A 35-year-old woman presented with a solitary neurofibroma in an unusual presacral location without neurofibromatosis manifesting as bilateral chronic sciatica for 2 years. She was initially considered as having a giant right ovarian mass, but was referred with a prediagnosis of solitary giant sacral nerve sheath tumor. The initial differential diagnosis was based on neuroimaging. A right-sided J incision with the extraperitoneal approach provided good exposure and handling of the tumor bed. Almost total excision without neurological deficit was possible. The histological diagnosis was neurofibroma. Benign retroperitoneal neural sheath tumors in patients without von Recklinghausen's disease are quite rare. Intrapelvic tumors are often diagnosed at a later stage. Neuroimaging is very helpful to delineate this unusual site and the extent of tumor development, and to determine the appropriate surgical intervention. A clear understanding of retroperitoneal anatomy is essential for safe removal of such tumors. Complete resection is preferred to prevent local recurrence and malignant transformation. Although root section is inevitable, neurological deficit is unlikely.

Adult↗

Neurofibroma in the median nerve treated with resection and free nerve transplantation. Case reports.

The commonest tumours of the peripheral nerves are neurolemomas and neurofibromas, both arising from the nerve sheath. Both tumour types are described. It is established that the neurofibroma becomes malignant in about 10% of cases and that removal involves resection of the nerve. Radical resection is nevertheless recommended and the defect in the nerve may be overcome by free nerve transplantation. Two cases are reported.

Adolescent↗

Bilateral posterolateral approach to mirror-image C-2 neurofibromas. Report of four cases.

Multiple nerve root tumors are usually present in patients afflicted with neurofibromatosis Type 1. Although rare, upper cervical mirror-image neurofibromas have been reported in the medical literature, and their surgical management has been addressed in several reports; however, little has been mentioned or is known regarding upper cervical or craniocervical stability following resection of these tumors. In this report the authors describe four cases of large mirror-image C-2 neurofibromas resected in two stages via the posterolateral approach. One patient presented with acute neurological deterioration after a biopsy sample had been obtained, whereas the other three presented with gradual onset of lower-extremity weakness over several months. The time interval between the first and second decompressive surgery ranged from 10 days to 12 weeks. There were no surgery-related complications, and all patients recovered motor function in their extremities. During a follow-up period of 16 to 36 months, there was no clinical or radiological evidence of upper cervical spine instability. Although the series is too small to draw any definitive conclusions, in the authors' experience the posterolateral approach provides a direct route for the successful surgical treatment of bilateral craniocervical nerve root tumors without destabilizing the upper cervical segments.

Adult↗

Cervical intramedullary neurofibroma.

Intramedullary spinal neurofibroma is extremely rare. A case of cervical intramedullary neurofibroma is presented in a 21-year-old man. Only 20 case have been previously reported in the literature. The site of origin is discussed with various hypotheses.

Adult↗

[Extratesticular intraescrotal neurofibroma: case report].

OBJECTIVES: We report a rare case of intrascrotal neurofibroma in an adult patient not affected by neurofibromatosis (NF1). METHODS: Clinical diagnosis of asymptomatic right scrotal lipoma and left symptomatic inguinal hernia carried the patient to the surgical treatment. After the first surgical step of left hernia repair, we proceeded to the excision by an inguinoscrotal approach of a voluminous whitish neoplastic mass occupying the whole left scrotum and not involving homolateral testis. RESULTS/CONCLUSIONS: The histopathologic diagnosis was of neurofibroma, a benign neoplasm of peripheral nerves which rarely occurs at genital level.

Genital Neoplasms, Male↗

Peripheral nerve tumors. Large neurofibroma of the foot.

A neurofibroma of the foot is an uncommon finding and may be associated with von Recklinghausen's disease. A case of a large, solitary neurofibroma is presented with no apparent systemic involvement on history and physical examination. The surgical approach and perioperative treatment for this lesion is presented, and a review of peripheral nerve tumors and neurofibromatosis. A tumor of this size poses many unforeseen problems: large dead space, wound dehiscence, and infection caused by hematoma formation. Preoperative magnetic resonance imaging to determine position, depth, and involvements of vital structures is invaluable. The lazy S incision allows for greater exposure with less skin tension from retraction, thus reducing the risk of wound dehiscence. Careful dissection prevents cutting or injuring of underlying atrophied tissue. Two years after surgery, the plantar muscle has returned to normal strength and size and only a slight amount of sensory deficit exists. His gait is normal, without limp, and with normal heel-to-toe ambulation. This patient's life-style has returned to normal with no alterations.

Aged↗

[Neurofibroma plexiforme of the small pelvis: a case report].

There are reports of rare cases of nerve tissue tumors of the small bowel, ovary, urinary bladder or retroperitoneal space. We report a case of neurofibroma plexiforme of pelvis minoris in 42-year-old woman. The first diagnosis was adnexal tumor. Laparotomy was performed and a big, solid tumor of left parametrium was found. The histology of the tumor was described as neurofibroma plexiforme. There was also an inflammatory tumor of appendix involving right Fallopian tube. The described by other authors cases suggest, that diagnosis of nerve tissue tumors in pelvis minoris is very difficult. Surgery is an adequate way of final diagnosis and management.

Adult↗

[Neurofibroma with mucus-producing glands. Report of a case and literature review].

INTRODUCTION: Most tumors of peripheral nerve sheaths containing glands are malignant tumors associated with Von Recklinghausen's disease. CLINICAL CASE: A 39 year old man consulted with a tumour on a finger of the right hand, which was not painful, and was slow growing. There was no past history of neurofibromatosis. Histological study showed a tumour of the peripheral nerve sheath, a benign type of neurofibroma containing glands. Immunohistochemical techniques confirmed that it had the stroma of a Schwannoma with well-defined glandular epithelial elements. CONCLUSIONS: A neurofibroma with glands is considered to be a rare type of divergent differentiation, and of considerable interest to pathologists, since it must be differentiated from other tumoral lesions. Immunohistochemical study is very useful for this.

Adult↗

CO2-laser in the removal of a plexiform neurofibroma from the eyelid.

We present a child with neurofibromatosis type I (NF-I) who underwent excision of a large upper lid neurofibroma using a CO2-laser. Plexiform neurofibromas are notorious for their bleeding tendency and the inability to achieve complete surgical excision. The CO2-laser is an adjunct to achieving better hemostasis and delineation of the tissue in the absence of natural tissue planes caused by the tumor's diffuse mode of growth. Using the CO2-laser achieves better cosmesis, while reducing operation time and limiting complications.

Child↗

[Gastric neurofibroma--still a diagnostic problem].

We report a case of gastric neurofibroma encountered in 41-year-old woman who complained of dyspepsia and physical examination revealed palpable mass in her abdomen. It was not possible to determine the nature and origin of the tumor by radiological and endoscopic investigations. At laparotomy the tumor was found to be pendiculated and growing extramurally from the anterior wall of the stomach. Wedge gastric resection, including the mass, was performed. Histological examination revealed a spindle cell gastric tumor, immunohistochemically differentiated as a neurofibroma.

Adult↗

Concomitant vagal neurofibroma and aplasia of the internal carotid artery in neurofibromatosis type 1.

We report the case of a patient with neurofibromatosis type 1 who had both aplasia of an internal carotid artery (ICA) and a vagal neurofibroma. To our knowledge, this is the first report in the literature of the simultaneous presence of these two rare disorders in a single patient. We believe that this is also the first report of an absence of an ICA in a patient with neurofibromatosis type 1. The patient was a 19-year-old woman who complained of a slowly growing neck mass. The mass occupied the right parapharyngeal space and upper cervical region. The patient had no other masses on physical examination, but widespread café au lait spots were evident. This led us to suspect the presence of a vagal neurofibroma. The tumor was removed, and pathology confirmed the diagnosis. No intracranial aneurysms were detected on cerebral angiography.

Adult↗

Diffuse neurofibroma of scalp.

Diffuse neurofibroma is an uncommon but distinct variety of neurofibroma, usually affecting trunk, head and neck regions of adolescents and young adults. The clinical features, gross macroscopic and histopathological findings are enunciated and the criteria for instituting the preferred modality of treatment for such lesions has been reviewed, stressing upon the need to exclude the neurofibromatoses preoperatively.

Adult↗

Solitary intraosseous neurilemmoma of the tibia: review of intraosseous neurilemmoma and neurofibroma.

This is a case report of a 59-year-old woman with a neurilemmoma involving the tibia, a site not previously reported for this tumor. Neurilemmoma, a benign neoplasm which rarely involves bone, arises from the Schwann cells in the nerve sheath. It is a distinct pathological entity separate from solitary neurofibroma, and arises from the connective tissue coats of nerves, but which also involves bone. The incidence of the 2 types of nerve sheath tumors is obscured by the tendency of some authors to use the terms interchangeably. Forty-eight cases of true intraosseous neurilemmoma have been reported in the literature. Of 18 cases of solitary intraosseous neurofibroma primary in bone, all were in the jaw and 6 were malignant. The diagnosis of intraosseous neurilemmoma may be missed because of its rarity and roentgenologic appearance of cystic bone disease which is not distinctive except that it resembles other commonly encountered bone lesions. A positive diagnosis is based only on the microscopic pathology. Complete local resection generally produces an excellent result without recurrence.

Bone Neoplasms↗

[Laryngeal neurofibroma. A case report and literature review].

Neurofibroma is a rare pathology on the larynx. We distinguish two types of neurofibromas: plexiform and not plexiform in which the difference is at the cytoarchitectural level. The plexiform type is usually associated to the Von Recklinhausen disease, also know as neurofibromatosis type 1. They usually affect the supraglottic structure, and more frequently they are described in aritenoids, ariepiglottic folds, and posterior commissure. We present a case located at the glottic level of plexiform type, with a postsurgical follow-up of five years. We make a revision of the aethiopathogenesis, evolution and prognostic of these pathology in the bibliography.

Adult↗

Aberrant CpG island methylation in neurofibromas and neurofibrosarcomas.

Aberrant methylation of the promoter CpG island of human genes is an alternative gene inactivation mechanism that contributes to the carcinogenesis of human tumours. We have determined the methylation status of the CpG island of 11 tumour-related genes (RB1, p14ARF, p16INK4a, p73, TIMP-3, MGMT, DAPK, THBS1, caspase 8, TP53 and GSTP1) in 18 neurofibromas (including one plexiform neurofibroma) and three neurofibrosarcomas, as well as two non-neoplastic peripheral nerve sheath samples, using methylation-specific polymerase chain reaction. The series included sporadic and neurofibromatosis type 1-associated tumours. The incidence of aberrant methylation in the tumour samples was 52% for THBS1, 43% for MGMT, 33% for TIMP-3, 19% each for p16INK4a and p73, 14% for RB1, 5% for p14ARF, and 0% for DAPK, caspase 8, TP53 and GSTP1. No methylation of these genes was detected in the two samples of non-neoplastic peripheral nerve sheath. All but three samples in the study displayed aberrant methylation in at least one of the studied genes, and there was no correlation between methylation status and the patients' clinical parameters. These findings suggest that methylation of some tumour-related genes may play a significant role in the tumourigenesis of neurofibromas/neurofibrosarcomas.

Adult↗