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At least 541 records · Page 30Linked to original sources
PubMed1984

Pigmented storiform neurofibroma.

A case of pigmented storiform neurofibroma is presented. The lesion was present in the right loin of a 35-year-old Japanese woman. Grossly, a round, slightly elevated, approximately 1.5 cm in diameter tumor showed a red-purplish coloration of the central part of the overlying skin without ulceration. The characteristic storiform pattern and scattered melanin-containing cells were the relevant features for histological diagnosis. To our knowledge, our case is the second reported case in Japan. The histogenesis and its relationship with tumors of neural crest origin is briefly discussed.

Adult↗
PubMed1990

Formation of nerve twig-like nests and schwannoma in an unusual case of neurofibroma.

A case of unusual neurofibroma in an 18-year-old Japanese male is reported. The histology of the tumor was characterized by nerve twig-like nests intermingled with fascicular bundles. In the central portion, the tumor also contained a lobular lesion showing features characteristic of schwannoma. Immunohistochemically, the tumor cells in both the nests and the lobular lesion demonstrated a mostly positive reaction for S-100 protein. S-100 protein-positive and -negative cells were observed in equal numbers in the fascicular bundles surrounding the nests. This unusual nerve sheath tumor in which the S-100 protein-positive cells form both nerve twig-like nests and lobular schwannoma has not been reported previously. The origin of the S-100 protein-positive cells in the two lesions is also discussed.

Adolescent↗
PubMed1988

Supratentorial neurofibroma unassociated with cranial nerves.

A rare case of a solitary neurofibroma occurring in a 42-year old female is described. It was attached to the lateral wall of the cavernous sinus. The literature of previously described intracranial nerve sheath tumours not obviously associated with cranial nerves is briefly reviewed.

Adult↗
PubMed1977

Pigmented storiform neurofibroma.

A case of pigmented storiform neurofibroma (PSN) is presented. The lesion was present in the shoulder of a 7-year-old black girl and recurred after 10 years. The characteristic storiform pattern and melanin production are the relevant features for histologic diagnosis. The histogenesis and taxonomic position of this lesion is discussed with emphasis on its relationship with dermatofibrosarcoma protuberans (DFSP) and cellular blue nervus.

Adolescent↗
PubMed1972

Vertebral artery occlusion by a cervical 'hour-glass' neurofibroma.

A case of total vertebral artery occlusion by a cervical `hour-glass' or `dumb-bell' neurofibroma is reported. To the author's knowledge this is the first reported case in the English literature. A causal relationship between the arterial occlusion and the patient's symptoms has been postulated. The angiographic study of these patients is further emphasized to enhance safer and more effectual removal of these lesions.

Cerebral Angiography↗
PubMed1973

Intraneural neurofibromas involving the posterior interosseous nerve.

Two patients presented with a posterior interosseous nerve palsy with supinator weakness. In each case an intraneural neurofibroma was found proximal to the supinator tunnel with no other stigmata of von Recklinghausen's disease. Both tumours showed a whorl-like histological pattern that has been previously confused with onion-bulb structures in hypertrophic polyneuropathy. Electron-microscopy was useful for the pathological diagnosis. The prognosis and treatment of the lesions are discussed.

Adolescent↗
PubMed1992

Giant pre-sacral neurofibroma.

We report a case of pre-sacral neurofibroma diagnosed on a routine medical examination. The pathological features and management criteria of these rare tumours are discussed.

Humans↗
PubMed2001

Intramural neurofibroma of the trachea treated by multiple stents.

The case history is presented of a patient in whom an intramural tracheal neurofibroma developed, causing severe airway stenosis. The patient was treated with multiple stents over a period of 5 years because of progression of the disease and associated airflow limitation. Clinicians should be aware of this rare complication of neurofibromatosis.

Bronchoscopy↗
PubMed1978

Neurofibroma of the trachea: a case report.

A tracheal tumour was discovered by radiology in a 36-year-old man with haemoptysis, bronchitis, and dyspnoea. The tumour was situated 5 cm below the vocal cords. A frozen section of a bronchoscopic biopsy specimen confirmed it to be a neurofibroma. Resection was performed endoscopically.

Adult↗
PubMedDate not supplied

Neurofibroma in the mastoid segment of the facial canal.

Neural tissue-derived facial nerve tumours usually present as neurinomas. We describe the extremely rare occurrence of a histologically verified neurofibroma primarily arising in the mastoid segment of the facial canal in a patient not fulfilling diagnostic criteria for neurofibromatosis. The tumour showed evidence of perineural growth into the jugular foramen, as suggested by cross-sectional imaging and intraoperative findings.

Facial Nerve Diseases↗
PubMed1991

Neurofibroma of the clitoris. A case report.

We report an unusual case of clitoral hypertrophy due to neurofibromatosis of the external genitalia. Because of no definite skin signs or other nervous or osseous symptoms such as are commonly found in neurofibromatosis, the patient was at first suspected of being a case of hermaphroditism. Finally, the disorder was properly diagnosed after laparotomy followed by clitorectomy. This patient seems to be the 12th case of clitoral neurofibroma reported in the English literature.

Child, Preschool↗
PubMedDate not supplied

Pathology of jugular foramen neurofibroma.

Tumors of jugular foramen may closely resemble glomus jugulare tumors clinically and radiographically. A tissue diagnosis is necessary to make a differentiation of these tumors. This conclusion is supported by the findings in a temporal bone from a patient who was diagnosed clinically as having a glomus jugulare tumor 57 years before her death at the age of 84 years. Compression of the 7th and 8th cranial nerves in the internal auditory canal and the 10th and 11th cranial nerves at the jugular foramen represents the mechanism of neural signs produced by a neurofibroma arising in the jugulare foramen. This case further demonstrates that conservative treatment of benign extradural tumors may be compatible with a long and useful life.

Adult↗
PubMed2006

Phase I trial and pharmacokinetic study of the farnesyltransferase inhibitor tipifarnib in children with refractory solid tumors or neurofibromatosis type I and plexiform neurofibromas.

PURPOSE: This pediatric phase I trial of tipifarnib determined the maximum-tolerated dose (MTD), pharmacokinetics, and pharmacodynamics of tipifarnib in children with refractory solid tumors and neurofibromatosis type 1 (NF1) -related plexiform neurofibromas. PATIENTS AND METHODS: Tipifarnib was administered twice daily for 21 days, repeated every 28 days starting at 150 mg/m2/dose (n = 4), with escalations to 200 (n = 12), 275 (n = 12), and 375 (n = 6) mg/m2/dose. The MTD was also evaluated on a chronic continuous dosing schedule (n = 6). Pharmacokinetic sampling was performed for 36 hours after the first dose and peripheral-blood mononuclear cells (PBMCs) were collected at baseline and steady state for determination of farnesyl protein transferase (FTase) activity and HDJ-2 farnesylation. RESULTS: Twenty-three solid tumor and 17 NF1 patients were assessable for toxicity. The MTD was 200 mg/m2/dose, and dose-limiting toxicities on cycle 1 were myelosuppression, rash, nausea, vomiting, and diarrhea. The 200 mg/m2/dose was also tolerable on the continuous dosing schedule. Cumulative toxicity was not observed in the 17 NF1 patients who received a median of 10 cycles (range, 1 to 32 cycles). The plasma pharmacokinetics of tipifarnib were highly variable but not age dependent. At steady state on 200 mg/m2/dose, FTase activity was 30% compared with baseline, and farnesylation of HDJ-2 was inhibited in PBMCs. CONCLUSION: Oral tipifarnib is well tolerated in children receiving the drug twice daily for 21 days and a continuous dosing schedule at 200 mg/m2/dose, which is equivalent to the MTD in adults. The pharmacokinetic profile of tipifarnib in children is similar to that in adults, and at the MTD, FTase is inhibited in PBMC in vivo.

Adolescent↗
PubMed2003

Phase I study of thalidomide for the treatment of plexiform neurofibroma in neurofibromatosis 1.

An open-label phase I trial of thalidomide (TL) in 20 patients with neurofibromatosis 1 (NF1) treated symptomatic plexiform neurofibroma (PNF). TL was well tolerated in doses up to 200 mg/d. Adverse reactions included transient somnolence in four, evanescent rash in two, and reversible mild peripheral neuropathy in two patients. Four patients showed less than 25% reduction in the tumor size. TL may have a role in the treatment of PNF and should be explored in a larger controlled study, possibly using higher doses of TL.

Adolescent↗