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At least 559 records · Page 31Linked to original sources

Plexiform neurofibroma of the larynx in a child.

A case of a female child of six years of age with a plexiform neurofibroma of the larynx caused by von Recklinghausen's disease is presented. Laryngeal involvement in neurofibromatosis type 1 (NF1) is rare and only 19 paediatric cases have been reported. The tumour was biopsied and lateral pharyngotomy with supraglottic hemilaryngectomy was performed in order to relieve obstructive symptoms. Problems related to this unusual tumour localization are discussed and a review of the literature is presented.

Child↗

Plexiform neurofibroma of the small bowel infiltrated with metastatic adenocarcinoma.

Neurofibromatosis Type 1 (NF1) is not classically associated with gastrointestinal manifestations although these patients are at increased risk of several GI complications. We describe the ultrasound, CT and barium findings in a patient with NF1 who had a huge benign plexiform neurofibroma of the ileum that was infiltrated with metastatic adenocarcinoma.

Adenocarcinoma↗

Facial plexiform neurofibroma.

Facial plexiform neurofibroma is a relatively rare manifestation of Von Recklinghausen's neurofibromatosis. Two patients with extensive facial tumor involvement are presented, each having undergone wide local resection after multiple previous local excisions had failed to control the tumor and its locally deforming and destructive effects. A discussion of the problem, its etiology, pathogenesis, natural history, prognosis and suggested treatment is presented.

Adolescent↗

Melanotic neurofibroma in a steer.

A melanotic neurofibroma in a steer was investigated histologically, immunohistochemically and ultrastructurally. A very large tumor mass was located in the region of the head and right cheek. The tumor tissue consisted of an admixture of cells resembling Schwann cells and spindle-shaped cells, and they frequently contained melanin granules. Neoplastic Schwann cells were positive for S100 protein, with variation in intensity of staining, but most spindled cells were S100 negative. The tumor cells displayed ultrastructural features similar to those of Schwann cells or perineurial cells. The presence of melanosomes in varying stages of melanization in both cell types suggests that they have a common origin. This is a tumor of neural crest origin showing schwannian and perineurial differentiation, with ectopic production of melanin granules.

Animals↗

Somatostatinoma: collision with neurofibroma and ultrastructural features.

The clinical presentation, histopathology and immunoelectron microscopic features of two cases of duodenal somatostatinoma are described, one of which is a hitherto unreported example of a collision tumour with a neurofibroma. Ultrastructural morphometric immunoelectron microscopy studies revealed the presence of four types of cells in both tumours, but there was no difference in the proportions of these cells between the collision tumour and the non-collision tumour. Neurosecretory granules ranging in size from 255-815 nm were generally larger than those previously reported for somatostatinomas and somatostatin was identified in granules of all sizes across this range. Neither tumour was associated with the somatostatinoma syndrome comprising associated diabetes mellitis, steatorrhoea and cholelithiasis.

Adult↗

Malignant schwannoma of the sciatic nerve originating in a spinal plexiform neurofibroma associated with neurofibromatosis type 1--case report.

A 26-year-old man with neurofibromatosis type 1 (NF1) presented with a giant malignant schwannoma of the sciatic nerve. The differential diagnosis of malignant peripheral nerve sheath tumor (MPNST) was based on clinical, radiological, and histological evidence. The tumor apparently originated in a spinal plexiform neurofibroma. The lesion was resected totally without neural damage to the sciatic nerve. However, the tumor recurred within 2 months. The patient died of unknown factors probably associated with the spinal involvement. MPNST associated with NF1 has a poor prognosis due to recurrence or metastasis despite complete macroscopic removal.

Adult↗

Solitary cervical lymphoma presenting as a neurofibroma.

A patient is described who presented with cervical cord compression. The imaging and operative findings were typical of a neurofibroma of the right third cervical root. However, histological studies confirmed that the tumour was a B-cell lymphoma. Isolated spinal lymphomas can therefore occur and may present as nerve sheath tumours.

Aged↗

Mucosal neuromas and plexiform neurofibromas: an immunocytochemical study.

Mucosal neuromas (MN), a component of multiple endocrine neoplasia (MEN) type IIb, may be confused histologically with plexiform neurofibromas (PN), a component of neurofibromatosis. The ability to distinguish between these two markers for different genetic diseases is crucial, as the risk of development of medullary thyroid carcinoma and pheochromocytoma in affected patients with MEN IIb is great. We studied two cases each of MN and PN by immunocytochemistry (IC). Epithelial membrane antigen (EMA) proved to be the most useful marker. MN consisted of bundles of disorganized and tortuous nerve fibers surrounded by a thickened perineurium that expressed the cellular phenotype EMA(+), S-100(-). PN consisted of enlarged nerve fascicles with a loose myxoid stroma and was EMA negative. Thus, IC highlighted the differing pattern of growth and histogenesis of the proliferating cells in the two lesions and is likely to be especially useful in those lesions with atypical histology.

Adult↗

"Congenital" plexiform neurofibroma of the occipital scalp. Case report.

An occipital scalp tumor first noted in a Nigerian girl during the first month of her life gradually increased in size and 13 years later measured 10 X 16 X 17 cm. A skull film revealed a 2 X 4-cm skull defect underneath the tumor over the lambdoid suture. Complete excision of the tumor was achieve although it was adherent to the dura of the transverse sinus. It was histologically confirmed to be plexiform neurofibroma. There were no other signs of van Recklinghausen's neurofibromatosis.

Adolescent↗

Tapia's syndrome caused by a neurofibroma of the hypoglossal and vagus nerves: case report.

Tapia's syndrome is characterized by unilateral paralysis of the tongue and vocal cord, and is caused by a lesion of the 10th and 12th cranial nerves below the nodose ganglion, without involvement of the pharyngeal branches of the 10th nerve. The authors report the case of a 25-year-old man who presented with a 4-year history of progressive glossolaryngeal paralysis. Operation through the right laterocervical region allowed complete removal of a neurofibroma involving the 10th and 12th nerves at their crossing below the nodose ganglion.

Adult↗

Paramedian transmuscular access to C-3 dumbbell-type neurofibroma without paravertebral muscle dissection from the spinous process or facetectomy. Technical note.

The authors devised a paramedian transmuscular approach to the C2-3 facet joint that enabled total removal of C-3 dumbbell-type neurofibroma; dissection of the paravertebral muscles from the spinous process was not required and the facet joint was preserved. Only splitting or retraction of the paravertebral muscles was necessary. The anatomical features and procedures involved in muscle splitting are described.

Adult↗

MRI of peripheral neurofibromas in children.

Magnetic resonance imaging was performed as a preoperative examination in three children with neurofibromatosis and soft-tissue plexiform neurofibromas. In all three patients, MRI showed more widespread soft tissue involvement than was suspected clinically including extension towards the spine in each case. Patient management was changed in each case after MRI was performed.

Arm↗

Abdominal wall neurofibroma presenting as an inguinal hernia.

Inguinal hernias are a common cause of abdominal wall pain and are the most common abdominal wall abnormality. They can usually be differentiated from other abnormalities by history and physical examination. Occasionally, the diagnosis may be difficult with very small or very large lesions. The following case report describes an abdominal wall neurofibroma presenting as an inguinal hernia in a young, active duty, male soldier with previously undiagnosed neurofibromatosis.

Abdominal Neoplasms↗

[Intrathoracic neurofibroma originating in the left vagus nerve].

A 20-year-old man was admitted because of an abnormal mass shadow on chest X-ray film. Computed tomography (CT) and magnetic resonance imaging (MRI) disclosed a mass lesion in the superior portion of the left mediastinum. CT scans showed a well-defined mass with low density. Axial MRI rendered the mass lesion with intermediate signal intensity on T1-weighted images and high signal intensity on T2-weighted images. The preoperative diagnosis was bronchogenic cyst. Video-assisted thoracic surgery revealed that the tumor originated in the truncus of the left vagus nerve. The resected tumor was 90 x 24 x 18 mm in size. The postoperative course was uneventful and hoarseness did not develop. The pathologic diagnosis was benign mediastinal neurofibroma without von Recklinghausen's disease. Such cases are extremely rare in the Japanese literature.

Adult↗