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At least 127 records · Page 7Linked to original sources
PubMed2002

Immunoprofile of MITF, tyrosinase, melan-A, and MAGE-1 in HMB45-negative melanomas.

A majority of desmoplastic melanomas and some of the other forms of melanomas are S-100 positive and HMB45 negative; this pattern of immunoreactivity is similar to certain nerve-derived tumors such as malignant peripheral nerve sheath tumor. In this study the immunostaining profile of HMB45-negative malignant melanomas was evaluated by a panel of antibodies against markers associated with melanoma and melanocytic differentiation, including microphthalmia transcription factor, tyrosinase, Melan-A, and MAGE-1. Immunodetection was performed on paraffin sections of 22 cases of HMB45-negative malignant melanomas (including 8 spindle cell melanomas, 8 desmoplastic melanomas, and 6 epithelioid melanomas), 8 HMB45-and S-100-positive malignant melanomas, 15 malignant peripheral nerve sheath tumors, 16 schwannomas, and 11 neurofibromas. Of eight HMB45-positive malignant melanomas, all were positive for Melan-A, tyrosinase, and melanocyte-specific transcription factor, and three were positive for MAGE-1. In the 14 HMB-45 negative, nondesmoplastic melanomas, melanocyte-specific transcription factor was positive in 9, Melan-A in 9, tyrosinase in 6, and MAGE-1 in 11. In eight desmoplastic malignant melanomas, MAGE-1 was positive in three, and all other markers were negative. The five markers tested were negative in all but two schwannomas, one with focal melanocyte-specific transcription factor and the other with tyrosinase and weak MAGE-1 reactivity. MAGE-1, melanocyte-specific transcription factor, tyrosinase, and Melan-A are useful markers in the diagnosis of malignant melanocytic lesions when HMB45 is negative. MAGE-1 may be useful in differentiating melanocytic lesions from nerve-derived lesions, but its sensitivity is relatively low. The immunostaining profile of desmoplastic malignant melanomas more closely resembles that of malignant peripheral nerve sheath tumor than that of other types of malignant melanoma. Melanocyte-specific transcription factor is not a useful marker for desmoplastic melanoma.

Antigens, Neoplasm↗
PubMed1997

Recurrent orbital malignant peripheral nerve sheath tumor 18 years after initial resection.

Malignant peripheral sheath tumor (malignant PNS tumor) is a rare, aggressive, and lethal orbital neoplasm that usually arises from sensory branches of the trigeminal nerve in adults. Malignant PNS tumor spreads along nerves to involve the brain, and most patients die within 5 years of clinical diagnosis. The clinical history and examination, imaging studies, surgical procedures, and pathologic specimens of a 23-year-old man with rapid, posttraumatic onset of left orbital proptosis and pain are reported. Biopsy specimens of the tumor were compared with specimens from the same orbit obtained 18 years previously. Histopathology of the left orbital tumor compared with that of an incomplete excision of a tumor 18 years previously in the same orbit revealed both to be malignant PNS tumor. The patient is alive without recurrence 8 months after orbital exenteration and 18 years after initial tumor presentation. This is the youngest age of occurrence and the longest documented survival of a patient with orbital malignant PNS tumor.

Adult↗
PubMed2001

Positron emission tomography in the detection and management of sarcomatous transformation in neurofibromatosis.

Benign neurofibromas undergo sarcomatous transformation in approximately 5% of patients with neurofibromatosis type I. The clinical and radiologic diagnosis of sarcomatous change remains difficult. Positron emission tomography with F-18 fluorodeoxyglucose is a method to assess increased glucose metabolism in malignant tissue such as sarcomas. In this case report, positron emission tomography accurately distinguished malignant from benign neurofibromas. The technique may be useful as a noninvasive screening tool for malignant transformation of neurofibromas.

Adult↗
PubMed1991

Adult peripheral neuroepithelioma in Meckel's cave.

A case of peripheral neuroepithelioma arising from the trigeminal nerve in Meckel's cave is presented. The discussion emphasizes the pathological criteria for the diagnosis of a peripheral neuroepithelioma and the current controversy about the classification of this and related tumors.

Humans↗
PubMed2000

Intraneural perineurioma involving the median nerve.

Intraneural perineurioma is a rare clinical entity, which tends to affect major nerve trunks in the upper extremities. On light microscopy, numerous pseudo-onion-bulb structures having a central clear area are surrounded by concentric layers of eosinophilic elongate cells having spindled nuclei. Immunohistochemistry of concentric cells stains positive for epithelial membrane antigen but negative for S100 protein. Because of the small number of cases, no consensus has been made on proper treatment of this entity. Although none of the patients who have had excision of tumor with nerve grafting have had sensory nerve recovery, we believe each patient should be individualized until more data are available regarding this tumor.

Humans↗
PubMed1995

Perineurioma.

Perineurioma is a rare, probably benign peripheral nerve sheath neoplasm. Perineurial cell origin is most efficiently demonstrated immunohistochemically by positivity for epithelial membrane antigen and negativity for S-100 protein and Leu 7.

Humans↗
PubMed2005

Radiotherapy and radiosurgery for benign neurofibromas.

The purpose of this study was to evaluate the efficacy of radiotherapy (RT) and stereotactic radiosurgery (SRS) for neurofibromas. We studied 4 patients treated with RT (3 patients) or SRS (1 patient) and followed from 1.7 to 14.8 years. The tumor remained locally controlled in all patients. No significant complications related to treatment were observed. RT and SRS are likely to locally control neurofibromas in patients who require treatment and are not good candidates for complete resection.

Adult↗
PubMed2003

The management of perineural spread of squamous cell carcinoma to the ocular adnexae.

PURPOSE: To analyze a series of patients with periorbital perineural spread of squamous cell carcinoma and propose treatment guidelines. METHODS: Retrospective, noncomparative, interventional case series of 17 patients with clinical, radiologic, or histologic evidence of distant perineural spread. Treatment, recurrence, progression, and mortality rates were recorded. RESULTS: Numbness and pain were the most common symptoms, whereas ophthalmoplegia, ptosis, and facial palsy were the most frequent signs. All cases received wide-field radiotherapy to at least 50 Gy. Chemotherapy and surgery (biopsy, debulking, exenteration) were used in selected cases. Disease progression occurred in 6 patients, 4 of whom died. Median disease-free survival in the remainder was 37.5 months. CONCLUSIONS: The diagnosis of perineural spread is largely clinical and may be confirmed with imaging findings in the majority of cases. The role of biopsy is usually complementary but may be essential in some cases. In patients with evidence of distant perineural spread, radiotherapy volumes inclusive of potential antegrade and retrograde spread are recommended. Three-dimensional conformal planning or intensity-modulated radiation therapy assists in minimizing damage to adjacent structures. Synchronous chemotherapy should be considered to potentiate the effectiveness of radiation. The place of surgery in the treatment of perineural spread is palliative.

Adult↗
PubMed2005

Peripheral and cranial nerve sheath tumors.

PURPOSE OF REVIEW: The intention of the authors is to provide the reader with an overview of the recent advances in the diagnosis and treatment of nerve sheath tumors. Vestibular schwannomas, neurogenetic syndromes such as schwannomatosis and multiple isolated neurofibromas, and malignant peripheral nerve sheath tumors are covered in this review. RECENT FINDINGS: Over the last year, literature focusing on different management strategies for patients with vestibular schwannomas dominated the field. Surgical options for this group of patients are changing. Stereotactic radiation is also employed more frequently with promising results. New insights into the biology of peripheral nerve tumor development and growth, including expression of vascular endothelial growth factor by vestibular schwannomas and the role of Notch signaling in malignant transformation of benign neurofibromas have been described. Diagnostic criteria for schwannomatosis, a recently described condition, are being developed. Several cases of multiple isolated neurofibromas and spinal neurofibromas were reported. SUMMARY: Peripheral nerve tumors are classified according to the specific features of cellular differentiation. The most common types include schwannoma and neurofibroma. These tumors can occur sporadically or as manifestations of genetic syndromes such as neurofibromatosis types 1 and 2 or schwannomatosis. The majority of peripheral nerve tumors are benign but malignant transformation does occur. Metastatic tumors can also affect peripheral nerves. The diagnostic modality of choice is magnetic resonance imaging. Positron emission tomography is a useful technique in the presurgical differentiation between benign and malignant peripheral nerve sheath tumors. Treatment is directed towards symptomatic control. Surgery, radiation and, in rare instances, chemotherapy are the major treatment modalities employed.

Cranial Nerve Neoplasms↗
PubMed2007

Epithelioid sarcoma of the median nerve mimicking a peripheral nerve sheath tumour.

We describe a case of epithelioid sarcoma of the median nerve in a 57-year-old woman presenting with symptoms and signs of carpal tunnel syndrome for 2 years. The clinical examination was suggestive of a wrist ganglion compressing the median nerve. Magnetic resonance imaging (MRI) showed a 5 cm x 3 cm mass involving the median nerve in the carpal tunnel and appearances mimicked a benign peripheral nerve sheath tumour. This report illustrates a rare tumour presenting in a rare location and emphasizes the atypical clinical and MRI features that should alert the radiologist to the possibility of a rare sarcoma mimicking a benign peripheral nerve sheath tumour.

Diagnosis, Differential↗
PubMed2004

Intraneural perineurioma involving the ulnar nerve.

Intraneural perineurioma is a rare peripheral nerve sheath tumor consisting of intraneural proliferation of neoplastic perineurial cells. Clinical and pathological findings of a perineurioma involving the ulnar nerve is presented. A 7-year-old girl presented with a 2 year history of weakness and atrophy of the right hand muscles. Physical examination and imaging study revealed a pea-sized tumor in the ulnar side of the right forearm. At surgery, a fusiform swelling of the ulnar nerve was found and an excisional biopsy of the lesion was performed. Light microscopy revealed numerous whorls consisting of concentric layers of spindle cells encircling the nerve fibers. The proliferating cells were immunoreactive for vimentin, epithelial membrane antigen and glucose transporter protein 1 (Glut1), but negative for S-100 protein and CD34. Ultrastructural examination revealed features of perineurial cell differentiation. The current study suggests that Glut1 is a useful marker of intraneural perineurioma.

Biomarkers, Tumor↗
PubMedDate not supplied

Diagnosis of sciatic nerve tumour in two dogs by electromyography and magnetic resonance imaging.

Sciatic nerve tumour was diagnosed in a Staffordshire Bull Terrier cross-bred and a Bichon Frise, both presenting with chronic left hind limb lameness. Neurological examination in each case was consistent with left sciatic nerve deficits and this was confirmed by EMG studies. Rectal examination of both dogs revealed a palpable intrapelvic mass that was not apparent on survey radiographs. A sciatic nerve tumour was identified using MRI in each case. Histological examination of tissue taken from the Staffordshire Bull Terrier cross-bred was consistent with a malignant peripheral nerve sheath tumour.

Animals↗