Cases reported "Neurofibrosarcoma"

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1/12. Malignant spinal neurofibrosarcoma.

    STUDY DESIGN: A report of a case of metastatic spinal neurofibrosarcoma. OBJECTIVE: To document metastatic neurofibrosarcoma as a cause of spinal cord compression and to review the literature. SUMMARY OF BACKGROUND DATA: Three previously reported cases of metastatic neurofibrosarcoma of the spine were reviewed. methods: The patient's clinical record and radiologic investigations as well as the result of a search of the English literature are reported. Magnetic resonance images, computed tomographic scans, and histology photomicrographs are displayed. RESULTS: paraparesis developed in this patient, due to a posterior extradural thoracic spinal cord compression by a neurofibrosarcoma believed to be metastatic from a neurofibrosarcoma of the femoral nerve. CONCLUSIONS: Malignant spinal metastasis remains a rare complication of neurofibromatosis, with a very poor prognosis.
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ranking = 1
keywords = nerve
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2/12. Rectal neurogenic sarcoma: case report and review of the literature.

    A neurogenic sarcoma without NF-1 was discovered in a 73-year-old woman in the anorectal region, an unusual site for these tumors. The tumor was of high-grade malignancy and deeply located with mesorectal infiltration; it did not originate from a major nerve. We presume an origin from less differentiated neural crest cells and present a review of the literature on the best treatment for these neoplasms.
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keywords = nerve
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3/12. Malignant peripheral nerve sheath tumor of the cauda equina.

    Only one case of malignant peripheral nerve sheath tumor (MPNST) affecting the cauda equina region has been reported earlier. A 32-year-old male with congenital multiple subcutaneous swellings presented with low back pain, progressive paraparesis and bladder-bowel dysfunction. magnetic resonance imaging (MRI) demonstrated a heterogeneously enhancing intradural lesion at L2-L4. At operation, on opening the dura, multiple nodular, firm matted masses attached to the lumbosacral nerve roots were encountered. Peripheral lesions were partially excised. Histopathological exam revealed varied cellularity with necrosis and pleomorphic nuclei suggestive of MPNST. MRI features, pathophysiological characteristics and the literature are reviewed.
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ranking = 499.07237253998
keywords = peripheral nerve, peripheral, nerve
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4/12. 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.
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ranking = 105.614474508
keywords = peripheral nerve, peripheral, nerve
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5/12. Metastatic spinal neurofibrosarcoma.

    Neurofibrosarcomas are rare tumors usually arising in somatic soft tissues or peripheral nerves. Four cases of metastatic neurofibrosarcoma to the spine have been reported before. The current case is unusual because of the presence of two distinct, metachronous spinal metastasis and lung metastasis. A 30-year-old woman with neurofibromatosis and a history of previous neurofibrosarcoma resection presented with back pain. Radiologic evaluation revealed a lytic lesion of the eleventh thoracic vertebra. A transthoracal corpectomy, reconstruction by Harms' cage and posterior instrumentation, and fusion were carried out. After the completion of adjuvant chemotherapy, a solitary pulmonary nodule was detected. Shortly after resection of the metastatic pulmonary nodule, the patient complained of acute onset low-back pain. Radiologic assessment revealed another lytic lesion in the L5 vertebra after 6 months. Again, a corpectomy, anterior and posterior instrumentation, and fusion were carried out. Eight months after the second spinal resection, another solitary pulmonary metastasis was diagnosed and resected. The patient's health status suddenly deteriorated 26 months after the initial spinal metastatectomy, and she died. Though local control can be achieved in more than 80% of the patients with neurofibrosarcoma by wide surgical resection followed by adjuvant chemo- and radiotherapy, most patients die of systemic metastasis. The current patient survived 50 months after the initial resection of a forearm neurofibrosarcoma. Despite achieving local control, she died due to systemic recurrence. Prolonged survival with the help of chemo- and radiotherapy justifies our aggressive surgical strategy for the treatment of spinal metastasis in order to achieve neurologic cure and spinal stability.
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ranking = 99.614474507996
keywords = peripheral nerve, peripheral, nerve
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6/12. Intraparotid facial nerve neurofibromas.

    OBJECTIVES: To provide an awareness of intraparotid facial nerve neurofibroma as a cause of parotid masses and to describe their characteristics and management considerations. STUDY DESIGN: Case report with literature review. methods: The medical records of three patients with intraparotid facial nerve neurofibromas are reviewed, and data concerning the patient's presentations, treatment, and disease course are presented with a review of the world's literature on intraparotid facial nerve neurofibromas. CONCLUSIONS: Tumors arising from the extratemporal course of the facial nerve are quite rare. The tumors arise from schwann cells and include the schwannoma and the neurofibroma. The overwhelming benign nature of these lesions necessitates a conservative course of treatment. Histological diagnosis should be followed by a limited tumor excision with emphasis on retaining normal facial nerve function. Malignant lesions require wide excision with facial nerve grafting or facial nerve reanimation.
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ranking = 11
keywords = nerve
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7/12. Neurogenic tumors of the mediastinum originated from the vagus nerve.

    Thirty-nine patients with neurogenic tumors observed between 1974 to 1992 were reviewed. There were 32 patients with neurilemoma, one with neurofibroma, five with ganglioneuroma, and one with malignant neurilemoma. Two cases of neurilemoma originated from the vagus nerve, which is very rare. Surgical resection is recommended, not only to confirm the nature of the lesion, but also to prevent further growth and compression on adjacent structures. For benign encapsulated neurogenic tumors, resection is curative.
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ranking = 5
keywords = nerve
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8/12. Congenital plexiform neurofibroma with a sarcomatous nodule in a three month old child.

    Congenital plexiform neurofibroma is regarded as pathognomonic of neurofibromatosis (NF) especially when it is large and involves a major nerve trunk with changes of elephantiasis neuromatosa. Only very rarely malignant changes have been reported in cases who have NF for less than 5 years. 'Borderline' lesions are seen especially in patients with NF. In such cases criteria of mitotic activity should be utilized in establishing the diagnosis of malignancy. A case of 3 month child with congenital plexiform neurofibroma involving neck with elephantiasis neuromatosa with sarcomatous nodule has been described in the present article. The criteria for malignancy in nerve sheath tumors have also been discussed.
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ranking = 2
keywords = nerve
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9/12. cytogenetic analysis of a malignant triton tumor and a malignant peripheral nerve sheath tumor and a review of the literature.

    Cytogenetic analyses of malignant peripheral nerve sheath tumors (MPNST) and malignant triton tumors (MTT) are few to date. Two separate triton tumor specimens from one patient and a MPNST specimen from another patient, both with peripheral neurofibromatosis (NF-1, von Recklinghausen disease), showed complex near-triploid complements and partial deletion of the short arm of chromosome 1. Notably, a structural abnormality of chromosome 17 was detected in the MPNST, and loss of chromosome 22 was detected in the MTT. The genes for peripheral neurofibromatosis (NF-1) and central neurofibromatosis (NF-2) have been mapped to these two chromosomes respectively.
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ranking = 512.60453007838
keywords = peripheral nerve, peripheral, nerve
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10/12. Bone scans in neurofibromatosis: neurofibroma, plexiform neuroma and neurofibrosarcoma.

    Neurofibromatosis type 1 or von Recklinghausen's disease is one of the most common autosomal dominant genetic disorders. Between 29% and 77% of patients may suffer from a wide range of skeletal abnormalities and, thus, patients with neurofibromatosis frequently undergo skeletal scintigraphy, at which time the common peripheral nerve soft-tissue tumors that occur in this syndrome (neurofibromas, plexiform neuromas and neurofibrosarcomas) may be demonstrated. methods: Single or multiphase 99mTc methylenediphosphonate (MDP) bone scans were performed in five patients with neurofibromatosis as part of their clinical evaluation. RESULTS: We imaged neurofibrosarcomas in three patients, cutaneous neurofibromas in one patient and a plexiform neuroma in one patient. CONCLUSION: Single- or multiphasic bone scans may localize common soft-tissue tumors in neurofibromatosis.
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ranking = 99.614474507996
keywords = peripheral nerve, peripheral, nerve
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