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1/72. Malignant triton tumor of the head and neck: A case report and review of the literature.

    BACKGROUND: Malignant triton tumor (MTT) is a relatively rare, aggressive tumor comprised of both malignant schwannoma cells and malignant rhabdomyoblasts. Because MTT frequently arises in the head and neck, the otolaryngologist must be aware of the nature of the tumor and its response to various treatment modalities. METHOD: This article reviews the treatment and outcome of all reported cases of MTT arising in the head and neck. CONCLUSIONS: Although statistical analysis is limited by the short duration of follow-up of many patients, complete tumor resection appears to carry an improved chance of survival. Adjuvant radiation and chemotherapy may also improve survival, although a benefit of these therapies was not well demonstrated in this small series.
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ranking = 1
keywords = schwann
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2/72. Spinal melanotic schwannoma: a tumour with poor prognosis.

    AIM: To clarify the prognosis of melanotic schwannoma. This is a rare tumour which is generally considered as a benign lesion, reported in many cases with a short follow-up only. methods AND RESULTS: Five cases of spinal melanotic schwannoma were retrospectively studied. The tumours were examined using standard histological, immunohistochemical and ultrastructural methods. No features of malignancy (high mitotic count, atypia or necrosis) were found in the primary tumours. The follow-up period ranged from 3 to 7 years. Malignant clinical behaviour was clear-cut in four cases: three patients died from metastases to various sites and one presented several discrete spinal tumours of the same type seven years after the first operation. Only one patient presented no recurrence and was free of disease 6 years after initial diagnosis. The review of 57 cases of the literature (including our cases), showed that 15% of the cases had recurrences and 26.3% were complicated by metastasis. Only 53% of the cases followed for more than 5 years, were free of disease vs. 67.5% of the cases with shorter follow-up. Twenty additional cases had no follow-up. CONCLUSION: Appropriate long-term follow-up is required for all melanotic schwannomas, as it may recur or metastasize after more than 5 years, even in the absence of overt malignant histological features.
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ranking = 7
keywords = schwann
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3/72. Surgical treatment of locally recurrent malignant schwannoma of the lower leg.

    This report describes a case of locally recurrent limb malignant schwannoma in a 74-year-old patient. The light microscopy and immunohistochemical findings are presented and current methods of surgical treatment reviewed. We describe treatment using wide local excision with frozen section control of the marginal nerves and present a 3-year follow-up.
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ranking = 5
keywords = schwann
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4/72. Pediatric acoustic schwannoma showing rapid regrowth with high proliferative activity.

    Acoustic schwannoma is a slow-growing tumor and usually occurs in adult patients. We report a rare pediatric case of acoustic schwannoma with high proliferative potential. A 10-year-old boy was diagnosed as having a right cerebellopontine angle tumor. The tumor was subtotally resected. Histological examination revealed a typical acoustic schwannoma with a few mitotic figures. Chromosomal analysis showed no abnormality on the long arm of chromosome 22 associated with neurofibromatosis type 2. The lesion re-grew rapidly as an acoustic schwannoma, necessitating subtotal resection on three occasions and CyberKnife radiosurgery. The immunohistochemical MIB-1 staining indices of the specimens obtained at the first, second, and third operations were 2.3%, 4.6% and 14.7%, respectively. The immunohistochemical proliferative potential of acoustic schwannoma is discussed.
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ranking = 9
keywords = schwann
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5/72. Primary intracranial malignant schwannoma of trigeminal nerve. A case report with review of the literature.

    The authors report a rare case of primary intracranial malignant schwannoma of the trigeminal nerve occuring in a 30-year-old woman without von Recklinghausen's neurofibromatosis (VRNF). The tumour arose from the intracranial part of the left trigeminal nerve, without skull base destruction. The tumour was partially removed operatively, followed by focal 50 Gy irradiation, but unfortunately the tumour showed rapid regrowth. A second operation was attempted, but she died of cerebral infarction soon after operation. Histologically, the original tumour was characterized by the presence of foci of extremely high cellularity, pleomorphism and mitosis in an otherwise typical benign schwannoma. Immunohistochemically, the tumour cells were positive for S-100 protein even in the atypical areas, suggesting the tumour to be of Schwann cell origin. However, the recurrent tumour was composed of undifferentiated spindle cells, which were negative for S-100 protein. Thus, it is possible to consider that the S-100 protein expression could reflect the degree of differentiation of schwann cells. Furthermore, the postoperative irradiation might have exacerbated the malignant progression in the present case.
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ranking = 6
keywords = schwann
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6/72. Malignant schwannoma of the digital nerve in a child. A case report.

    An extremely rare case of malignant schwannoma (malignant peripheral nerve sheath tumor) arising in the digital nerve in the right ring finger of a child is described. The patient was an 8-year-old boy who did not have neurofibromatosis (von Recklinghausen's disease). He noticed a small tender mass on the tip of his right ring finger in 1991. The lesion was excised twice. However, the tumor recurred soon after each surgery. Histologic examination revealed malignant schwannoma. He eventually underwent disarticulation of the ring finger at the metacarpophalangeal joint in 1995. There was no recurrence or distant metastasis at followup in July 1999. Solitary malignant schwannomas can occur in the small peripheral nerves and in the major nerves.
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ranking = 7
keywords = schwann
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7/72. Giant schwannoma of the back.

    We treated a patient with giant schwannoma of the back. The tumor measured 35 x 25 x 12 cm and weighted 1840 g. Histological diagnosis was benign schwannoma. To our knowledge, there is no previous report of such a large schwannoma of the back.
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ranking = 7
keywords = schwann
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8/72. Giant foot schwannoma.

    Schwannoma of the foot is rare; only 12 cases have been reported. A schwannoma is a benign neurogenic tumour derived from schwann cells. The diagnosis is often delayed because the symptoms are mainly those of compression disorders. We describe a 7cm schwannoma of the heel in a 30-year-old man. Ten years earlier a schwannoma was removed from the same site. The recurrent lesion was widely excised and a medial plantar flap was used to repair the heel.
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ranking = 7
keywords = schwann
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9/72. Malignant schwannoma of the trigeminal nerve.

    SUMMARY: We present the MR imaging, CT, and clinical findings of a patient with malignant schwannoma of the trigeminal nerve. Local tumor recurrence is frequent and may be mistaken for lymphatic spread. In this report, we emphasize the natural history of this rare tumor and discuss the importance of imaging in diagnosis and surveillance.
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ranking = 5
keywords = schwann
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10/72. Successful removal of a giant recurrent mediastinal liposarcoma involving both hemithoraces.

    Primary liposarcomas of the mediastinum are unusual tumors. We report herein a case of a 52-year-old woman, who was found to have a mediastinal tumor involving both hemithoraces and radiologically showing non-resectable-invasive features to the adjacent vital structures. She had a history of left thoracotomy for mediastinal schwannoma 14 years previously. The patient underwent an exploratory thoracotomy following a preoperative misdiagnosis of an ancient schwannoma. Complete removal of the tumor was accomplished through a right posterolateral thoracotomy with a subsequent histological diagnosis of a recurrent low-grade liposarcoma. A resectable liposarcoma should be considered in the differential diagnosis of a mediastinal tumor, although radiologically, the tumor presents with invasive features.
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ranking = 2
keywords = schwann
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