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Results for “Neurofibrosarcoma”

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At least 73 records · Page 4Linked to original sources

Hyperinsulinemic hypoglycemia associated with a neurofibrosarcoma.

A patient with a posterior mediastinal malignant neurofibrosarcoma and recurrent episodes of severe hypoglycemia was demonstrated to have basal hyperinsulinemia on repeated study. Despite hypoglycemia, there was no glucagon response to aminogenic stimulation. No immunoreactive insulin was detected on extraction of the tumor and no secretory granules of beta type were seen on electron microscopic examination of tumor tissue. Following tumor removal, plasma glucose and insulin levels returned to normal. Alpha cell function improved. These findings suggest stimulation of pancreatic beta cell secretion and possible inhibition of alpha cell response by an extrapancreatic tumor.

Aged↗

Neurofibrosarcoma as a complication of von Recklinghausen neurofibromatosis.

Neurofibrosarcoma (NFS), which is rare in the general population, occurs with excessive frequency among patients with von Recklinghausen neurofibromatosis (NF-1). Among 693 patients with NF-1 enrolled in the Baylor NF Program, 24 patients (3.5%) developed NFS or one of its variant forms. Nine of the twenty-four patients developed NFS within 3-94 months after enrolling in the Program for other reasons. Focus on these 9 patients alone suggests that the relative risk for patients with NF-1 developing NFS is 10-10,000 times greater than for the general population. Also, in NFS cohorts, patients with NF-1 are almost twice as likely to be under 30 years of age.

Adolescent↗

Neurofibrosarcoma of the mandible.

A case has been reported to illustrate the apparent transition of a neurofibroma into a neurofibrosarcoma. Biopsy of the inferior alveolar nerve, proximal to the lesion, was used to determine the extent of tumor extension. En block excision of the tumor was performed. The patient has been without evidence of recurrence for approximatley 3 1/2 years. Bone graft reconstruction has been completed and full function has been restored to the mandible.

Adult↗

[Sciatica. Neurofibrosarcoma of the thigh].

A 61-year-old patient had complained of sciatica in the left lower limb for three years. The pain was located in the posterior area of the thigh and leg and radiated to the first toe. Examination showed a tumor of the middle part of the thigh. A sciatic nerve tumor was confirmed at operation. Pathological examination revealed a neurofibrosarcoma. The patient died from pulmonary metastasis 17 months later.

Female↗

Immunoreactivity for Leu-7 in neurofibrosarcoma and other spindle cell sarcomas of soft tissue.

Monoclonal antibodies to Leu-7 have been reported to recognize a constituent of myelin-associated glycoprotein. Because of this, the authors studied 20 cases of neurofibrosarcoma (NFS), 7 of leiomyosarcoma (LMS), 3 of fibrosarcoma (FS), 8 of malignant fibrous histiocytoma (MFH), 5 of monophasic spindle cell synovial sarcoma (MSS), 5 of neurilemmoma, and 5 of neurofibroma for Leu-7 reactivity, to determine its utility in differential diagnosis. Selected examples of each tumor type were also studied ultrastructurally. Leu-7 was compared with the expression of S-100 protein, myelin basic protein, desmin, cytokeratin, and epithelial membrane antigen in the 43 neoplasms. Fifteen examples of NFS, 2 of LMS, and 2 of MSS were Leu-7+; 2 neurofibromas and 4 neurilemmomas also demonstrated reactivity with this antibody. In contrast, all cases of FS and MFH were Leu-7-. Synovial sarcomas could be effectively separated from NFS by reactivities for cytokeratin and epithelial membrane antigen in the former, but not the latter, lesions. However, LMSs and NFSs demonstrated significant immunocytochemical overlap, including shared positivity for S-100, desmin, and myelin basic protein in several cases. These results suggest that Leu-7 has restricted utility in immunohistochemical diagnosis, and must be utilized only in concert with other antigens. Moreover, it would appear that in selected instances, smooth-muscle and neurogenic sarcomas must be separated on the basis of clinical, histopathologic, and ultrastructural features alone.

Adolescent↗

Neurofibrosarcoma and leiomyosarcoma in slaughterhouse broilers.

Two uncommon neoplastic lesions--a neurofibrosarcoma and a leiomyosarcoma--were found in 7-week-old slaughterhouse chickens. Grossly, the neoplasms both appeared as locally extensive, nodular lesions that displaced the mesentery and abdominal viscera. Histologically, these tumors were anaplastic and invasive into the surrounding tissues.

Abattoirs↗

[Neurofibroma--neurofibromatosis--neurofibrosarcoma].

The author presents 14 patients with the gradations "neurofibroma--neurofibromatosis-neurofibrosarcoma". The great variability of forms of this disease ist emphasized. Apart from pure soft-tissue tumours, primary and secondary osseous changes were demonstrated in 4 patients. Intra-oral neurofibromas were seen in 5 cases. In 3 of these, the tongue was involved. Suggestions are given for the treatment of this clinical picture.

Adolescent↗

Update on biologic behavior and surgical implications of neurofibromatosis and neurofibrosarcoma.

Two instances of neurofibrosarcoma arising in young siblings with neurofibromatosis are reported to add to the body of information concerning this potentially lethal complication of von Recklinghausen's disease. The optimum treatment to achieve control of the local disease, especially for centrally located tumors, has yet to be discovered. Results of recent data suggest that radical surgical resection combined with preoperative irradiation and chemotherapy may enhance local control of peripherally located sarcomas. This treatment modality may also obviate the need for amputation in those patients with soft tissue malignant conditions of the extremities. The efficacy of controlling systemic disease with adjuvant chemotherapy has not been demonstrated conclusively. Gallium 67 scanning may provide a method for the early detection of malignant degeneration in neurofibromas.

Adolescent↗