Malignant Peripheral Nerve Sheath Tumor Arising from Neurofibromatosis

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Malignant Peripheral Nerve Sheath Tumor Arising from Neurofibromatosis

Neurofibromatosis type 1 (NF 1) is an inherited autosomal dominant disorder with an estimated incidence of 1 in 2,500 to 3,000 live births [1]. Neurofibromas are benign peripheral nerve sheath tumors developed from the proliferation of fibroblasts and Schwann cells. Wallac [2] categorized neurofibromas into four types: cutaneous neurofibromas of the epidermis or dermis, subcutaneous neurofibrom...

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Intrathoracic Malignant Peripheral Nerve Sheath Tumor: Histopathological and Immunohistochemical Features

Malignant peripheral nerve sheath tumor (MPNST) is a rare nerve sheath tumor derived from Schwann cells or pleuripotent cells of neural crest. Neurogenic tumors make about 10-20% of all mediastinal tumors. Incidence of MPNST is 0.001% in general population and 0.16% in patients with neurofibromatosis I (NF I). We report a case of 60 year female presenting with progressive cough and breathlessne...

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Malignant Peripheral Nerve Sheath Tumour Arising From Solitary Sclerotic Neurofibroma.

Neurofibroma is a benign neoplasm of the peripheral nerve, which occurs as multiple tumours in neurofibromatosis type 1 (NF1) or as a solitary tumour with no association with NF1. Malignant change is sometimes reported in neurofibroma in NF1, but there are few reports of malignant transformation of solitary neurofibroma. We report here a case of malignant change in a solitary sclerotic neurofib...

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A Case of Malignant Peripheral Sheath Tumor Arising from Neurofibromatosis Type 1.

Malignant peripheral nerve sheath tumor (MPNST) is a term encompassing tumors previously diagnosed as malignant schwannoma, malignant neurilemmoma, neurogenic sarcoma, and neurofibrosarcoma The occurrence rate of MPNST in neurofibromatosis type 1 patients is known to be about 4.6%. Tumors occurring in this particular group have a worse prognosis in that they occur at an earlier age, are more ce...

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Malignant peripheral nerve sheath tumor of kidney.

A 40-year-old man presented with complaints of left-sided lower back discomfort for 2 to 3 months. There was no other significant history. Physical examination was unremarkable except for a palpable mass in the left flank. Ultrasonography showed a hypoechoic mass. Biphasic contrast-enhanced computerized tomogram revealed a large, heterogenous, infiltrating, necrotic mass lesion arising from the...

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ژورنال

عنوان ژورنال: Archives of Plastic Surgery

سال: 2013

ISSN: 2234-6163,2234-6171

DOI: 10.5999/aps.2013.40.3.272