Neurofibromatosis type 1 and disseminated malignant peripheral nerve sheath tumor
نویسندگان
چکیده
منابع مشابه
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...
متن کاملMalignant peripheral nerve sheath tumor with and without neurofibromatosis type 1.
Objective In this study, we review the institution's experience in treating malignant peripheral nerve sheath tumors (MPNSTs). A secondary aim was to compare outcomes between MPNSTs with and without neurofibromatosis type 1 (NF1). Methods Ninety-two patients with MPNSTs, over a period of 20 years, were reviewed. A retrospective chart review was performed. The median age was 43.5 years (range,...
متن کاملMalignant 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...
متن کاملMetastatic malignant peripheral nerve sheath tumor in neurofibromatosis type 1: a geriatric patient report.
Neurofibromatosis type 1 (NF1) (von Recklinghausen disease) is an autosomal dominantly inherited neurocutaneous disorder which affects many systems like ocular, cutaneous and nervous systems and seen in 1:3500 births. Cardinal diagnostic criteria of NF1 were established in 1987 by National Institutes of Health Consensus.Early diagnosis and the findings of NF1 are unclear in childhood, but with ...
متن کاملA Case of Occipital Malignant Peripheral Nerve Sheath Tumor with Neurofibromatosis Type 1.
INTRODUCTION The prognosis of malignant peripheral nerve sheath tumor (MPNST) with neurofibromatosis type 1 (NF-1) is worse than that of a solitary MPNST, because of the tumor size and location difficult to resect completely. We experienced a case of MPNST in the occipital region with NF-1. CASE REPORT A 59-year-old woman presented with NF-1 and an MPNST of the occipital region. We performed ...
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ژورنال
عنوان ژورنال: QJM: An International Journal of Medicine
سال: 2017
ISSN: 1460-2725,1460-2393
DOI: 10.1093/qjmed/hcx071