Malignant peripheral nerve sheath tumor in a patient with neurofibromatosis 1
نویسندگان
چکیده
منابع مشابه
Metastatic 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 کاملIntrathoracic 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 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 کاملMesenteric and Retroperitoneal Malignant Peripheral Nerve Sheath Tumors in a Patient with Neurofibromatosis Type 1
Malignant peripheral nerve sheath tumor (MPNST), an aggressive spindle cell sarcoma is found predominantly in adults. The tumor is closely associated with neurofibromas, neurofibromatosis type 1 (NF-1) and from the sites previously irradiated. Individuals with NF-1 account for 50-60% of all of the patients with MPNSTs [1]. Herein, we describes a fatal case of MPNST in NF-1 with atypical age of ...
متن کاملMalignant peripheral nerve sheath tumors in neurofibromatosis 1.
One of the most clinically aggressive cancers associated with neurofibromatosis 1 (NF1) is the malignant peripheral nerve sheath tumor (MPNST). To determine the incidence and relative risk (RR) of MPNSTs in individuals with NF1, 1,475 individuals with NF1 were included from a cohort of patients examined by a single experienced geneticist from 1977 to 1996. The end points were incidence of MPNST...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
ژورنال
عنوان ژورنال: The Journal of Japanese Society of Lumbar Spine Disorders
سال: 2007
ISSN: 1345-9074,1882-1863
DOI: 10.3753/yotsu.13.104