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Case No. 208
2010-04-19~2010-05-03
출제자 : 관리자
Hit : 378
| Period | 2010-04-19~2010-05-03 |
|---|---|
| Diagnosis | |
| Clinical information | 23/F Diplopia and headache 3 weeks ago 16세에 multiple skin mass 발생, neurofibromatosis type 1 진단 |
| Discussion | 진단 : Giant cell glioblastoma(grade IV by WHO) Imaging findings: Focal areas of signal intensity at the basal ganglia A large necrotic mass with internal hemorrhage and severe perilesional edema 고찰: Neurofibromatosis type I – most common neurocutaneous disease, autosomal dominant The majority of NF1 patients are only mildly affected, but clinical course is highly variable, though generally progressive 10% lifetime risk of progressing to malignant peripheral nerve sheath tumors NF1 patient have an increased risk of developing neoplasm, such as leukemia, pheochromocytomas, and brain tumor. The most common of these are hypothalamic-optic gliomas, flowed by brainstem and cerebellar pilocytic astrocytoma The astrocytomas observed in 15-20% of these patients generally have a benign nature. NF1 patients however have a 5-fold increased incidence of high-grade astrocytoma(WHO grade IV), also known as glioblastoma multiforme. These tumors are usually located in supratentorial region. |
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