Giant Mediastinal Neurofibroma in a Child with Neurofibromatosis Type I
Giant Mediastinal Neurofibroma in a Child with Neurofibromatosis Type I作者机构：Department of Gastroenterological Surgery I Hokkaido University Graduate School of Medicine Sapporo Japan Department of Pediatrics Hokkaido University Graduate School of Medicine Sapporo Japan Department of Surgical Pathology Hokkaido University Hospital Sapporo Japan
出 版 物：《Surgical Science》 (外科学（英文）)
年 卷 期：2012年第3卷第12期
页 面：564-567页
学科分类：1002[医学-临床医学] 100214[医学-肿瘤学] 10[医学]
主 题：Mediastinal Tumor Neurofibromatosis Type I Neurofibroma Open Biopsy
摘 要：Aim: There are a variety of malignant tumors related to neurofibromatosis type 1 (NF1). This report describes a rare pediatric NF1 case with an unresectable giant mediastinal tumor. Case: A 6-year-old girl with wheezing was admitted to our institution for the further evaluation of a right mediastinal mass on plain chest radiography. On examination, there were multiple café au lait spots mainly on the trunk, and a well-defined, immobile, painless mass was palpable on her neck. The mediastinal lesion was detected as nonuniform mass surrounding the aortic arch, pulmonary artery, and right main bronchus on the contrast-enhanced CT and MRI. Open biopsy was useful to rule out malignancy and revealed neurofibroma, and contributed to follow up and treatment. Discussion: Open biopsy was useful to rule out malignancy, such as malignant peripheral nerve sheath tumor, revealed neurofibroma, and also contributed to follow up and treatment. The authors report successful management by open biopsy and discuss several clinical points regarding mediastinal neurofibroma for NF1.
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