tailieunhanh - Báo cáo y học: "Surgical treatment of giant plexiform neurofibroma associated with pectus excavatum"

Tuyển tập các báo cáo nghiên cứu về y học được đăng trên tạp chí y học Wertheim cung cấp cho các bạn kiến thức về ngành y đề tài: Surgical treatment of giant plexiform neurofibroma associated with pectus excavatum. | Ji et al. Journal of Cardiothoracic Surgery 2011 6 119 http content 6 1 119 JCTS JOURNAL OF CARDIOTHORACIC SURGERY CASE REPORT Open Access Surgical treatment of giant plexiform neurofibroma associated with pectus excavatum Yi Ji1 Bing Xu1 Xuejun Wang1 Wenying Liu1 and Siyuan Chen1 2 Abstract Plexiform neurofibromas are benign tumors originating from subcutaneous or visceral peripheral nerves which are usually associated with neurofibromatosis type 1. They are almost always congenital lesions and often cause the surrounding soft tissue and bone to grow aberrantly. We treated a 12-year-old boy who presented with asymmetric pectus excavaum and an anterior chest wall plexiform neurofibroma. The pectus excavaum was corrected by modified Nuss procedure followed by simultaneous resection of the giant mass. The patient is doing well at the 4 years follow-up visit. Keywords Plexiform neurofibromas Pectus excavaum Nuss procedure 1. Background Plexiform neurofibromas PNFs are benign nerve tumor resulting from aberrant growth of the cells of nerve sheath. They are usually congenital but they may instead present during the first year as a subtle soft-tissue enlargement or a large patch of cutaneous hyperpigmentation. PNFs are generally painless slowly growing neoplasmas. Although most neoplasms are asymptomatic they can be particularly debilitating due to their potential to grow to very large sizes. Presenting symptoms depend on the location of the tumors. Tumors of head neck and face are most common followed by facial disfigurement and lesions of the spine extremities and abdomen 1 . Early childhood puberty and childbearing age are considered to be the periods of greatest risk for disease progression. Furthermore PNFs have a potential for transformation into highly malignant peripheral nerve sheath tumors which occur in approximately 5 of patients 2 . Unfortunately there is no accepted effective medical treatment for PNFs. Current management of .