Management of large dorsal diffuse plexiform neurofibroma

被引：0
作者：
D'Arpa S. [1 ]
Tuttolomondo A. [1 ]
Melloni C. [1 ]
Cordova A. [1 ]
Moschella F. [1 ]
机构：
[1] Plastic and Reconstructive Surgery Unit, University of Palermo, Palermo
来源：
European Journal of Plastic Surgery | 2014年 / 37卷 / 6期
关键词：
Chemoembolization; Large plexiform neurofibroma; Neurofibroma; NF1; disease; Surgical management;
D O I：
10.1007/s00238-014-0953-5
中图分类号：
学科分类号：
摘要：
Neurofibromas are considered one of the hallmark signs of neurofibromatosis type 1(NF1). Diffuse plexiform neurofibroma is a congenital NF1-associated tumor, characterized by overgrowth and interference with function of the affected area. The rich vascular plexus associated with neurofibromas, together with their infiltrative pattern, makes them difficult to eradicate. Complications of neurofibromas are rare but include malignant transformation and potentially life-threatening hemorrhages. The use of the term "giant" to define a neurofibroma is controversial because there is no clear consensus and descriptions are limited to few case reports. We report a case of a large dorsal diffuse plexiform neurofibroma responsible for significant worsening of a patient's quality of life. These features make us believe that the mass can be defined as "giant." Comprehensive perioperative management, including arterial embolization of the feeding vessels and stabilization of the patient's conditions, is required to perform a safe surgical procedure in such a complicated case. Level of Evidence: Level V, therapeutic study © 2014 Springer-Verlag.
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页码：349 / 352
页数：3
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