Vélez Roberto, Barrera-Ochoa Sergi, Barastegui David, Pérez-Lafuente Mercedes, Romagosa Cleofe, Pérez Manuel
Orthopaedic Oncology Unit, Orthopaedic Surgery and Traumatology Department, Hospital Universitari Vall d'Hebron, Pg Vall d'Hebron 129-139, 08035 Barcelona, Spain.
Case Rep Neurol Med. 2013;2013:987623. doi: 10.1155/2013/987623. Epub 2013 Mar 28.
Plexiform neurofibromas are benign tumors originating from subcutaneous or visceral peripheral nerves, which are usually associated with neurofibromatosis type 1. Giant neurofibromas are very difficult to manage surgically as they are extensively infiltrative and highly vascularized. These types of lesions require complex preoperative and postoperative management strategies. This case report describes a 22-year-old female with a giant plexiform neurofibroma of the lower back and buttock who underwent pre-operative embolization and intraoperative use of a linear cutting stapler system to assist with haemostasis during the surgical resection. Minimal blood transfusion was required and the patient made a good recovery. This case describes how a multidisciplinary management of these large and challenging lesions is technically feasible and appears to be beneficial in reducing perioperative blood loss and morbidity. Giant neurofibroma is a poorly defined term used to describe a neurofibroma that has grown to a significant but undefined size. Through a literature review, we propose that the term "giant neurofibroma" be used for referring to those neurofibromas weighing 20% or more of the patient's total corporal weight.
丛状神经纤维瘤是起源于皮下或内脏周围神经的良性肿瘤，通常与1型神经纤维瘤病相关。巨大神经纤维瘤手术治疗非常困难，因为它们具有广泛的浸润性且血管高度丰富。这类病变需要复杂的术前和术后管理策略。本病例报告描述了一名22岁女性，患有下背部和臀部的巨大丛状神经纤维瘤，她在手术切除过程中接受了术前栓塞，并在术中使用线性切割吻合器系统辅助止血。患者仅需少量输血，恢复良好。本病例描述了对这些巨大且具有挑战性的病变进行多学科管理在技术上是可行的，并且似乎有利于减少围手术期失血和发病率。巨大神经纤维瘤是一个定义不明确的术语，用于描述已生长到显著但未明确大小的神经纤维瘤。通过文献综述，我们建议将“巨大神经纤维瘤”一词用于指代那些重量占患者总体重20%或更多的神经纤维瘤。
