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Multidisciplinary Management of a Giant Plexiform Neurofibroma by Double Sequential Preoperative Embolization and Surgical Resection

机译:双序术前栓塞和外科手术切除术治疗巨大的多形神经纤维瘤。

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摘要

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.
机译: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。巨神经纤维瘤由于其广泛浸润和高度血管化而很难手术治疗。这些类型的病变需要复杂的术前和术后处理策略。该病例报告描述了一名22岁的女性,其下背部和臀部有巨大的丛状神经纤维瘤,她们在术前进行了栓塞术,并在术中使用了直线切割吻合器系统以协助手术切除过程中的止血。需要最少的输血,患者恢复良好。该病例描述了对这些大而富挑战性病变的多学科治疗在技术上是可行的,并且在减少围手术期失血和发病率方面似乎是有益的。巨神经纤维瘤是一个定义不明确的术语,用于描述已经发展为明显但不确定的大小的神经纤维瘤。通过文献综述,我们建议使用“巨大的神经纤维瘤”一词来指称那些占患者总体重20%或更多的神经纤维瘤。

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